European Society of Cardiology, acute cardiovascular care association, SCAD study group: a position paper on spontaneous coronary artery dissection

European Society of Cardiology, acute cardiovascular care association, SCAD study group: a... ESC-ACCA Position Paper on spontaneous coronary artery dissection  Contents Introduction 3354 Definition 3354 Pathology 3354 Epidemiology 3356 Incidence 3356 Demographics 3356 Pathophysiology: risk factors and associations 3356 Female sex and pregnancy 3356 Fibromuscular dysplasia 3357 Inflammatory conditions 3358 Atherosclerotic risk factors 3358 Mechanical stressors and exercise 3359 Emotional stressors 3359 Inherited connective tissue disorders 3359 Genetics 3359 Clinical presentation 3359 Diagnosis 3359 Coronary angiographic classification 3360 Additional possible angiographic findings 3360 Multi-vessel spontaneous coronary artery dissection 3361 Secondary iatrogenic dissection in spontaneous coronary artery dissection 3361 Intracoronary imaging 3361  Intravascular ultrasound 3361  Optical coherence tomography 3362 Computed tomography coronary angiography 3363 Acute management 3364 Conservative management 3364 Percutaneous coronary intervention 3364 Coronary artery bypass grafting 3365 Adjunctive supportive devices and transplant 3365 Medical management 3365  Thrombolysis 3365  Antiplatelet therapies 3365  Anticoagulant therapies 3365  Angiotensin converting enzyme inhibitors, angiotensin receptor antagonists, mineralocorticoid receptor antagonists, beta-blockers and vasodilator therapies 3365  Statins 3366  Contraception and hormone replacement therapy 3366 Pregnancy 3366 Pregnancy-associated spontaneous coronary artery dissection: special considerations 3366 Pregnancy after spontaneous coronary artery dissection 3366 Outcomes and follow-up 3366 Follow-up imaging 3366  Echocardiography/cardiac magnetic resonance imaging 3366  Computed tomography-peripheral angiography or magnetic resonance-angiography 3366  Coronary angiography or CT-coronary angiography 3367 Prognosis: mortality, recurrence risk and major adverse cardiac events 3367 Post spontaneous coronary artery dissection chest pain and its management 3367 Cardiac rehabilitation and exercise 3368 Post-traumatic stress disorder and the emotional and psychological consequences of spontaneous coronary artery dissection 3368 Management considerations 3368 Research priorities 3368 Introduction Spontaneous coronary artery dissection (SCAD) has long been recognized as a cause of acute coronary syndromes (ACS). Initially considered very rare and associated primarily with pregnancy and the peripartum period, the use of higher sensitivity Troponin assays and early angiography in ACS, coupled with greater awareness of the condition, has led to increased diagnosis, and it is now understood that SCAD represents a significant cause of ACS in predominantly young to middle-aged women, with most cases occurring outside the context of recent pregnancy.1,2 Although there are no randomized controlled trials in SCAD, knowledge has further advanced in the last 5-years as a result of an international research effort primarily focused on building and studying national SCAD registries.3–19 These studies have demonstrated, not only that SCAD is a distinct pathophysiological entity, but that there are key differences in management and outcomes compared to ACS of atherosclerotic aetiology. This position paper aims to set-out current knowledge on SCAD for the benefit of practicing clinicians caring for patients with this condition. It presents the consensus on contemporary management and areas of controversy and uncertainty, which remain a focus of ongoing research. The information is provided to support clinical care providers but is not intended to replace individualized decision-making by clinicians and other health care professionals. Definition For the purposes of this article, SCAD refers to the acute development of a false lumen within the coronary artery wall which may compromise coronary flow by external compression of the true lumen. Dissections arising from coronary instrumentation (iatrogenic), trauma and as a consequence of a penetrating ulcer or plaque rupture secondary to atherosclerotic disease or primary aortic dissection are not considered here. Pathology Spontaneous coronary artery dissection is a recognized, relatively rare cause of sudden cardiac death, presumably as a result of ventricular arrhythmia triggered by myocardial ischaemia or infarction.20 Accurate diagnosis at autopsy can be challenging and the condition is likely under-represented in post mortem series.21,22 A high index of suspicion for SCAD is recommended in all potential cases with careful assessment of coronary histopathology, particularly of the mid-distal vessels which are predominantly affected in SCAD and examination of the peripheral arterial system for associated arteriopathies [such as fibromuscular dysplasia (FMD)]. Spontaneous coronary artery dissection results from the development of a false lumen, generally in the outer third of the tunica media (Figure 1A).21–27 The primary cause of false lumen formation is unclear with two potential mechanisms proposed: the ‘inside-out’ model (Figure 1B), where the causal event is the development of an endothelial and intimal discontinuity or ‘tear’, allowing blood to cross the internal elastic lamina and accumulate in the media; and the ‘outside-in’ mechanism (Figure 1C) where the causal event is the primary disruption of a vasa vasorum micro-vessel leading to haemorrhage directly into the tunica media.1,28,29 In either case blood propagates axially as the false lumen extends leading to compression of the true lumen. It remains unclear if there is a single dominant mechanism in SCAD or if both causal events are possible. However, a recent intracoronary imaging study with high resolution optical coherence tomography (OCT) has shown case examples where there is no demonstrable communication between false and true lumens,13,30,31 suggesting the ‘outside-in’ mechanism is likely in at least some cases. Figure 1 View largeDownload slide Schematic illustration of spontaneous coronary artery dissection. Accumulation and axial propagation of blood forms a false lumen in the outer third of the tunica media leading to external compression of the true lumen (A). Blood may enter through an endothelial-intimal disruption or ‘tear’ (B) or as a result of bleeding from a microvessel within the vessel wall (C) leading to an expanding and compressing false lumen (dotted arrows). Figure 1 View largeDownload slide Schematic illustration of spontaneous coronary artery dissection. Accumulation and axial propagation of blood forms a false lumen in the outer third of the tunica media leading to external compression of the true lumen (A). Blood may enter through an endothelial-intimal disruption or ‘tear’ (B) or as a result of bleeding from a microvessel within the vessel wall (C) leading to an expanding and compressing false lumen (dotted arrows). Histologically, fibrin-rich haematoma is present in the false lumen (Figure 2) with a neutrophil infiltrate extending into the media. There are frequent reports of a peri-adventitial inflammatory infiltrate with a predominance of eosinophils.21,23–25,27,32–36 The clot reorganizes and attaches to the media with granulation tissue formation and recanalization. Although this may not be specific to SCAD, it can be useful in distinguishing SCAD from post mortem artefact where fissure formation may occur but only red blood cells are present.21 Some early cases report features of cystic medial necrosis37–39 but more recent reports do not concur.32,34,40 Significant co-existent atherosclerotic coronary artery disease in SCAD is uncommon.21 Key Messages Spontaneous coronary artery dissection should be actively considered in the post-mortem differential diagnosis of unexplained sudden cardiac death with careful assessment of the entire coronary tree. Figure 2 View largeDownload slide Gross pathology (A) and histopathology (B) showing spontaneous coronary artery dissection with external compression of the true lumen by blood within a false lumen in the outer third of the tunica media. Figure 2 View largeDownload slide Gross pathology (A) and histopathology (B) showing spontaneous coronary artery dissection with external compression of the true lumen by blood within a false lumen in the outer third of the tunica media. Epidemiology Incidence The true incidence of SCAD is unknown as this condition remains under-diagnosed.2 Spontaneous coronary artery dissection was historically considered very rare but contemporary angiographic series report SCAD diagnosis rates of 0.07–0.2% of all angiograms and 2–4% of angiograms performed for ACS14,41,42 (although the study with the highest ACS prevalence did not exclude all atherosclerotic cases42). Furthermore, in younger women SCAD is reported to account for a much higher proportion of ACS presentations. In a Canadian series of women less than 50 years with myocardial infarction, SCAD accounted for 24% of cases.43 Likewise a Japanese registry reported SCAD in 35% of females patients under 50 years presenting with acute myocardial infarction (AMI),8 a French series reported SCAD in 36% of women under 60 years with ACS and one or fewer conventional cardiovascular risk factors13 and a smaller Australian series describes a SCAD prevalence of 23% in women under 60 years presenting with ACS.16 Pregnant and peripartum cases (P-SCAD) account for a minority of these cases (around 10% in most contemporary series)3–11,13–16,18,44,45 and SCAD should no longer be considered primarily a peripartum condition. However, 21–27% of myocardial infarctions in pregnancy and 50% of post-partum coronary events are reportedly due to SCAD.18,46 Demographics Previously considered primarily a disease of young adults, SCAD has now been described in patients aged 18–84 years4,44 with the mean age in large contemporary series ranging from 44 to 53 years.3–5,7–16 No ethnic variations have been reported but there is a strong female predominance (female sex and pregnancy section). The demographics of SCAD patients from 14 contemporary series with at least 20 patients are summarized in Table 1.3–16 Table 1 Demographics and risk factors of patients with spontaneous coronary artery dissection (SCAD) in contemporary case series (studies with n > 20) Max N Age (years) Gender (female, %) HTN (%) Chol (%) Smoking (%) DM (%) FH (%) P-SCAD (%) Mayo Clinic3 189 44 ± 9 92 31 22 15 2 NA 15 Saw4 168 52 ± 9 92 39 24 13 5 29 2 Lettieri5 134 52 ± 11 81 51 33 34 2 25 NA Faden6 79 33 ± 5 100 17 18 17 11 NA 100 Rogowski7 64 53 ± 11 94 45 52 28 0 19 5 Nakashima8 63 46 ± 10 94 33 23 32 0 8 8 Motreff13 55 50 100 27 11 22 4 22 4 McGrath-Cadell9 40 45 ± 10 95 18 10 8 5 28 8 Roura10 34 47 ± 12 94 NA NA NA NA 15 Alfonso11 27 52 ± 10 85 37 33 52 4 NA 4 Ito12 23 45 ± 11 100 57 22 30 4 NA 30 Vanzetto14 23 46 ± 9 74 26 39 43 13 13 0 Mortensen15 22 49 ± 9 81 38 NA 57 0 40 10 Rashid16 21 53 ± 9 95 48 48 47 5 24 0 Max N Age (years) Gender (female, %) HTN (%) Chol (%) Smoking (%) DM (%) FH (%) P-SCAD (%) Mayo Clinic3 189 44 ± 9 92 31 22 15 2 NA 15 Saw4 168 52 ± 9 92 39 24 13 5 29 2 Lettieri5 134 52 ± 11 81 51 33 34 2 25 NA Faden6 79 33 ± 5 100 17 18 17 11 NA 100 Rogowski7 64 53 ± 11 94 45 52 28 0 19 5 Nakashima8 63 46 ± 10 94 33 23 32 0 8 8 Motreff13 55 50 100 27 11 22 4 22 4 McGrath-Cadell9 40 45 ± 10 95 18 10 8 5 28 8 Roura10 34 47 ± 12 94 NA NA NA NA 15 Alfonso11 27 52 ± 10 85 37 33 52 4 NA 4 Ito12 23 45 ± 11 100 57 22 30 4 NA 30 Vanzetto14 23 46 ± 9 74 26 39 43 13 13 0 Mortensen15 22 49 ± 9 81 38 NA 57 0 40 10 Rashid16 21 53 ± 9 95 48 48 47 5 24 0 Data are given as mean ± standard deviation or percentages. HTN, hypertension; Chol, dyslipidaemia; DM, diabetes mellitus; FH, family history of coronary artery disease; NA, not available; P-SCAD, pregnancy-associated coronary artery dissection. Table 1 Demographics and risk factors of patients with spontaneous coronary artery dissection (SCAD) in contemporary case series (studies with n > 20) Max N Age (years) Gender (female, %) HTN (%) Chol (%) Smoking (%) DM (%) FH (%) P-SCAD (%) Mayo Clinic3 189 44 ± 9 92 31 22 15 2 NA 15 Saw4 168 52 ± 9 92 39 24 13 5 29 2 Lettieri5 134 52 ± 11 81 51 33 34 2 25 NA Faden6 79 33 ± 5 100 17 18 17 11 NA 100 Rogowski7 64 53 ± 11 94 45 52 28 0 19 5 Nakashima8 63 46 ± 10 94 33 23 32 0 8 8 Motreff13 55 50 100 27 11 22 4 22 4 McGrath-Cadell9 40 45 ± 10 95 18 10 8 5 28 8 Roura10 34 47 ± 12 94 NA NA NA NA 15 Alfonso11 27 52 ± 10 85 37 33 52 4 NA 4 Ito12 23 45 ± 11 100 57 22 30 4 NA 30 Vanzetto14 23 46 ± 9 74 26 39 43 13 13 0 Mortensen15 22 49 ± 9 81 38 NA 57 0 40 10 Rashid16 21 53 ± 9 95 48 48 47 5 24 0 Max N Age (years) Gender (female, %) HTN (%) Chol (%) Smoking (%) DM (%) FH (%) P-SCAD (%) Mayo Clinic3 189 44 ± 9 92 31 22 15 2 NA 15 Saw4 168 52 ± 9 92 39 24 13 5 29 2 Lettieri5 134 52 ± 11 81 51 33 34 2 25 NA Faden6 79 33 ± 5 100 17 18 17 11 NA 100 Rogowski7 64 53 ± 11 94 45 52 28 0 19 5 Nakashima8 63 46 ± 10 94 33 23 32 0 8 8 Motreff13 55 50 100 27 11 22 4 22 4 McGrath-Cadell9 40 45 ± 10 95 18 10 8 5 28 8 Roura10 34 47 ± 12 94 NA NA NA NA 15 Alfonso11 27 52 ± 10 85 37 33 52 4 NA 4 Ito12 23 45 ± 11 100 57 22 30 4 NA 30 Vanzetto14 23 46 ± 9 74 26 39 43 13 13 0 Mortensen15 22 49 ± 9 81 38 NA 57 0 40 10 Rashid16 21 53 ± 9 95 48 48 47 5 24 0 Data are given as mean ± standard deviation or percentages. HTN, hypertension; Chol, dyslipidaemia; DM, diabetes mellitus; FH, family history of coronary artery disease; NA, not available; P-SCAD, pregnancy-associated coronary artery dissection. Key Messages Whilst SCAD has been described across a broad demographic, it is a frequent cause of ACS in young- to middle-aged women and patients with myocardial infarction in egnancy or post-partuprm. Pregnancy-associated SCAD accounts for a minority of cases. Pathophysiology: risk factors and associations The pathophysiology of SCAD remains unknown. It is likely that a combination of predisposing factors increase susceptibility such that a relatively minor trigger event is sufficient to precipitate SCAD. There are a large number of reported associations with SCAD (Supplementary material online, Table S2).4,8,9,33,38,44,47–91,117–124,127–133,140–143 For some (e.g. female sex, pregnancy, and FMD), a link with SCAD has been established in multiple series. Other associations are based on anecdotal case reports, and in this context causality is harder to determine. Female sex and pregnancy The vast majority of SCAD patients (∼90%) are women.3–11,14–18 There is some evidence from a Canadian series that male SCAD patients are different from female cases, being slightly younger and with higher rates of preceding isometric exercise and lower prior emotional stress levels.92 The predilection of SCAD for female patients and the association with pregnancy suggest a pathophysiological role for female sex hormones. The precise nature of this association remains to be elucidated but may relate to hormonal influences on vascular connective tissue and/or the vessel microvasculature. Data from most contemporary SCAD series suggest that P-SCAD represents approximately 10% of SCAD cases. Multi-parity, fertility hormones and pre-eclampsia may increase risk.45,93–95 Pregnant and peripartum cases has been reported antepartum as early as the 5th week of pregnancy,96 although most events reported during pregnancy are in the third trimester.97,–99 It also occurs in the early (<6 weeks), late (6 weeks to 12 months), and very late post-partum (12 to 24 month) periods, with the peak occurring in the early post-partum period.95,97–99 Anecdotally, late post-partum SCAD may occur in association with breastfeeding.96 A Canadian study identified 79 P-SCAD cases from a nationwide cohort study of 4.4 million pregnancies between 2008 and 2012 (incidence 1.8 SCAD cases per 100 000 pregnancies),6 a higher incidence of P-SCAD compared to the historical literature. This study suggested the P-SCAD presentation may be more severe than SCAD outside the context of pregnancy, with ST-elevation myocardial infarction (STEMI) in 64%, cardiogenic shock in 24%, cardiac arrest in 14%, and maternal death in 4.5%. Moreover, P-SCAD was more likely to involve the proximal coronaries and was associated with a reduction in post-infarct ejection fraction. A recent report of P-SCAD cases from the US Mayo Clinic Series also reported increased STEMI, proximal or mutivessel disease and worse left ventricular function compared to non-P-SCAD. The findings of a more severe P-SCAD phenotype were confirmed in an analysis of contemporary published cases99 and a small retrospective study comparing 7 P-SCAD and 16 SCAD cases.12 Fibromuscular dysplasia Spontaneous coronary artery dissection has been associated with various predisposing arteriopathies (Figure 3).1,2,92 The most frequent is FMD, a non-atherosclerotic, non-inflammatory disease of arterial walls, which also occurs predominantly in middle-aged females with few cardiovascular risk factors. Fibromuscular dysplasia may lead to stenosis, dissections, and aneurysms of medium-sized arteries, including but not limited to renal, cervico-cephalic, and visceral arteries.100,101 It is currently classified by angiography into two subtypes, multifocal and (uni)focal. Multifocal FMD, with the typical ‘string-of-beads’ pattern, is the angiographic presentation of medial FMD and is at least four times more frequent than unifocal FMD.101–103 Figure 3 View largeDownload slide Extracoronary abnormalities in spontaneous coronary artery dissection including renal (A and D) and femoral (B) fibromuscular dysplasia, carotid and vertebrobasilar aneurysms and tortuosity (C and F) and a localised iliac dissection (E). Figure 3 View largeDownload slide Extracoronary abnormalities in spontaneous coronary artery dissection including renal (A and D) and femoral (B) fibromuscular dysplasia, carotid and vertebrobasilar aneurysms and tortuosity (C and F) and a localised iliac dissection (E). Since the first case series reporting the association of SCAD with extra-coronary FMD in 2012,104,105 FMD of extra-coronary vascular beds has been documented in 11–86% of patients with SCAD,4,9,44,47,50,106–108 with a narrower range of 41–86% after exclusion of three studies in which <50% of patients were screened9,44,47 (Figure 4). The prevalence of extra-coronary FMD in various cohorts may differ according to the proportion of patients screened, the screening protocol used (e.g. imaging technique, vascular beds, screening according to symptoms vs. systematic assessment etc.) and diagnostic criteria of FMD. Fibromuscular dysplasia of extra-coronary vascular beds may also be more frequent in SCAD patients with a higher coronary tortuosity score.47 Figure 4 View largeDownload slide Prevalence of extracoronary lesions of fibromuscular dysplasia in contemporary series of spontaneous coronary artery dissection patients. Figure 4 View largeDownload slide Prevalence of extracoronary lesions of fibromuscular dysplasia in contemporary series of spontaneous coronary artery dissection patients. In current series, the most commonly affected vascular beds are renal, cervico-cephalic and iliac arteries.4,50,106–108 In three studies in which the information was available,104,106,107 29–70% of SCAD patients with extra-coronary FMD had involvement of two or more vascular beds. While Saw et al.4 focused their analysis on the presence of the ‘string-of-beads’ appearance, almost pathognomonic of multifocal FMD, Prasad et al.107 extended vascular screening to isolated stenosis possibly due to unifocal FMD. Notably however, the 52 cases of extra-coronary FMD documented in their series of 115 SCAD patients were exclusively of the multifocal subtype. Finally, besides typical FMD lesions, other extra-coronary vascular abnormalities (EVAs) such as aneurysms, dissections, irregularities, undulations, and/or tortuosity have been reported.106,107 Whether the latter correspond to subtle forms of FMD or a different process such as a form of connective tissue disorder remains to be established107 The clinical implications of the association between EVAs and SCAD are described in the ‘Computed tomography-peripheral angiography or magnetic resonance-angiography section' below. In view of the high prevalence of extra-coronary FMD in patients with SCAD, it is tempting to speculate that SCAD is a complication of underlying coronary FMD in a substantial proportion of cases.108 Pathological demonstration of coronary FMD lesions in at least four cases of SCAD37,109–111 adds weight to this hypothesis. However, at present, a degree of caution is required in equating SCAD with FMD, as (i) current data on the association of SCAD with extra-coronary FMD mostly derive from two centres in Canada4,104 and the USA,44,47,50,106,107 with repeat publications from the same group likely representing different stages of recruitment and exploration of these two cohorts; (ii) typical FMD coronary lesions are rare112,113 and description of the angiographic characteristics of coronary FMD is only in its infancy114,115; (iii) the prevalence of coronary artery dissection is very low (<3%) in the US FMD registry116; and (iv) a proportion of cases of SCAD remain idiopathic or are associated with other vascular diseases. Inflammatory conditions Spontaneous coronary artery dissection has been associated with systemic inflammatory conditions (Supplementary material online, Table S2).33,68–77,117–124 While the prevalence of inflammatory systemic diseases was 8.9% in a Canadian series of 168 cases,4 this is not yet widely corroborated in other series.11 A clear mechanistic link between systemic inflammation and SCAD remains to be elucidated. Atherosclerotic risk factors Significant atherosclerosis is rare in typical SCAD (although this may partly reflect the criteria used to define cases). Spontaneous coronary artery dissection patients have fewer traditional cardiovascular risk factors for ischaemic heart disease than patients with atherosclerotic coronary artery disease,44 and some patients have no such risk factors at all. However, in contrast to the previous perception, many patients do have some risk factors for ischaemic heart disease including hypertension, smoking, and dyslipidaemia3–18 (Table 1), although there is no evidence these contribute directly to the risk of SCAD. For example, the mean low-density lipoprotein cholesterol (LDL-C) plasma concentration in the case series by Rogowski et al.7 was 3.3 mmol/L and some cases of SCAD have been reported in patients with severe hypertension.125,126 In contrast, diabetes seems to be rare in SCAD patients. In keeping with the wider population, coronary risk factors tend to be more prevalent in older patients with SCAD. Mechanical stressors and exercise A number of mechanical triggers have been linked to SCAD events, including extreme Valsalva-type manoeuvres and the provocation of coronary spasm (Supplementary material online, Table S2).4,9,79–91,127–133 Dissections have also been reported following isometric or extreme exercise,134–138 reported in 11.9% of cases in a recent prospective series19 and occurring more commonly in men.92 A mechanism linked to increased coronary wall shear stress has been proposed. There are also patients in whom there is a significant lag time between the last exercise event and the onset of symptoms suggesting a higher exercise performance capability may be a feature of the SCAD phenotype in some patients (potentially relating to a mild connective tissue phenotype).139 Emotional stressors Antecedent emotional stresses such as a bereavement or major personal crisis have been reported in a higher than expected proportion of SCAD cases, particularly in women.4,8,92 Inherited connective tissue disorders Although SCAD has frequently been reported in association with connective tissue disorders (Supplementary material online, Table S2),38,44,50–58,140–143 large contemporary series suggest such cases constitute a tiny minority of prevalent cases (around 1–2% in the US, Canadian, and Japanese series)4,107 and until now the yield from genetic screening of SCAD-survivors for associated genes has been reported to be very low.50 Genetics The genetics of SCAD remains to be clearly elucidated with very few focused studies to date. Whilst a number of sibling–sibling pairs and mother–daughter pairs have been described,144 SCAD outside the rare context of known connective tissue disorders does not appear to be a strongly inherited condition, with only 1.2% of 412 patients in one series describing a family history of SCAD.144 Hence whilst a thorough clinical assessment for rare connective tissue disorders is required in all SCAD-survivors, whether more systematic genetic screening is useful and cost-effective remains to be demonstrated.50,115 Key Messages The strong female predisposition and association with pregnancy suggest a role for female sex-hormones in the pathogenesis of SCAD but the mechanism remains unknown. Spontaneous coronary artery dissection is frequently associated with extra-coronary arteriopathies including FMD. Spontaneous coronary artery dissection is not a strongly inherited condition. Research is needed to better understand the pathophysiology of SCAD. Clinical presentation There is considerable evidence that SCAD remains under-diagnosed.2 Some patients fail to act on their symptoms and never present to medical services and a few cases may present with sudden cardiac death (see pathology section). However, of those who do present to medical services, missed or delayed diagnosis is common.1,145 Some patients are not referred for coronary investigations, primarily because most acute medical and cardiology services are focused on the identification of patients at high risk of obstructive atherosclerotic ACS and patients with SCAD typically fall into the lowest risk groups on the basis of traditional risk scores for ischaemic heart disease. A high index of suspicion in typical patients, coupled with familiarity with the angiographic variants of SCAD is key to minimizing missed or delayed diagnoses. Patients with SCAD usually present with an ACS associated with positive biomarkers of myocardial necrosis (e.g. high sensitivity troponin). The proportion of cases presenting with STEMI (26–55%) vs. non-ST-elevation myocardial infarction (NSTEMI) varies in the larger published series.3–5,8,13,45 This likely reflects differences in patient selection for these registries. A minority present with ventricular arrhythmia (2.8–10%),3–5,8,45 Chest pain is the most frequently described presenting symptom,15,44,146 an association which may be more common in SCAD than atherosclerotic ACS patients (chest pain reported in 60–90% of SCAD patients).146 This may be because, in addition to pain arising from myocardial ischaemia and infarction, dissection per se is inherently painful.145 In the Canadian series, chest pain was reportedly associated with radiation to the arm (49.5%) and neck (22.1%), nausea and vomiting (23.4%), diaphoresis (20.9%), dyspnoea (19.3%), and back pain (12.2%).146 Despite the strong association with chest pain, the nature of the pain may be atypical with ‘burning’ (9%), ‘pleuritic’ (3.0%), ‘tearing’ (1.0%), and ‘positional’ (1%) descriptors in a minority of patients.146 Key Messages Patients with SCAD usually present with ACS. Delayed diagnosis is common and SCAD should be actively considered in the differential diagnosis of ACS presentations in low risk patients. Diagnosis The differential diagnosis for SCAD includes atherosclerotic ACS, coronary artery spasm, Takotsubo cardiomyopathy, coronary thromboembolism, and myocardial infarction with non-obstructed coronary arteries (MINOCA). There are no currently identified specific blood biomarkers for SCAD. Coronary angiography represents the principal tool for the diagnosis of SCAD in clinical practice.1,145,147 Intracoronary nitroglycerin should be given, where blood pressure allows, to ensure complete vasodilation and to rule out the possibility of associated coronary spasm. With experience most SCAD cases can be diagnosed on angiography alone, with intracoronary imaging reserved for cases where diagnostic uncertainty exists.145 However, it is important to appreciate that the appearances of a radiolucent flap, dual lumen, and contrast hold-up seen with iatrogenic dissections148 and familiar to most interventional cardiologists are present in only a minority of SCAD angiograms. Coronary angiographic classification The most commonly used angiographic classification of SCAD has been adapted from Saw et al.149,150 (Figure 5), although other approaches are also proposed (Supplementary material online, Figure S6).13 From the Saw classification, Type 1 represents the classical angiographic radiolucent ‘flap’ and linear double lumen often associated with contrast hold-up (Figure 5A). This reportedly occurs in 29–48% of cases.4,7,8 However these features are absent in the commoner Type 2 pattern (52–67%)4,7,8 characterized by a long diffuse and smooth stenosis predominantly located in mid-to-distal segments. This has been divided into Type 2a (Figure 5B) where there is recrudescence of a normal calibre distal vessel and Type 2b (Figure 5C) where the stenosis extends angiographically to the end of the vessel. Intermediate appearances (between Type 1 and 2) are also seen where a typical Type 2 pattern is seen but with a short segment of dual lumen or contrast hold-up in keeping with a localized fenestration between true and false lumen (Figure 5F). Type 3 lesions (Figure 5D) are defined as angiographically indistinguishable from a focal atherosclerotic stenosis requiring diagnostic confirmation by intracoronary imaging (see intracoronary imaging section). However, these account for a small minority of cases (0–3.9%).4,7,8 Type 4 SCAD is described as a total occlusion, usually of a distal vessel (Figure 5E).145 In this uncommon circumstance, the diagnosis is particularly challenging and frequently can only be established during an ensuing coronary intervention once coronary flow is re-established or inferred by subsequent vessel healing and the exclusion of an embolic cause. Figure 5 View largeDownload slide Angiographic classification of spontaneous coronary artery dissection. Type 1 spontaneous coronary artery dissection (A), Type 2A spontaneous coronary artery dissection (B), Type 2B spontaneous coronary artery dissection (C), Type 3 spontaneous coronary artery dissection (D), Type 4 spontaneous coronary artery dissection (E), and Intermediate Type1/2 spontaneous coronary artery dissection (F). Figure 5 View largeDownload slide Angiographic classification of spontaneous coronary artery dissection. Type 1 spontaneous coronary artery dissection (A), Type 2A spontaneous coronary artery dissection (B), Type 2B spontaneous coronary artery dissection (C), Type 3 spontaneous coronary artery dissection (D), Type 4 spontaneous coronary artery dissection (E), and Intermediate Type1/2 spontaneous coronary artery dissection (F). Additional possible angiographic findings Other angiographic features reported in association with SCAD include: Increased coronary tortuosity47 Predilection for more distal coronary segments (in contrast to atherosclerotic disease)4,5,44,45 Predominant involvement of the left anterior descending coronary artery and its branches reported in most4,5,8,13,44 but not all45 series False lumen starting and/or ending at a side branch13 Absence or reduced incidence of co-existent atherosclerosis—unaffected coronaries are usually normal or near-normal13 Coronary FMD (see fibromuscular dysplasia section) Association of sites of dissection with myocardial bridging.151 Multi-vessel spontaneous coronary artery dissection Multi-vessel SCAD (Figure 6) is defined as simultaneous dissections occurring in more than one artery, without continuity, and is thus distinct from recurrent SCAD or a continuous dissection which extends into different coronary territories. It is well recognized152–156 with a reported frequency of 5–13%3–5,8,13,45 in the larger series and careful angiographic assessment of apparent non-culprit vessels during index angiography is recommended. There are no current specific studies of multi-vessel SCAD; however, the approach to management for multi-vessel SCAD should not differ from single vessel disease with each lesion being assessed for intervention on its individual merits and a preference for conservative management where possible (see conservative management section). Figure 6 View largeDownload slide Multivessel spontaneous coronary artery dissection affecting two branches of the circumflex coronary artery, the left anterior descending coronary artery and its diagonal branch (arrows). Figure 6 View largeDownload slide Multivessel spontaneous coronary artery dissection affecting two branches of the circumflex coronary artery, the left anterior descending coronary artery and its diagonal branch (arrows). Secondary iatrogenic dissection in spontaneous coronary artery dissection A single study has reported an increased risk of secondary iatrogenic dissections during angiography and percutaneous coronary intervention (PCI) in SCAD patients (2% risk during coronary angiography vs. 0.2% during non-SCAD angiography, 14.3% during PCI).157 High rates of secondary iatrogenic dissection were also reported in a second series (11 of 189 SCAD cases) but without a comparator population.3 For this reason, a meticulous co-axial catheter technique and avoidance of aggressive or deeply engaging guiding catheter designs is advised. Intracoronary imaging Most SCAD can be diagnosed angiographically and in scenarios where a conservative approach to management is feasible, coronary instrumentation should, if possible, be avoided.19,106,108 However, where diagnostic uncertainty exists or to guide coronary intervention when required, careful intracoronary imaging can be invaluable and appears safe.13,30 Because SCAD frequently affects more distal coronary segments, complete imaging of the axial extent of the false lumen may not be possible (e.g. for Type 2b SCAD). In one series only 5/11 affected cases could be imaged across the entire affected length.30 However imaging of shorter proximal segments may be sufficient for diagnostic purposes.30 Intravascular ultrasound (IVUS) and OCT provide tomographic images of the vessel wall and the coronary lumen that have proved to be of major value in the diagnosis of SCAD.13,30,31,158,159 Intracoronary imaging can also help to guide decision-making on stent size. The length of the stent may be planned according to the longitudinal extent of the false lumen with the aim of minimizing propagation of intramural haematoma (see percutaneous coronary intervention section). The reported risk of late mal-apposition on haematoma resorption due to stent under-sizing in the presence of extensive haematomas160 can also potentially be minimized by careful assessment of proximal and distal vessel dimensions with IVUS/OCT. Although there are relative advantages to each technology, OCT is generally favoured for SCAD imaging because of its higher spatial resolution.1,145 Intravascular ultrasound Intravascular ultrasound (axial resolution 150 μm) is able to differentiate atherosclerotic plaques from SCAD (Figure 7A).31,158,159,161–165 This technique readily depicts the true and the false coronary lumens and is able to demonstrate the extent of false lumen thrombosis. The principal advantage of IVUS over OCT is firstly, that blood clearance (and therefore pressurized contrast injection) is not required and secondly, IVUS has superior depth penetration, enabling complete visualization of the vessel wall to the external elastic lamina, including imaging through thrombus. The main limitation is poor spatial resolution which can limit identification of small structures associated with SCAD such as the intimal-medial membrane and localized fenestrations connecting true and false lumens.145 Figure 7 View largeDownload slide Intracoronary imaging of spontaneous coronary artery dissection by intravascular ultrasound (with outer border of false lumen arrowed, A), and optical coherence tomography showing partial (B) and circumferential (C) false lumens; the site of a fenestration (D) connecting true and false lumens and reduced light penetration through the false lumen (E). Three-dimensional image derived from segmentation of optical coherence tomography image showing how the false lumen tracks around the true lumen and is influenced (and frequently bounded) by side branches (F). Figure 7 View largeDownload slide Intracoronary imaging of spontaneous coronary artery dissection by intravascular ultrasound (with outer border of false lumen arrowed, A), and optical coherence tomography showing partial (B) and circumferential (C) false lumens; the site of a fenestration (D) connecting true and false lumens and reduced light penetration through the false lumen (E). Three-dimensional image derived from segmentation of optical coherence tomography image showing how the false lumen tracks around the true lumen and is influenced (and frequently bounded) by side branches (F). Optical coherence tomography Optical coherence tomography has the advantage of much higher spatial resolution (axial resolution 15 μm), and characteristic images of SCAD are well described (Figure 7B–E).30,42,150,159,166 Imaging with OCT necessitates blood clearance requiring a high pressure contrast injection which carries a potential risk of extension of the false lumen, especially in proximal Type I SCAD. However, reports to date suggest, with care, OCT imaging in SCAD can be conducted safety.13,30,150 Depth penetration is more limited with OCT, exacerbated by shadowing or attenuation e.g. by thrombus or haematoma. However this generally does not limit accurate diagnosis. Optical coherence tomography enables detailed characterization of: the true lumen including any associated thrombus; the size, nature, and extent of the false lumen including points where the false lumen surrounds the true lumen circumferentially; the relationship of the false lumen with side-branches; fenestrations or the more classical ‘entry tear’ connecting true and false lumens.30 Accurate measurement of key features is also practical including the thickness of the intimal-medial membrane (mean 350 μm)30 and the dimensions of the compressed true lumen. Optical coherence tomography has also been used to report areas characterized as ‘coronary FMD’ (see fibromuscular dysplasia section). It is important to recognize that the light attenuation (blackness) of the false lumen is highly variable (presumably reflecting phases in the maturation of the intramural thrombus and variability in contrast penetration of the false lumen).145 Careful assessment of the images is required to identify the classical crescentic semi-lunar false lumen (Figure 7B–E) and ensure an accurate OCT diagnosis. Optical coherence tomography is also of value in the subset of SCAD patients requiring coronary revascularization.30,167 Before any intervention it is critical to confirm, if there is any uncertainty, that the guidewire is located in the true lumen (Figure 8) as stenting into the false lumen can have serious consequences.168,–170 Long-term follow-up OCT has also been reported in a few cases showing the stages of vessel healing. In most cases restitutio ad integrum of the previously affected vessel wall can be appreciated.171 One report described an increase in the vasa vasorum density during the healing phase (median 44 days after presentation) of SCAD.172 Given the reported increased risk of iatrogenic dissection in SCAD patients, repeat imaging should only be considered where clinically necessary. Figure 8 View largeDownload slide Guidewire passage into the false lumen demonstrated by optical coherence tomography, with both wire (GW) and optical coherence tomography catheter (C) seen in the false lumen outside the compressed true lumen. Figure 8 View largeDownload slide Guidewire passage into the false lumen demonstrated by optical coherence tomography, with both wire (GW) and optical coherence tomography catheter (C) seen in the false lumen outside the compressed true lumen. Computed tomography coronary angiography There is currently limited data to support the use of computed tomography coronary angiography (CTCA) for the diagnosis of acute SCAD. However, this imaging modality is increasingly used clinically to assess acute chest pain presentations. For SCAD, it has the advantage of being non-invasive (allowing coronary assessment without the increased risk of iatrogenic dissection reported with invasive angiography. However it is limited by lower spatial resolution, of particular importance for accurate assessment and interpretation of the smaller mid-to-distal coronary territories for which SCAD has a predilection.173 Typical CTCA appearances have been described147,174 (Figure 9A) and are consistent with angiographic and OCT findings where some cases show clear-cut contrast penetration of the false lumen leading to a ‘classical’ dual lumen but many show compression of the true-lumen by haematoma but without contrast penetration. The sensitivity and specificity of CTCA as a primary diagnostic investigation for SCAD are not known and false negative findings are reported.173,175 In this context at present, patients in whom SCAD is suspected (e.g. low atherosclerotic risk young- to middle-aged females with typical symptoms and positive biomarkers) are recommended to undergo coronary angiography as the primary diagnostic investigation of choice. CTCA may however have a role in the follow-up assessment of SCAD (Figure 9B, see coronary angiography or computed tomography-coronary angiography section). Key Messages Most SCAD can be diagnosed by coronary angiography and a working knowledge of the typical angiographic findings is key. Intracoronary imaging with OCT appears safe and should be considered where there is diagnostic uncertainty. Multi-vessel SCAD is common and careful assessment for this is required during angiography. Figure 9 View largeDownload slide Coronary computed tomography findings with parallel angiographic images for a Type 2A dissection of the left anterior descending coronary artery at presentation (A) and following healing after 3-months follow-up (B). Figure 9 View largeDownload slide Coronary computed tomography findings with parallel angiographic images for a Type 2A dissection of the left anterior descending coronary artery at presentation (A) and following healing after 3-months follow-up (B). Acute management Conservative management There is good evidence that the majority of SCAD will first stabilize and then heal completely over time if managed conservatively (Supplementary material online, Figure S6).3,4,7,8,11 Revascularization in patients with SCAD is very challenging due to the presence of an underlying disrupted and friable coronary vessel wall. This is widely reported to lead to worse outcomes for PCI than in atherosclerotic coronary disease.3–5,7,8 For this reason where revascularization is not mandated (i.e. in haemodynamically stable patients with maintained distal flow in the culprit coronary and without demonstrable ongoing ischaemia) a conservative strategy is generally favoured.1,2,145 The proportion of healed cases described in contemporary series depends on the number of cases undergoing follow-up angiography and the timing and indication for repeat assessment. The US Mayo Clinic series reported on 59 patients (from 95 managed conservatively) who underwent repeat angiography for a range of reasons a median of 2.4 years after the index event. In all, 73% (43/59) were described as ‘healed’.3 Likewise, the Canadian series reported late revascularization in 3/134 conservatively managed cases but complete healing in all 79 of the remaining cases who underwent repeat angiography73; a Japanese series reported 68% healing in 28 conservatively managed patients assessed early by CTA a median of 3.4 months post event8; a Swiss series reported healing in all but one of 36 from 56 conservatively managed patients who underwent repeat angiography 6 months after the event.7 It does appear that a small number of cases initially managed conservatively subsequently require revascularization. In a recent prospective series, 9 of 272 (3.3%) patients managed conservatively required subsequent in-hospital revascularization.19 This may depend in part on the threshold for repeat investigation and intervention. It should be noted that dissections are often inherently painful and careful consideration should be given as to whether ongoing symptomatic chest pain is ischaemic in origin before proceeding to repeat angiography or intervention on a symptomatic basis.145 As the majority of cases failing a conservative management strategy occur early during follow-up, prolonged inpatient monitoring (∼5 days) in conservatively managed SCAD is suggested.1,2,148 Further prospective research is required to better understand vascular healing after SCAD and the characteristics and management approach to delayed or failed healing. Percutaneous coronary intervention Published studies consistently show an increased risk of coronary complications with PCI.3–5,7,8 In the Canadian series, revascularization procedural success was only achieved in 64% of patients and, in addition to that, only 30% of patients maintained durable results at long-term follow-up.4 In the large series from the Mayo Clinic, most patients (2/3 of the total cohort) underwent coronary revascularization during initial hospitalization.3 Coronary interventions, however, were associated with high complication rates. Procedural success was only achieved in 57% of cases. Furthermore revascularization was not associated with a reduced long-term risk of repeated revascularization or recurrent SCAD.3,8 Where ongoing ischaemia or infarction mandates intervention, interventional cardiologists should be mindful of specific additional risks associated with SCAD interventions. These include: Increased risk of secondary iatrogenic dissection Guidewire passage into the false lumen3,168–170 (Figure 8) Proximal and/or distal false lumen propagation during stent deployment33,36,38,39,41 (Figure 10) Persistent distal dissection Major side branch restriction or occlusion by propagation of haematoma Figure 10 View largeDownload slide False lumen propagation during stenting. A Type 2A dissection affecting a high obtuse marginal branch of the circumflex but with maintained distal flow (A). Stenting (B) leads to proximal haematoma migration (arrowed C). Two further stents were required in this case to fully exclude the haematoma from luminal restriction. Figure 10 View largeDownload slide False lumen propagation during stenting. A Type 2A dissection affecting a high obtuse marginal branch of the circumflex but with maintained distal flow (A). Stenting (B) leads to proximal haematoma migration (arrowed C). Two further stents were required in this case to fully exclude the haematoma from luminal restriction. Where stents are deployed, second generation drug-eluting stents (DES) are advised. Significant rates of in-stent restenosis are reported in one retrospective series [23/44 target vessel revascularization (TVR) from 87 patients managed by PCI and followed-up for a median 2.3 years], although the drug elution status of the stents used is not described.3 In a recent retrospective multicentre study of 238 SCAD patients,176 108 underwent PCI with DES or bare metal stents (BMS). Overall, 24 patients (22.2%) suffered procedural-related complications. After a median follow-up of 3 years there was a trend towards a reduction in major adverse cardiac events after DES compared with BMS (26 vs. 39%, P = 0.14) mainly driven by TVR (4 vs. 18%, P = 0.08). Although the differences did not achieve statistical significance, the trends are consistent with existing knowledge about the relative risk of BMS vs. DES in non-SCAD PCI. A recent meta-analysis also suggested an additional TVR risk of 6.3% in patients treated with revascularization.177 A single case has been reported of late strut mal-apposition purportedly due to the effects of haematoma resorption160 although to date an increased incidence of stent thrombosis has not been confirmed. Adequate stent sizing and expansion is therefore likely to be important but stent post-dilation/optimization is a balance between the risk of haematoma propagation and ensuring optimal strut expansion. Given the increased risk of adverse outcomes with PCI in SCAD, a number of less conventional interventional approaches have been reported. These include: Minimal plain old balloon angioplasty (POBA) to restore flow followed by a conservative strategy178 Extended stent lengths to reduce the chances of proximal or distal haematoma propagation Sealing the proximal and distal extremes of the affected segments with short stents to restrict the haematoma before stenting the intermediate segments.179,180 Targeting an intimal tear or ‘flap’ for focal stenting or stenting just the proximal extent of the dissection to prevent proximal propagation.8,181 Cutting balloon inflation to fenestrate the intimal-medial membrane and depressurize the false lumen as a stand-alone strategy or prior to stenting.182–185 Use of bioresorbable coronary scaffolds.184,186–188 These anecdotal case reports are subject to publication bias. Additionally, the role of bioresorbable coronary scaffolds more generally has been the subject of recent scrutiny.189 In the absence of randomized data, no specific alternative PCI strategy can at present be specifically recommended. Coronary artery bypass grafting Coronary artery bypass grafting (CABG) in SCAD is generally used as a bail-out strategy either for a failure of PCI with ongoing ischaemia or infarction of a significant at-risk myocardial territory (e.g. failure to wire the true lumen distal to a SCAD occlusion) or because the site and extent of the dissection (usually involving the left main stem or the presence of multiple dissections in different vessels) is felt to pose a prohibitive risk with either a conservative or a PCI strategy. Successful grafting may be challenging where the dissection extends beyond the graft anastomosis site and great care must be taken to ensure anastomosis to the true lumen.190 Coronary artery bypass grafting using arterial or venous conduits and both off-pump and robotic techniques are described but the literature on CABG in SCAD is limited to case reports and small case series (5–23 cases).3,6,12,14 Early outcomes from these limited data on CABG are reported to be good but the Mayo Clinic Series reported high rates of graft failure at follow-up, perhaps due to healing of the native coronary leading to competitive flow and conduit thrombosis.3 Adjunctive supportive devices and transplant For details, see Supplementary material online.191–,208 Medical management There are to date no randomized controlled trials comparing different pharmacological treatment strategies for SCAD. Current practice is therefore based on case and registry observations, clinical experience and the extrapolation (where appropriate) of guidelines for non-SCAD ACS treatment. Thrombolysis Although individual historical cases of SCAD managed apparently successfully with thrombolysis have been described,209 there are also reports of dissection extension and even coronary rupture leading to cardiac tamponade following lytic therapy.210–213 Thrombolysis is therefore contraindicated for the acute management of SCAD. Antiplatelet therapies The use of antiplatelet therapies and the duration of treatment remains an area of controversy and divergent practice in SCAD. This results from an apparent conflict between the strength of existing data of efficacy from non-SCAD ACS vs. an inherent concern (albeit unproven) about using medications that prolong bleeding time for a condition whose primary pathophysiology may be an intramural bleed.145 This may be further complicated by problematic menorrhagia which can be an issue in SCAD-survivors of menstrual age taking antiplatelet therapies.214 Patients who undergo stenting should receive dual antiplatelet therapy for 12 months and prolonged or lifelong monotherapy (usually with aspirin) in accordance with current ACS guidelines.206,207 In patients managed conservatively, there is evidence from OCT studies of high grade stenosis sometimes with true luminal thrombus in association with SCAD.30,166 This provides justification for antiplatelet therapy in the acute phase and most authors advocate acute dual antiplatelet therapy (usually with aspirin and clopidogrel rather than the newer P2Y12 inhibitors and avoiding intravenous antiplatelet therapies).19,106,108 The optimal duration of dual and subsequent monotherapy remains unknown with some authors advocating lifelong aspirin19,108 and others questioning this approach.106 Anticoagulant therapies The same concerns about the potential adverse impact described for antiplatelet therapies also apply to anticoagulant treatments. Anticoagulation should probably be limited to acute administration during revascularization procedures while chronic use should be restricted to situations where there is an unequivocal clinical indication (such as left ventricular thrombus or thromboembolism) which should over-ride what is at present a theoretical risk.145 Angiotensin converting enzyme inhibitors, angiotensin receptor antagonists, mineralocorticoid receptor antagonists, beta-blockers, and vasodilator therapies Medical management of SCAD patients with significant impairment of left ventricular systolic function should follow current guidelines aimed at maximizing angiotensin converting enzyme (ACE) or angiotensin receptor blocker (ARB) and β-blocker doses and adding in a mineralocorticoid receptor antagonist (MRA) as indicated,206,207 although hypotension frequently limits dose escalation in this younger population. More controversial is the management of SCAD-survivors without significant impairment of left ventricular systolic function. One recent multivariate analysis from a prospective cohort reported an association between hypertension and an increased risk of recurrent SCAD and β-blocker treatment with a reduced risk of recurrence.19 These findings are not from a randomized study and await validation in other cohorts215 but if confirmed provide the first evidence that SCAD recurrence risk may be reduced therapeutically. Vasodilatory therapies (e.g. nitrates or calcium channel blockers) are reserved for the empirical treatment of chest pain during the acute phase and recurrent chest pain following the index event (see post-spontaneous coronary artery dissection chest pain and its management section). Statins The rationale for prescribing statins for a condition whose pathophysiology has no known association with cholesterol is unclear. One small study reported higher statin use in patients with SCAD recurrence44 but this non-randomized finding should be interpreted with caution and an adverse signal was not reported in a larger prospective cohort.19 In general, statins are reserved for patients with conventional indications for treatment independent of their SCAD event. Contraception and hormone replacement therapy Concerns about hormonal contraception62,63 and hormone replacement therapy30 following SCAD are largely based on the presumed pathophysiological association between SCAD and female sex hormones arising from the female sex predominance and the known association of SCAD with the peripartum period.1,2 However, the exact nature of this association remains to be elucidated. A number of cases have been reported of SCAD in association with exogenous sex hormones but given the high prevalence of use in the population, a causative link has yet to be determined. At present a reasonable strategy may be to avoid hormonal contraception where possible. In patients with recurrent cyclical chest pain following SCAD, low dose local hormone delivery intrauterine contraceptive devices have been anecdotally reported to be helpful.145 The use of the levonorgestrel releasing intra uterine system (LNG-IUS) or endometrial ablation may also be considered in women with prolonged, severe menorrhagia (e.g. where antiplatelet therapies are mandated in the context of previous stenting). Key Messages Coronary revascularization is associated with an increased risk of complications and adverse outcomes compared with atherosclerotic coronary disease. Conservatively (without revascularization) managed SCAD usually heals completely over a few months. Where flow is maintained and in the absence of ongoing ischaemia or infarction, a conservative approach should be considered followed by a period of inpatient observation. Further research is needed to clarify the optimal PCI strategy in cases where revascularization is necessary. The acute and convalescent medical strategy in SCAD may have key differences from post atherosclerotic AMI and further research is needed to establish the optimal treatment approach. Pregnancy Pregnancy-associated spontaneous coronary artery dissection: special considerations Special considerations for the management of P-SCAD include the avoidance of teratogenic drugs, minimization of exposure of the unborn foetus to ionizing radiation216 and timing of delivery to minimize as much as possible risk to both mother and baby. Obstetric issues are reviewed elsewhere96 and are not considered in detail here but coronary management should not differ greatly from other SCAD patients with diagnosis by limited coronary angiography and a management preference for a conservative approach to revascularization where practical. Pregnancy after spontaneous coronary artery dissection There is a single published report of nine pregnancies in SCAD-survivors with one recurrence occurring in a patients whose first event was not peripartum.217 The degree of left ventricular impairment post-SCAD will also contribute independently to the risk. Some authors have advocated a blanket recommendation to avoid pregnancy in SCAD-survivors.1,2 Patients should certainly be carefully counselled before contemplating pregnancy, potentially teratogenic drugs discontinued, and any planned or un-planned pregnancy should be considered high risk and monitored accordingly. Key Messages P-spontaneous coronary artery dissection should be managed by a multidisciplinary team with individualized cardiovascular and obstetric management. There is limited data on the risk of pregnancy in SCAD-survivors but at present pregnancy should be considered high risk pending further research to better quantify this risk. Outcomes and follow-up Follow-up imaging Echocardiography/cardiac magnetic resonance imaging Following SCAD, as with myocardial infarction of other causes, an assessment of left ventricular systolic function is mandatory to guide medical and potentially device therapy.206,207 Computed tomography-peripheral angiography or magnetic resonance-angiography In view of the association between SCAD, FMD and other underlying EVAs, affecting multiple vascular beds (see fibromuscular dysplasia section) and the potential implications for patient management and follow-up,106 imaging of extra-coronary vascular beds in patients with SCAD is advised.101,103,106 Saw et al.1 have advocated non-selective angiography of the corresponding arteries with a pigtail catheter on the occasion of the index coronary angiography. However, this strategy has the potential risks of additional vascular imaging, catheter manipulation and prolonged procedural times in unstable patients, with possibly increased vascular fragility.106 Furthermore, this approach requires separate non-invasive imaging of cerebral aneurysms, which have been identified in 8 to 14% of cases despite a low screening rate, sometimes in the absence of typical FMD lesions.4,104,107 Alternatively, Liang et al.106 have shown the feasibility of a dedicated CTA protocol from the neck to the pelvis, with low-osmolar contrast agents and radiation dose-limiting techniques. While the sensitivity of CTA is lower than that of conventional angiography, it is unlikely to miss clinically relevant FMD lesions. MR-angiography leads to a further decrease in spatial resolution but remains a reasonable, radiation-free alternative,105 and may be preferred in case of diabetes, renal insufficiency or iodine-contrast intolerance. There is currently no specific data on follow-up of EVAs identified in SCAD patients and therefore currently this should follow best practice for similar conditions in non-SCAD patients. Coronary angiography or computed tomography-coronary angiography Whilst it is clear that the majority of conservatively managed SCAD heals completely over time, some cases of persistent dissection are reported (see conservative management section). The value of follow-up coronary imaging to determine SCAD-healing and how this should be used to guide subsequent management remains unclear. It may be relevant, for decision-making about the duration of anti-platelet therapy (see antiplatelet therapies section) or in symptomatic patients where the diagnosis is not clear (see post-spontaneous coronary artery dissection chest pain and its management section). Non-invasive imaging is attractive as invasive angiography in SCAD patients has been associated with an increased risk of iatrogenic dissections (see secondary iatrogenic dissection in spontaneous coronary artery dissection section). Computed tomography coronary angiography has been proposed as an alternative although current data are limited to single case discussions and one small series. Although the issue of spatial resolution in the smaller, more distal, SCAD coronary sites is still relevant (see computed tomography coronary angiography section), CT may have greater utility in assessing healing where the site of dissection has already been determined by angiography (Figure 9B).147,218 Roura et al.10 reported the largest series of patients with SCAD studied by multi-slice CT (MSCT) at follow-up (24 cases). In 83% of cases, complete resolution of SCAD was shown. Further data is required before CTCA can be recommended for follow-up SCAD imaging. Where essential, invasive angiography should be performed with meticulous attention to minimize the risk of iatrogenic dissection. Prognosis: mortality, recurrence risk, and major adverse cardiac events Outcomes following SCAD are summarized in Supplementary material online, Table S3.3,5,7,8,19, In patients surviving SCAD, long-term mortality is low. In the US Mayo Clinic series 10-year survival from Kaplan-Meier estimates is reported at 92%.44 Similarly an Italian series reported 94.4% 6-year survival,5 whilst a Swiss series reported no deaths after the index event in 63 patients followed-up to a median 4.5 years,7 a Japanese series reported one death from 63 patients followed up for a median 34 months8 and a prospective Canadian series report 1.2% mortality at median follow-up 3.1 years.19 However this masks significant morbidity. The overall major adverse cardiac events (MACE) rate in SCAD patients is significant but with considerable variation between published series (47.4% MACE over 10-years from Kaplan–Meier estimates in the US series;44 MACE in the prospective Canadian series was 19.9% over median 3.1 years follow-up;19 5-year MACE in the Japanese series was 37%;8 in the Italian series 6-year MACE was 14.6%).5 This is primarily driven by recurrent dissections and a high rate of target vessel failure in patients undergoing PCI (see percutaneous coronary intervention section).3 Recurrence in SCAD has been widely reported.1,3–5,7,8,44 The US series reported SCAD recurrence in 17% of patients across a median follow-up period of 47 months with a 10-year recurrence rate of 29.4% [the median time to a second event was 2.8 years (ranging from 3 days to 12 years)]44; the Canadian prospective series report recurrent de novo SCAD in 10.4% of 327 patients followed up for a median 3.1 years and a recurrent MI rate of 16.8% in the same cohort19; the Japanese series reported seven recurrent SCAD after the first 30-days from the index event from 63 patients followed up for a median 34 months8; the Swiss series report 3/63 recurrent SCAD followed-up for a median 4.5 years7; the Italian series report 4.7% recurrence over a median 22 month follow-up].5 Recurrence often appears to affect new territories (e.g. in 12/15 patients in the US series)44 and stenting at the time of the first event does not appear to be protective. Although reported rates may represent a slight overestimation of true recurrence rates due to the selection bias inherent to self-referral based cohort studies and prospective data is needed, recurrences are certainly a justified concern in SCAD patients. One study has reported a borderline association between recurrence and increased coronary tortuosity, although it is unclear if tortuosity is a marker of an underlying vasculopathy or provides a mechanism for arterial injury.47 Apart from the potential benefit of betablockers and control of hypertension previously described, no current treatment strategy has, to date, been shown to reduce rates of recurrence. Post spontaneous coronary artery dissection chest pain and its management Recurrent chest pain, often with associated hospital admission, is common after SCAD. In some patients symptoms occur cyclically, usually pre-menstrually.219 Given the SCAD recurrence risk, patients presenting with recurrent chest pain require careful assessment with serial electrocardiography (ECG) and high sensitivity troponin measurement. However, given the reported increased risk of secondary iatrogenic dissection in SCAD patients (see secondary iatrogenic dissection in spontaneous coronary artery dissection section), invasive angiography should be reserved for patients with hard evidence of ischaemia or myocardial necrosis. A role for CTCA to rule out recurrent SCAD, when evaluating post SCAD chest pain, although potentially attractive, remains to be clearly elucidated (see coronary angiography or computed tomography-coronary angiography section). Anecdotally, where recurrent SCAD has been excluded, symptoms may respond to a treatment strategy aimed at reducing vasospasm with vasodilator therapies (where left ventricular function is normal reducing blood pressure lowering medications may be necessary to allow the initiation of vasodilators).145 Likewise it has been reported that cyclical symptoms may respond to low dose contraception (e.g. the progesterone hormonal coil).145 Cardiac rehabilitation and exercise For details, see Supplementary material online.220,221 Post-traumatic stress disorder and the emotional and psychological consequences of spontaneous coronary artery dissection For details, see Supplementary material online.220,222 Key Messages Although the prognosis following SCAD appears good, recurrent SCAD is well recognized. Assessment for extra-coronary arteriopathies is advised in SCAD-survivors. Cardiac rehabilitation should be considered in SCAD patients and a return to full-activity with an avoidance of extreme or isometric exercise encouraged. Recurrent chest pain after SCAD is common and requires careful assessment and management. Management considerations Whilst there are several areas in the management of patients with SCAD where the optimal approach remains uncertain and research is needed to provide evidence to justify firm recommendations (see research priorities section), the following considerations may be useful for clinicians: Early coronary angiography should be considered to exclude the diagnosis of SCAD in patients presenting with clinical features of ACS but at low risk of atherosclerotic AMI, in particular young to middle aged women Where angiographic diagnosis of SCAD is uncertain, intracoronary imaging with OCT should be considered In clinically stable patients with maintained coronary flow, a conservative management strategy is preferred because of the increased risk of adverse outcomes with revascularization In patients with confirmed SCAD imaging for extra-coronary arteriopathies is advised A diagnosis of SCAD should be actively sought at post mortem in unexplained cases of sudden cardiac death by careful assessment of the full length of the coronary tree Research priorities Despite huge progress in the clinical characterization of SCAD from an international effort to register, assess and follow-up patients, this remains a condition for which the pathophysiology is poorly understood and for which, despite a significant recurrence risk, there is no specific disease-modifying therapy. Current international efforts are focused on building large prospectively recruited multicentre cohorts, phenotyped by state-of-the-art imaging for pathophysiological (including genetic) and clinical studies. For example, the European Observational Research Platform (EORP) SCAD study will open for recruitment in the summer of 2018. Prospective studies (including ultimately randomized studies) assessing the best medical therapies (e.g. role and duration of antiplatelet therapy, use of beta-blockers and other secondary prevention drugs) as well as the optimal coronary intervention strategy are urgently needed. It is hoped these ongoing collaborative research efforts will shed new light and expand knowledge on this relatively rare, elusive and challenging clinical entity. Acknowledgements We wish to acknowledge our European patients with SCAD, EURODIS, BeatSCAD; the ESC-ACCA SCAD Study Group and the ACCA secretariat—in particular Eugenie Delaveau and Celine Serio; Dr J. Cade for the image in Figure 9, Dr S Parsons for Figure 2. A Persu is grateful to Prof. Patrick Chenu (Cardiology Department, Cliniques Universitaires Saint Luc, UCL, Brussels, Belgium) for sharing his knowledge and experience on Spontaneous Coronary Artery Dissection (SCAD) and to Mr Xiang Li, medical student at the same University for his contribution to Figure 4 and literature review. Funding British Heart Foundation, the NIHR RD-TRC and the Leicester NIHR Biomedical Research Centre to D.A. Conflict of interest: D.A. declares grants from Astra Zeneca and St Jude Medical (now part of Abbott Vascular) as well as grants from the British Heart Foundation, NIHR RD-TRC and BeatSCAD. H.B. reports grants and personal fees from ASTRA ZENECA, personal fees from DAICHII-SANKYO, personal fees from ELI-LILLY, personal fees from BAYER, personal fees from SANOFI, during the conduct of the study; personal fees from NOVARTIS, personal fees from BMS-PFIZER, from SERVIER, outside the submitted work. Footnotes The opinions expressed in this article are not necessarily those of the Editors of the European Heart Journal or of the European Society of Cardiology. References 1 Saw J , Mancini GB , Humphries KH. Contemporary review on spontaneous coronary artery dissection . J Am Coll Cardiol 2016 ; 68 : 297 – 312 . Google Scholar Crossref Search ADS PubMed 2 Tweet MS , Gulati R , Hayes SN. Spontaneous coronary artery dissection . Curr Cardiol Rep 2016 ; 18 : 60. Google Scholar Crossref Search ADS PubMed 3 Tweet MS , Eleid MF , Best PJ , Lennon RJ , Lerman A , Rihal CS , Holmes DR Jr. , Hayes SN , Gulati R. Spontaneous coronary artery dissection: revascularization versus conservative therapy . Circ Cardiovasc Interv 2014 ; 7 : 777 – 786 . Google Scholar Crossref Search ADS PubMed 4 Saw J , Aymong E , Sedlak T , Buller CE , Starovoytov A , Ricci D , Robinson S , Vuurmans T , Gao M , Humphries K , Mancini GB. Spontaneous coronary artery dissection: association with predisposing arteriopathies and precipitating stressors and cardiovascular outcomes . Circ Cardiovasc Interv 2014 ; 7 : 645 – 655 . Google Scholar Crossref Search ADS PubMed 5 Lettieri C , Zavalloni D , Rossini R , Morici N , Ettori F , Leonzi O , Latib A , Ferlini M , Trabattoni D , Colombo P , Galli M , Tarantini G , Napodano M , Piccaluga E , Passamonti E , Sganzerla P , Ielasi A , Coccato M , Martinoni A , Musumeci G , Zanini R , Castiglioni B. Management and long-term prognosis of spontaneous coronary artery dissection . Am J Cardiol 2015 ; 116 : 66 – 73 . Google Scholar Crossref Search ADS PubMed 6 Faden MS , Bottega N , Benjamin A , Brown RN. A nationwide evaluation of spontaneous coronary artery dissection in pregnancy and the puerperium . Heart 2016 ; 102 : 1974 – 1979 . Google Scholar Crossref Search ADS PubMed 7 Rogowski S , Maeder MT , Weilenmann D , Haager PK , Ammann P , Rohner F , Joerg L , Rickli H. Spontaneous coronary artery dissection: angiographic follow-up and long-term clinical outcome in a predominantly medically treated population . Catheter Cardiovasc Interv 2017 ; 89 : 59 – 68 . Google Scholar Crossref Search ADS PubMed 8 Nakashima T , Noguchi T , Haruta S , Yamamoto Y , Oshima S , Nakao K , Taniguchi Y , Yamaguchi J , Tsuchihashi K , Seki A , Kawasaki T , Uchida T , Omura N , Kikuchi M , Kimura K , Ogawa H , Miyazaki S , Yasuda S. Prognostic impact of spontaneous coronary artery dissection in young female patients with acute myocardial infarction: a report from the Angina Pectoris-Myocardial Infarction Multicenter Investigators in Japan . Int J Cardiol 2016 ; 207 : 341 – 348 . Google Scholar Crossref Search ADS PubMed 9 McGrath-Cadell L , McKenzie P , Emmanuel S , Muller DW , Graham RM , Holloway CJ. Outcomes of patients with spontaneous coronary artery dissection . Open Heart 2016 ; 3 : e000491. Google Scholar Crossref Search ADS PubMed 10 Roura G , Ariza-Sole A , Rodriguez-Caballero IF , Gomez-Lara J , Ferreiro JL , Romaguera R , Teruel L , de Albert M , Gomez-Hospital JA , Cequier A. Noninvasive follow-up of patients with spontaneous coronary artery dissection with CT angiography . JACC Cardiovasc Imaging 2016 ; 9 : 896 – 897 . Google Scholar Crossref Search ADS PubMed 11 Alfonso F , Paulo M , Lennie V , Dutary J , Bernardo E , Jiménez-Quevedo P , Gonzalo N , Escaned J , Bañuelos C , Pérez-Vizcayno MJ , Hernández R , Macaya C. Spontaneous coronary artery dissection: long-term follow-up of a large series of patients prospectively managed with a “conservative” therapeutic strategy . JACC Cardiovasc Interv 2012 ; 5 : 1062 – 1070 . Google Scholar Crossref Search ADS PubMed 12 Ito H , Taylor L , Bowman M , Fry ET , Hermiller JB , Van Tassel JW. Presentation and therapy of spontaneous coronary artery dissection and comparisons of postpartum versus nonpostpartum cases . Am J Cardiol 2011 ; 107 : 1590 – 1596 . Google Scholar Crossref Search ADS PubMed 13 Motreff P , Malcles G , Combaret N , Barber-Chamoux N , Bouajila S , Pereira B , Amonchot A , Citron B , Lusson JR , Eschalier R , Souteyrand G. How and when to suspect spontaneous coronary artery dissection: novel insights from a single-centre series on prevalence and angiographic appearance . EuroIntervention 2017 ; 12 : e2236 – e2243 . Google Scholar Crossref Search ADS PubMed 14 Vanzetto G , Berger-Coz E , Barone-Rochette G , Chavanon O , Bouvaist H , Hacini R , Blin D , Machecourt J. Prevalence, therapeutic management and medium-term prognosis of spontaneous coronary artery dissection: results from a database of 11, 605 patients . Eur J Cardiothorac Surg 2009 ; 35 : 250 – 254 . Google Scholar Crossref Search ADS PubMed 15 Mortensen KH , Thuesen L , Kristensen IB , Christiansen EH. Spontaneous coronary artery dissection: a Western Denmark Heart Registry Study . Catheter Cardiovasc Intervent 2009 ; 74 : 710 – 717 . Google Scholar Crossref Search ADS 16 Rashid HN , Wong DT , Wijesekera H , Gutman SJ , Shanmugam VB , Gulati R , Malaipan Y , Meredith IT , Psaltis PJ. Incidence and characterisation of spontaneous coronary artery dissection as a cause of acute coronary syndrome—a single-centre Australian experience . Int J Cardiol 2016 ; 202 : 336 – 338 . Google Scholar Crossref Search ADS PubMed 17 Barber-Chamoux N , Souteyrand G , Combaret N , Ouedraogo E , Lusson JR , Motreff P. Contribution of optical coherence tomography imaging in management of iatrogenic coronary dissection . Cardiovasc Revasc Med 2016 ; 17 : 138 – 142 . Google Scholar Crossref Search ADS PubMed 18 Elkayam U , Jalnapurkar S , Barakkat MN , Khatri N , Kealey AJ , Mehra A , Roth A. Pregnancy-associated acute myocardial infarction: a review of contemporary experience in 150 cases between 2006 and 2011 . Circulation 2014 ; 129 : 1695 – 1702 . Google Scholar Crossref Search ADS PubMed 19 Saw J , Humphries K , Aymong E , Sedlak T , Prakash R , Starovoytov A , Mancini GBJ. Spontaneous coronary artery dissection: clinical outcomes and risk of recurrence . J Am Coll Cardiol 2017 ; 70 : 1148 – 1158 . Google Scholar Crossref Search ADS PubMed 20 Basso C , Aguilera B , Banner J , Cohle S , d'Amati G , de Gouveia RH , di Gioia C , Fabre A , Gallagher PJ , Leone O , Lucena J , Mitrofanova L , Molina P , Parsons S , Rizzo S , Sheppard MN , Mier MPS , Kim Suvarna S , Thiene G , van der Wal A , Vink A , Michaud K. Association for European Cardiovascular P . Guidelines for autopsy investigation of sudden cardiac death: 2017 update from the Association for European Cardiovascular Pathology . Virchows Arch 2017 ; 471 : 691 – 705 . Google Scholar Crossref Search ADS PubMed 21 Desai S , Sheppard MN. Sudden cardiac death: look closely at the coronaries for spontaneous dissection which can be missed. A study of 9 cases . Am J Forensic Med Pathol 2012 ; 33 : 26 – 29 . Google Scholar Crossref Search ADS PubMed 22 Hill SF , Sheppard MN. Non-atherosclerotic coronary artery disease associated with sudden cardiac death . Heart 2010 ; 96 : 1119 – 1125 . Google Scholar Crossref Search ADS PubMed 23 Mandal R , Brooks EG , Corliss RF. Eosinophilic coronary periarteritis with arterial dissection: the mast cell hypothesis . J Forensic Sci 2015 ; 60 : 1088 – 1092 . Google Scholar Crossref Search ADS PubMed 24 Melez İE , Arslan MN , Melez DO , Akçay A , Büyük Y , Avşar A , Kumral B , Şirin G , Karayel FA , Daş T , Dokudan YE , Şam B. Spontaneous coronary artery dissection: report of 3 cases and literature review hormonal, autoimmune, morphological factors . Am J Forensic Med Pathol 2015 ; 36 : 188 – 192 . Google Scholar Crossref Search ADS PubMed 25 Stoukas V , Dragovic LJ. Sudden deaths from eosinophilic coronary monoarteritis: a subset of spontaneous coronary artery dissection . Am J Forensic Med Pathol 2009 ; 30 : 268 – 269 . Google Scholar Crossref Search ADS PubMed 26 Basso C , Morgagni GL , Thiene G. Spontaneous coronary artery dissection: a neglected cause of acute myocardial ischaemia and sudden death . Heart 1996 ; 75 : 451 – 454 . Google Scholar Crossref Search ADS PubMed 27 Robinowitz M , Virmani R , McAllister HAJ. Spontaneous coronary artery dissection and eosinophilic inflammation: a cause and effect relationship? Am J Med 1982 ; 72 : 923 – 928 . Google Scholar Crossref Search ADS PubMed 28 Alfonso F , Bastante T. Spontaneous coronary artery dissection: novel diagnostic insights from large series of patients . Circ Cardiovasc Interv 2014 ; 7 : 638 – 641 . Google Scholar Crossref Search ADS PubMed 29 Vrints CJ. Spontaneous coronary artery dissection . Heart 2010 ; 96 : 801 – 808 . Google Scholar Crossref Search ADS PubMed 30 Alfonso F , Paulo M , Gonzalo N , Dutary J , Jimenez-Quevedo P , Lennie V , Escaned J , Banuelos C , Hernandez R , Macaya C. Diagnosis of spontaneous coronary artery dissection by optical coherence tomography . J Am Coll Cardiol 2012 ; 59 : 1073 – 1079 . Google Scholar Crossref Search ADS PubMed 31 Paulo M , Sandoval J , Lennie V , Dutary J , Medina M , Gonzalo N , Jimenez-Quevedo P , Escaned J , Banuelos C , Hernandez R , Macaya C , Alfonso F. Combined use of OCT and IVUS in spontaneous coronary artery dissection . JACC Cardiovasc Imaging 2013 ; 6 : 830 – 832 . Google Scholar Crossref Search ADS PubMed 32 Fengping Y , Jue H , Qingchun Y , Fangxing H. A case of sudden death due to spontaneous coronary artery dissection . Am J Forensic Med Pathol 2011 ; 32 : 312 – 313 . Google Scholar Crossref Search ADS PubMed 33 Kanaroglou S , Nair V , Fernandes JR. Sudden cardiac death due to coronary artery dissection as a complication of cardiac sarcoidosis . Cardiovasc Pathol 2015 ; 24 : 244 – 246 . Google Scholar Crossref Search ADS PubMed 34 Lunebourg A , Letovanec I , Eggenberger P , Lehr HA. Images in cardiovascular medicine. Sudden cardiac death due to triple vessel coronary dissection . Circulation 2008 ; 117 : 2038 – 2040 . Google Scholar Crossref Search ADS PubMed 35 Wei JP , Kay D , Fishbein MC. Spontaneous dissection of the distal obtuse marginal coronary artery: a rare cause of sudden death . Am J Forensic Med Pathol 2008 ; 29 : 199 – 201 . Google Scholar Crossref Search ADS PubMed 36 Carreon CK , Esposito MJ. Eosinophilic coronary monoarteritis . Arch Pathol Lab Med 2014 ; 138 : 979 – 981 . Google Scholar Crossref Search ADS PubMed 37 Asuncion CM , Hyun J. Dissecting intramural hematoma of the coronary artery in pregnancy and the puerperium . Obstet Gynecol 1972 ; 40 : 202 – 210 . Google Scholar PubMed 38 Conraads VM , Vorlat A , Colpaert CG , Rodrigus IE , De Paep RJ , Moulijn AC , Vrints CJ. Spontaneous dissection of three major coronary arteries subsequent to cystic medial necrosis . Chest 1999 ; 116 : 1473 – 1475 . Google Scholar Crossref Search ADS PubMed 39 Madu EC , Kosinski DJ , Wilson WR , Burket MW , Fraker TD Jr , Ansel GM. Two-vessel coronary artery dissection in the peripartum period. Case report and literature review . Angiology 1994 ; 45 : 809 – 816 . Google Scholar Crossref Search ADS PubMed 40 Makino Y , Inokuchi G , Yokota H , Hayakawa M , Yajima D , Motomura A , Chiba F , Torimitsu S , Nakatani Y , Iwase H. Sudden death due to coronary artery dissection associated with fibromuscular dysplasia revealed by postmortem selective computed tomography coronary angiography: a case report . Forensic Sci Int 2015 ; 253 : e10 – e15 . Google Scholar Crossref Search ADS PubMed 41 Mortensen KH , Thuesen L , Kristiensen IB , Christiansen EH. Spontaneous coronary artery dissection - a western Denmark heart registry study . Eur Heart J 2009 ; 30 : 787 – 787 . 42 Nishiguchi T , Tanaka A , Ozaki Y , Taruya A , Fukuda S , Taguchi H , Iwaguro T , Ueno S , Okumoto Y , Akasaka T. Prevalence of spontaneous coronary artery dissection in patients with acute coronary syndrome . Eur Heart J Acute Cardiovasc Care 2016 ; 5 : 263 – 270 . Google Scholar Crossref Search ADS PubMed 43 Saw J , Aymong E , Mancini GB , Sedlak T , Starovoytov A , Ricci D. Nonatherosclerotic coronary artery disease in young women . Can J Cardiol 2014 ; 30 : 814 – 819 . Google Scholar Crossref Search ADS PubMed 44 Tweet MS , Hayes SN , Pitta SR , Simari RD , Lerman A , Lennon RJ , Gersh BJ , Khambatta S , Best PJ , Rihal CS , Gulati R. Clinical features, management, and prognosis of spontaneous coronary artery dissection . Circulation 2012 ; 126 : 579 – 588 . Google Scholar Crossref Search ADS PubMed 45 Rogowski S , Maeder MT , Weilenmann D , Haager PK , Ammann P , Rohner F , Joerg L , Rickli H. Spontaneous coronary artery dissection: angiographic follow-up and long-term clinical outcome in a predominantly medically treated population . Catheter Cardiovasc Interv 2017 ; 89 : 59 – 68 . Google Scholar Crossref Search ADS PubMed 46 Bush N , Nelson-Piercy C , Spark P , Kurinczuk JJ , Brocklehurst P , Knight M. Myocardial infarction in pregnancy and postpartum in the UK . Eur J Prev Cardiol 2013 ; 20 : 12 – 20 . Google Scholar Crossref Search ADS PubMed 47 Eleid MF , Guddeti RR , Tweet MS , Lerman A , Singh M , Best PJ , Vrtiska TJ , Prasad M , Rihal CS , Hayes SN , Gulati R. Coronary artery tortuosity in spontaneous coronary artery dissection: angiographic characteristics and clinical implications . Circ Cardiovasc Interv 2014 ; 7 : 656 – 662 . Google Scholar Crossref Search ADS PubMed 48 Huikuri HV , Mallon SM , Myerburg RJ. Cardiac arrest due to spontaneous coronary artery dissection in a patient with coronary ectasia—a case report . Angiology 1991 ; 42 : 148 – 151 . Google Scholar Crossref Search ADS PubMed 49 Mieghem CA , Ligthart JM , Cademartiri F. Images in cardiology . Spontaneous dissection of the left main coronary artery in a patient with Osler-Weber-Rendu disease . Heart 2005 ; 92 : 394. Google Scholar Crossref Search ADS 50 Henkin S , Negrotto SM , Tweet MS , Kirmani S , Deyle DR , Gulati R , Olson TM , Hayes SN. Spontaneous coronary artery dissection and its association with heritable connective tissue disorders . Heart 2016 ; 102 : 876 – 881 . Google Scholar Crossref Search ADS PubMed 51 Sato C , Wakabayashi K , Suzuki H. Natural course of isolated spontaneous coronary artery dissection in Marfan syndrome . Int J Cardiol 2014 ; 177 : 20 – 22 . Google Scholar Crossref Search ADS PubMed 52 Fattori R , Sangiorgio P , Mariucci E , Ritelli M , Wischmeijer A , Greco C , Colombi M. Spontaneous coronary artery dissection in a young woman with Loeys-Dietz syndrome . Am J Med Genet A 2012 ; 158A : 1216 – 1218 . Google Scholar Crossref Search ADS PubMed 53 Hampole CV , Philip F , Shafii A , Pettersson G , Anesi GL , Patel JB , Menon V. Spontaneous coronary artery dissection in Ehlers-Danlos syndrome . Ann Thorac Surg 2011 ; 92 : 1883 – 1884 . Google Scholar Crossref Search ADS PubMed 54 Giugliano GR , Sethi PS. Spontaneous left anterior descending coronary artery dissection in a patient with neurofibromatosis . J Invasive Cardiol 2009 ; 21 : e103 – e105 . Google Scholar PubMed 55 Sibon I , Sommer P , Lamaziere JM , Bonnet J. Lysyl oxidase deficiency: a new cause of human arterial dissection . Heart 2005 ; 91 : e33. Google Scholar Crossref Search ADS PubMed 56 Martín Dávila F , Delgado Portela M , García Rojo M , González García J , Puig Rullán AM , López Pérez R , Carbajo Vicente M. Coronary artery dissection in alpha-1-antitrypsin deficiency . Histopathology 1999 ; 34 : 376 – 378 . Google Scholar Crossref Search ADS PubMed 57 Diez-del Hoyo F , Sanz-Ruiz R , Diez-Villanueva P , Nunez-Garcia A , Casado-Plasencia A , Angulo-Llanos R , Clavero-Olmos M , Elizaga Corrales J , Fernandez-Aviles F. A novel cardiovascular presentation of Alport Syndrome: spontaneous coronary artery dissection . Int J Cardiol 2014 ; 177 : e133 – e134 . Google Scholar Crossref Search ADS PubMed 58 Itty CT , Farshid A , Talaulikar G. Spontaneous coronary artery dissection in a woman with polycystic kidney disease . Am J Kidney Dis 2009 ; 53 : 518 – 521 . Google Scholar Crossref Search ADS PubMed 59 Marcoff L , Rahman E. Menstruation-associated spontaneous coronary artery dissection . J Invasive Cardiol 2010 ; 22 : E183 – E185 . Google Scholar PubMed 60 Iltumur K , Karahan Z , Ozmen S , Danis R , Toprak N. Spontaneous coronary artery dissection during hemodialysis in the post-abortion period . Int J Cardiol 2008 ; 127 : e45 – e47 . Google Scholar Crossref Search ADS PubMed 61 Luceri S , Paolillo V,D , Benedictis M , Scrocca I. Spontaneous dissection of the left coronary tree after an interruption of pregnancy treated with extensive stenting . J Invasive Cardiol 2006 ; 18 : E117 – E120 . Google Scholar PubMed 62 Azam MN , Roberts DH , Logan WF. Spontaneous coronary artery dissection associated with oral contraceptive use . Int J Cardiol 1995 ; 48 : 195 – 198 . Google Scholar Crossref Search ADS PubMed 63 Zehir R , Karabay CY , Kocabay G. Myocardial infarction and spontaneous dissection of coronary artery due to oral contraceptive . J Cardiovasc Med (Hagerstown) 2011 ; 12 : 448 – 450 . Google Scholar Crossref Search ADS PubMed 64 Pan AL , Fergusson D , Hong R , Badawi RA. Spontaneous coronary artery dissection following topical hormone replacement therapy . Case Rep Cardiol 2012 ; 2012 : 1. Google Scholar Crossref Search ADS 65 Abuzeyad FH , Ibnaouf ES , Farras MA. Clomiphene associated inferior STEMI in a young female due to right coronary artery Dissection . Case Rep Emerg Med 2017 ; 2017 : 1. Google Scholar Crossref Search ADS 66 Lempereur M , Grewal J , Saw J. Spontaneous coronary artery dissection associated with beta-HCG injections and fibromuscular dysplasia . Can J Cardiol 2014 ; 30 : 464 e1 – 463 . Google Scholar Crossref Search ADS 67 Mirra M , Kola N , Mattiello G , Morisco C , Spinelli L. Spontaneous coronary artery dissection in a young woman with polycystic ovarian syndrome . Am J Emerg Med 2017 ; 35 : 936 e5 – 936 e7 . Google Scholar Crossref Search ADS 68 Aldoboni AH , Hamza EA , Majdi K , Ngibzadhe M , Palasaidi S , Moayed DA. Spontaneous dissection of coronary artery treated by primary stenting as the first presentation of systemic lupus erythematosus . J Invasive Cardiol 2002 ; 14 : 694 – 696 . Google Scholar PubMed 69 Srinivas M , Basumani P , Muthusamy R , Wheeldon N. Active inflammatory bowel disease and coronary artery dissection . Postgrad Med J 2005 ; 81 : 68 – 70 . Google Scholar Crossref Search ADS PubMed 70 Chu KH , Menapace FJ , Blankenship JC , Hausch R , Harrington T. Polyarteritis nodosa presenting as acute myocardial infarction with coronary dissection . Cathet Cardiovasc Diagn 1998 ; 44 : 320 – 324 . Google Scholar Crossref Search ADS PubMed 71 Hunsaker JC 3rd , O'Connor WN , Lie JT. Spontaneous coronary arterial dissection and isolated eosinophilic coronary arteritis: sudden cardiac death in a patient with a limited variant of Churg-Strauss syndrome . Mayo Clin Proc 1992 ; 67 : 761 – 766 . Google Scholar Crossref Search ADS PubMed 72 Shah AH , Kinnaird TD. Recurrent ST elevation myocardial infarction: what is the aetiology? Heart Lung Circ 2015 ; 24 : e169 – e172 . Google Scholar Crossref Search ADS PubMed 73 Saw J , Aymong E , Sedlak T , Buller CE , Starovoytov A , Ricci D , Robinson S , Vuurmans T , Gao M , Humphries K , Mancini GBJ. Spontaneous coronary artery dissection: association with predisposing arteriopathies and precipitating stressors and cardiovascular outcomes . Circulation 2014 ; 7 : 645 – 655 . Google Scholar PubMed 74 Gerede DM , Yüksel B , Tutar E , Küçükşahin O , Uzun Ç , Atasoy KÇ , Düzgün N , Bengisun U. Spontaneous coronary artery dissection in a male patient with Takayasu’s arteritis and antiphospholipid antibody syndrome . Case Rep Rheumatol 2013 ; 2013 : 1. Google Scholar Crossref Search ADS 75 Ionescu CN , Chrissoheris M , Caraccciolo EA. Spontaneous coronary artery dissection and severe hypothyroidism . J Invasive Cardiol 2009 ; 21 : E60 – E62 . Google Scholar PubMed 76 Bayar N , Çağırcı G , Üreyen ÇM , Kuş G , Küçükseymen S , Arslan Ş. The relationship between spontaneous multi-vessel coronary artery dissection and celiac disease . Korean Circ J 2015 ; 45 : 242 – 244 . Google Scholar Crossref Search ADS PubMed 77 Kay IP , Williams MJ. Spontaneous coronary artery dissection: long stenting in a patient with polycythemia vera . Int J Cardiovasc Intervent 1999 ; 2 : 191 – 193 . Google Scholar Crossref Search ADS PubMed 78 Tsujita K , Miyazaki T , Kaikita K , Chitose T , Takaoka N , Soejima H , Tayama S , Hokimoto S , Sugiyama S , Ogawa H. Premenopausal woman with acute myocardial infarction caused by spontaneous coronary artery dissection and potential association with coronary vasospasm . Cardiovasc Interv Ther 2012 ; 27 : 121 – 126 . Google Scholar Crossref Search ADS PubMed 79 Jaffe BD , Broderick TM , Leier CV. Cocaine-induced coronary-artery dissection . N Engl J Med 1994 ; 330 : 510 – 511 . Google Scholar Crossref Search ADS PubMed 80 Suh SY , Kim JW , Choi CU , Kim EJ , Rha SW , Park CG , Seo HS , Oh DJ. Spontaneous coronary dissection associated with sleep deprivation presenting with acute myocardial infarction . Int J Cardiol 2007 ; 115 : e78 – e79 . Google Scholar Crossref Search ADS PubMed 81 Velusamy M , Fisherkeller M , Keenan ME , Kiernan FJ , Fram DB. Spontaneous coronary artery dissection in a young woman precipitated by retching . J Invasive Cardiol 2002 ; 14 : 198 – 201 . Google Scholar PubMed 82 Schifferdecker B , Pacifico L , Ramsaran EK , Folland ED , Spodick DH , Weiner BH. Spontaneous coronary artery dissection associated with sexual intercourse . Am J Cardiol 2004 ; 93 : 1323 – 1324 . Google Scholar Crossref Search ADS PubMed 83 Tsimikas S , Giordano FJ , Tarazi RY , Beyer RW. Spontaneous coronary artery dissection in patients with renal transplantation . J Invasive Cardiol 1999 ; 11 : 316 – 321 . Google Scholar PubMed 84 Mallon DH , McKenzie D , Dayer M. A spontaneous coronary arterial dissection associated with a calcineurin inhibitor . BMJ Case Rep 2012 ; 2012 : bcr2012006414. Google Scholar Crossref Search ADS PubMed 85 Abbott JD , Curtis JP , Murad K , Kramer HM , Remetz MS , Setaro JF , Brennan JJ. Spontaneous coronary artery dissection in a woman receiving 5-fluorouracil—a case report . Angiology 2003 ; 54 : 721 – 724 . Google Scholar Crossref Search ADS PubMed 86 Goli AK , Koduri M , Haddadin T , Henry PD. Spontaneous coronary artery dissection in a woman on fenfluramine . Rev Cardiovasc Med 2007 ; 8 : 41 – 44 . Google Scholar PubMed 87 Keir ML , Dehghani P. Corticosteroids and spontaneous coronary artery dissection: a new predisposing factor? Can J Cardiol 2016 ; 32 : 395 e7 – 398 . Google Scholar Crossref Search ADS 88 Mahmood MM , Wright RA. Spontaneous coronary artery dissection in a patient on methylphenidate for attention deficit hyperactivity disorder . Int J Cardiol 2016 ; 222 : 830 – 831 . Google Scholar Crossref Search ADS PubMed 89 Garcia Garcia C , Casanovas N , Recasens L , Miranda F , Bruguera J. Spontaneous coronary artery dissection in ergotamine abuse . Int J Cardiol 2007 ; 118 : 410 – 411 . Google Scholar Crossref Search ADS PubMed 90 Saunders SL , Ford SE. Primary coronary artery dissection possibly related to drug hypersensitivity in a male . Can J Cardiol 1991 ; 7 : 138 – 140 . Google Scholar PubMed 91 Karabinos I , Papadopoulos A , Koulouris S , Kranidis A , Korovesis S , Katritsis D. Spontaneous coronary artery dissection during a dobutamine stress echocardiography . Echocardiography 2006 ; 23 : 232 – 234 . Google Scholar Crossref Search ADS PubMed 92 Fahmy P , Prakash R , Starovoytov A , Boone R , Saw J. Pre-disposing and precipitating factors in men with spontaneous coronary artery dissection . JACC Cardiovasc Interv 2016 ; 9 : 866 – 868 . Google Scholar Crossref Search ADS PubMed 93 Vijayaraghavan R , Verma S , Gupta N , Saw J. Pregnancy-related spontaneous coronary artery dissection . Circulation 2014 ; 130 : 1915 – 1920 . Google Scholar Crossref Search ADS PubMed 94 Cade JR , Szarf G , de Siqueira ME , Chaves A , Andrea JC , Figueira HR , Gomes MM Jr , Freitas BP , Filgueiras Medeiros J , Dos Santos MR , Fiorotto WB , Daige A , Goncalves R , Cantarelli M , Alves CM , Echenique L , de Brito FS Jr , Perin MA , Born D , Hecht H , Caixeta A. Pregnancy-associated spontaneous coronary artery dissection: insights from a case series of 13 patients . Eur Heart J Cardiovasc Imaging 2017 ; 18 : 54 – 61 . Google Scholar Crossref Search ADS PubMed 95 Tweet MS , Hayes SN , Codsi E , Gulati R , Rose CH , Best PJM. Spontaneous coronary artery dissection associated with pregnancy . J Am Coll Cardiol 2017 ; 70 : 426 – 435 . Google Scholar Crossref Search ADS PubMed 96 Codsi E , Tweet MS , Rose CH , Arendt KW , Best PJ , Hayes SN. Spontaneous coronary artery dissection in pregnancy: what every obstetrician should know . Obstet Gynecol 2016 ; 128 : 731 – 738 . Google Scholar Crossref Search ADS PubMed 97 Appleby CE , Barolet A , Ing D , Ross J , Schwartz L , Seidelin P , Silversides C , Horlick E. Contemporary management of pregnancy-related coronary artery dissection: a single-centre experience and literature review . Exp Clin Cardiol 2009 ; 14 : e8 – e16 . Google Scholar PubMed 98 Koul AK , Hollander G , Moskovits N , Frankel R , Herrera L , Shani J. Coronary artery dissection during pregnancy and the postpartum period: two case reports and review of literature . Catheter Cardiovasc Interv 2001 ; 52 : 88 – 94 . Google Scholar Crossref Search ADS PubMed 99 Havakuk O , Goland S , Mehra A , Elkayam U. Pregnancy and the risk of spontaneous coronary artery dissection: an analysis of 120 contemporary cases . Circ Cardiovasc Interv 2017 ; 10 : e004941. Google Scholar Crossref Search ADS PubMed 100 Olin JW , Gornik HL , Bacharach JM , Biller J , Fine LJ , Gray BH , Gray WA , Gupta R , Hamburg NM , Katzen BT , Lookstein RA , Lumsden AB , Newburger JW , Rundek T , Sperati CJ , Stanley JC ; American Heart Association Council on Peripheral Vascular Disease, American Heart Association Council on Clinical Cardiology, American Heart Association Council on Cardiopulmonary Critical Care, Perioperative and Resuscitation, American Heart Association Council on Cardiovascular Disease in the Young, American Heart Association Council on Cardiovascular Radiology and Intervention, American Heart Association Council on Epidemiology and Prevention, American Heart Association Council on Functional Genomics and Translational Biology, American Heart Association Council for High Blood Pressure Research, American Heart Association Council on the Kidney in Cardiovascular Disease, American Heart Association Stroke Council. Fibromuscular dysplasia: state of the science and critical unanswered questions: a scientific statement from the American Heart Association . Circulation 2014 ; 129 : 1048 – 1078 . Google Scholar Crossref Search ADS PubMed 101 Persu A , Giavarini A , Touze E , Januszewicz A , Sapoval M , Azizi M , Barral X , Jeunemaitre X , Morganti A , Plouin PF , de Leeuw P ESH Working Group Hypertension and the Kidney . European consensus on the diagnosis and management of fibromuscular dysplasia . J Hypertens 2014 ; 32 : 1367 – 1378 . Google Scholar Crossref Search ADS PubMed 102 Savard S , Steichen O , Azarine A , Azizi M , Jeunemaitre X , Plouin PF. Association between 2 angiographic subtypes of renal artery fibromuscular dysplasia and clinical characteristics . Circulation 2012 ; 126 : 3062 – 3069 . Google Scholar Crossref Search ADS PubMed 103 Persu A , Van der Niepen P , Touze E , Gevaert S , Berra E , Mace P , Plouin PF , Jeunemaitre X. Revisiting fibromuscular dysplasia: rationale of the European fibromuscular dysplasia initiative . Hypertension 2016 ; 68 : 832 – 839 . Google Scholar Crossref Search ADS PubMed 104 Saw J , Poulter R , Fung A , Wood D , Hamburger J , Buller CE. Spontaneous coronary artery dissection in patients with fibromuscular dysplasia: a case series . Circ Cardiovasc Interv 2012 ; 5 : 134 – 137 . Google Scholar Crossref Search ADS PubMed 105 Toggweiler S , Puck M , Thalhammer C , Manka R , Wyss M , Bilecen D , Corti R , Amann-Vesti BR , Lüscher TF , Wyss CA. Associated vascular lesions in patients with spontaneous coronary artery dissection . Swiss Med Wkly 2012 ; 142 : w13538. Google Scholar PubMed 106 Liang JJ , Prasad M , Tweet MS , Hayes SN , Gulati R , Breen JF , Leng S , Vrtiska TJ. A novel application of CT angiography to detect extracoronary vascular abnormalities in patients with spontaneous coronary artery dissection . J Cardiovasc Comput Tomogr 2014 ; 8 : 189 – 197 . Google Scholar Crossref Search ADS PubMed 107 Prasad M , Tweet MS , Hayes SN , Leng S , Liang JJ , Eleid MF , Gulati R , Vrtiska TJ. Prevalence of extracoronary vascular abnormalities and fibromuscular dysplasia in patients with spontaneous coronary artery dissection . Am J Cardiol 2015 ; 115 : 1672 – 1677 . Google Scholar Crossref Search ADS PubMed 108 Saw J , Ricci D , Starovoytov A , Fox R , Buller CE. Spontaneous coronary artery dissection: prevalence of predisposing conditions including fibromuscular dysplasia in a tertiary center cohort . JACC Cardiovasc Interv 2013 ; 6 : 44 – 52 . Google Scholar Crossref Search ADS PubMed 109 Lie JT , Berg KK. Isolated fibromuscular dysplasia of the coronary arteries with spontaneous dissection and myocardial infarction . Hum Pathol 1987 ; 18 : 654 – 656 . Google Scholar Crossref Search ADS PubMed 110 Brodsky SV , Ramaswamy G , Chander P , Braun A. Ruptured cerebral aneurysm and acute coronary artery dissection in the setting of multivascular fibromuscular dysplasia: a case report . Angiology 2007 ; 58 : 764 – 767 . Google Scholar Crossref Search ADS PubMed 111 Mather PJ , Hansen CL , Goldman B , Inniss S , Pina I , Norris R , Jeevanandam V , Bove AA. Postpartum multivessel coronary dissection . J Heart Lung Transplant 1994 ; 13 : 533 – 537 . Google Scholar PubMed 112 Camuglia A , Manins V , Taylor A , Hengel C. Case report and review: epicardial coronary artery fibromuscular dysplasia . Heart Lung Circ 2009 ; 18 : 151 – 154 . Google Scholar Crossref Search ADS PubMed 113 Pate GE , Lowe R , Buller CE. Fibromuscular dysplasia of the coronary and renal arteries? Catheter Cardiovasc Interv 2005 ; 64 : 138 – 145 . Google Scholar Crossref Search ADS PubMed 114 Michelis KC , Olin JW , Kadian-Dodov D , d'Escamard V , Kovacic JC. Coronary artery manifestations of fibromuscular dysplasia . J Am Coll Cardiol 2014 ; 64 : 1033 – 1046 . Google Scholar Crossref Search ADS PubMed 115 Saw J , Bezerra H , Gornik HL , Machan L , Mancini GB. Angiographic and intracoronary manifestations of coronary fibromuscular dysplasia . Circulation 2016 ; 133 : 1548 – 1559 . Google Scholar Crossref Search ADS PubMed 116 Kadian-Dodov D , Gornik HL , Gu X , Froehlich J , Bacharach JM , Chi YW , Gray BH , Jaff MR , Kim ES , Mace P , Sharma A , Kline-Rogers E , White C , Olin JW. Dissection and aneurysm in patients with fibromuscular dysplasia: findings from the U.S. Registry for FMD . J Am Coll Cardiol 2016 ; 68 : 176 – 185 . Google Scholar Crossref Search ADS PubMed 117 Buccheri D , Piraino D , Andolina G. Behcet disease and spontaneous coronary artery dissection: the chicken or the egg? Int J Cardiol 2016 ; 215 : 504 – 505 . Google Scholar Crossref Search ADS PubMed 118 Reddy S , Vaid T , Ganiga Sanjeeva NC , Shetty RK. Spontaneous coronary artery dissection as the first presentation of systemic lupus erythematosus . BMJ Case Rep 2016 ;doi:10.1136/bcr-2016-216344. 119 Rekik S , Lanfranchi P , Jacq L , Bernasconi F. Spontaneous coronary artery dissection in a 35 year-old woman with systemic lupus erythematosus successfully treated by angioplasty . Heart Lung Circ 2013 ; 22 : 955 – 958 . Google Scholar Crossref Search ADS PubMed 120 Nisar MK , Mya T. Spontaneous coronary artery dissection in the context of positive anticardiolipin antibodies and clinically undiagnosed systemic lupus erythematosus . Lupus 2011 ; 20 : 1436 – 1438 . Google Scholar Crossref Search ADS PubMed 121 Kothari D , Ruygrok P , Gentles T , Occleshaw C. Spontaneous coronary artery dissection in an adolescent man with systemic lupus erythematosus . Intern Med J 2007 ; 37 : 342 – 343 . Google Scholar Crossref Search ADS PubMed 122 Sharma AK , Farb A , Maniar P , Ajani AE , Castagna M , Virmani R , Suddath W , Lindsay J. Spontaneous coronary artery dissection in a patient with systemic lupus erythematosis . Hawaii Med J 2003 ; 62 : 248 – 253 . Google Scholar PubMed 123 Canpolat U , Dural M , Atalar E. Acute inferior myocardial infarction in a young female patient with polyarteritis nodosa . Herz 2012 ; 37 : 461 – 463 . Google Scholar Crossref Search ADS PubMed 124 Fernández-Gutiérrez B , Zamorano J , Batlle E , Alfonso F , Conde A , Sánchez-Harguindey L , Jover JA. Coronary dissection associated with hepatitis C virus-related cryoglobulinaemia . Rheumatology (Oxford) 1999 ; 38 : 1299 – 1301 . Google Scholar Crossref Search ADS PubMed 125 Iyisoy A , Agac MT , Celik T , Jata B. Spontaneous dissection of left main coronary artery associated with hypertensive crisis: a probable fatal complication detected by intravascular ultrasound . Int J Cardiol 2010 ; 139 : e5 – e7 . Google Scholar Crossref Search ADS PubMed 126 Greenblatt JM , Kochar GS , Albornoz MA. Multivessel spontaneous coronary artery dissection in a patient with severe systolic hypertension: a possible association. A case report . Angiology 1999 ; 50 : 509 – 513 . Google Scholar Crossref Search ADS PubMed 127 Sivam S , Yozghatlian V , Dentice R , McGrady M , Moriarty C , Di Michiel J , Bye PT , Rees D. Spontaneous coronary artery dissection associated with coughing . J Cyst Fibros 2014 ; 13 : 235 – 237 . Google Scholar Crossref Search ADS PubMed 128 Afzal AM , Sarmast SA , Weber NA , Schussler JM. Spontaneous coronary artery dissection in a 22-year-old man on lisdexamfetamine . Proc (Bayl Univ Med Cent) 2015 ; 28 : 367 – 368 . Google Scholar Crossref Search ADS PubMed 129 Ijsselmuiden A , Verheye S. Cocaine-induced coronary artery dissection . JACC Cardiovasc Interv 2009 ; 2 : 1031. Google Scholar Crossref Search ADS PubMed 130 Kanwar M , Gill N. Spontaneous multivessel coronary artery dissection . J Invasive Cardiol 2010 ; 22 : E5 – E6 . Google Scholar PubMed 131 Katikaneni PK , Akkus NI , Tandon N , Modi K. Cocaine-induced postpartum coronary artery dissection: a case report and 80-year review of literature . J Invasive Cardiol 2013 ; 25 : E163 – E166 . Google Scholar PubMed 132 Wickremaarachchi C , Olinga J , Ooi SY , Cranney G. Complete angiographic resolution of cocaine induced coronary artery dissection within eight days without coronary stenting—a case report . Heart Lung Circ 2016 ; 25 : e24 – e28 . Google Scholar Crossref Search ADS PubMed 133 Reriani M , Sara JD , Flammer AJ , Gulati R , Li J , Rihal C , Lennon R , Lerman LO , Lerman A. Coronary endothelial function testing provides superior discrimination compared with standard clinical risk scoring in prediction of cardiovascular events . Coron Artery Dis 2016 ; 27 : 213 – 220 . Google Scholar Crossref Search ADS PubMed 134 Balakrishnan K , Scott P , Oliver L. A confluence of circumstances: a case of IVF, extreme exercise and spontaneous coronary artery dissection . Int J Cardiol 2016 ; 203 : 76 – 77 . Google Scholar Crossref Search ADS PubMed 135 El-Sherief K , Rashidian A , Srikanth S. Spontaneous coronary artery dissection after intense weightlifting UCSF Fresno Department of Cardiology . Catheter Cardiovasc Interv 2011 ; 78 : 223 – 227 . Google Scholar Crossref Search ADS PubMed 136 Ellis CJ , Haywood GA , Monro JL. Spontaneous coronary artery dissection in a young woman resulting from an intense gymnasium “work-out”. Int J Cardiol 1994 ; 47 : 193 – 194 . Google Scholar Crossref Search ADS PubMed 137 Choi JW , Davidson CJ. Spontaneous multivessel coronary artery dissection in a long-distance runner successfully treated with oral antiplatelet therapy . J Invasive Cardiol 2002 ; 14 : 675 – 678 . Google Scholar PubMed 138 Aghasadeghi K , Aslani A. Spontaneous coronary artery dissection in a professional body builder . Int J Cardiol 2008 ; 130 : e119 – e120 . Google Scholar Crossref Search ADS PubMed 139 Adlam D , Cuculi F , Lim C , Banning A. Management of spontaneous coronary artery dissection in the primary percutaneous coronary intervention era . J Invasive Cardiol 2010 ; 22 : 549 – 553 . Google Scholar PubMed 140 Basile C , Lucarelli K , Langialonga T. Spontaneous coronary artery dissection: one more extrarenal manifestation of autosomal dominant polycystic kidney disease? J Nephrol 2009 ; 22 : 414 – 416 . Google Scholar PubMed 141 Grover P , Fitzgibbons TP. Spontaneous coronary artery dissection in a patient with autosomal dominant polycystic kidney disease: a case report . J Med Case Rep 2016 ; 10 : 62. Google Scholar Crossref Search ADS PubMed 142 Klingenberg-Salachova F , Limburg S , Boereboom F. Spontaneous coronary artery dissection in polycystic kidney disease . Clin Kidney J 2012 ; 5 : 44 – 46 . Google Scholar Crossref Search ADS PubMed 143 Nakamura M , Yajima J , Oikawa Y , Ogasawara K , Uejima T , Abe K , Aizawa T. Vascular Ehlers-Danlos syndrome–all three coronary artery spontaneous dissections . J Cardiol 2009 ; 53 : 458 – 462 . Google Scholar Crossref Search ADS PubMed 144 Goel K , Tweet M , Olson TM , Maleszewski JJ , Gulati R , Hayes SN. Familial spontaneous coronary artery dissection: evidence for genetic susceptibility . JAMA Intern Med 2015 ; 175 : 821 – 826 . Google Scholar Crossref Search ADS PubMed 145 Al-Hussaini A , Adlam D. Spontaneous coronary artery dissection . Heart 2017 ; 146 Luong C , Starovoytov A , Heydari M , Sedlak T , Aymong E , Saw J. Clinical presentation of patients with spontaneous coronary artery dissection . Catheter Cardiovasc Interv 2017 ; 147 Tweet MS , Gulati R , Williamson EE , Vrtiska TJ , Hayes SN. Multimodality imaging for spontaneous coronary artery dissection in women . JACC Cardiovasc Imaging 2016 ; 9 : 436 – 450 . Google Scholar Crossref Search ADS PubMed 148 Rogers JH , Lasala JM. Coronary artery dissection and perforation complicating percutaneous coronary intervention . J Invasive Cardiol 2004 ; 16 : 493 – 499 . Google Scholar PubMed 149 Saw J. Coronary angiogram classification of spontaneous coronary artery dissection . Catheter Cardiovasc Interv 2014 ; 84 : 1115 – 1122 . Google Scholar Crossref Search ADS PubMed 150 Saw J , Mancini GB , Humphries K , Fung A , Boone R , Starovoytov A , Aymong E. Angiographic appearance of spontaneous coronary artery dissection with intramural hematoma proven on intracoronary imaging . Catheter Cardiovasc Interv 2016 ; 87 : E54 – E61 . Google Scholar Crossref Search ADS PubMed 151 De-Giorgio F , Grassi VM , Abbate A , d'Aloja E , Arena V. Causation or coincidence? A case of sudden death due to spontaneous coronary artery dissection in presence of myocardial bridging . Int J Cardiol 2012 ; 159 : e32 – e34 . Google Scholar Crossref Search ADS PubMed 152 Burke AP , Kutys R , Fowler D , Virmani R. Multiple spontaneous coronary artery dissections in association with anomalous origin of right coronary and intramural coronary artery dysplasia . Cardiovasc Pathol 2004 ; 13 : 173 – 175 . Google Scholar Crossref Search ADS PubMed 153 Degrauwe S , Zuffi A , Muller O , Schiele F , Eeckhout E , Iglesias JF. Very late multiple recurrent spontaneous coronary artery dissection in a young woman with recidivating acute myocardial infarction . Int J Cardiol 2016 ; 223 : 168 – 170 . Google Scholar Crossref Search ADS PubMed 154 Motreff P , Souteyrand G , Dauphin C , Eschalier R , Cassagnes J , Lusson JR. Management of spontaneous coronary artery dissection: review of the literature and discussion based on a series of 12 young women with acute coronary syndrome . Cardiology 2010 ; 115 : 10 – 18 . Google Scholar Crossref Search ADS PubMed 155 Sanchez-Recalde A , Guzman G , Armada E , Moreno R. Multiple spontaneous coronary artery dissection associated with a left main coronary artery lesion treated by stenting. Late multiple stent fractures detected by multislice CT . Rev Esp Cardiol 2009 ; 62 : 225 – 226 . Google Scholar Crossref Search ADS PubMed 156 Sharma AM , Herrera B , Aronow HD. Simultaneous spontaneous coronary and vertebral artery dissection in a postpartum woman . J Invasive Cardiol 2010 ; 22 : E229 – E232 . Google Scholar PubMed 157 Prakash R , Starovoytov A , Heydari M , Mancini GB , Saw J. Catheter-induced iatrogenic coronary artery dissection in patients with spontaneous coronary artery dissection . JACC Cardiovasc Interv 2016 ; 9 : 1851 – 1853 . Google Scholar Crossref Search ADS PubMed 158 Arnold JR , West NE , van Gaal WJ , Karamitsos TD , Banning AP. The role of intravascular ultrasound in the management of spontaneous coronary artery dissection . Cardiovasc Ultrasound 2008 ; 6 : 24. Google Scholar Crossref Search ADS PubMed 159 Poon K , Bell B , Raffel OC , Walters DL , Jang IK. Spontaneous coronary artery dissection: utility of intravascular ultrasound and optical coherence tomography during percutaneous coronary intervention . Circ Cardiovasc Interv 2011 ; 4 : e5 – e7 . Google Scholar Crossref Search ADS PubMed 160 Lempereur M , Fung A , Saw J. Stent mal-apposition with resorption of intramural hematoma with spontaneous coronary artery dissection . Cardiovasc Diagn Ther 2015 ; 5 : 323 – 329 . Google Scholar PubMed 161 Haraki T , Uemura R , Masuda S , Lee T. Progressed multivessel spontaneous coronary artery dissection that naturally healed in a male patient with non-st segment elevation myocardial infarction . Case Rep Cardiol 2016 ; 2016 : 4109496. Google Scholar PubMed 162 Auer J , Punzengruber C , Berent R , Weber T , Lamm G , Hartl P , Eber B. Spontaneous coronary artery dissection involving the left main stem: assessment by intravascular ultrasound . Heart 2004 ; 90 : e39. Google Scholar Crossref Search ADS PubMed 163 Danzi GB , Sesana M , Capuano C , Baglini R. Intravascular ultrasound assessment of a spontaneous coronary artery dissection causing acute myocardial infarction . Ital Heart J 2004 ; 5 : 796 – 797 . Google Scholar PubMed 164 Kearney P , Erbel R , Ge J , Zamorano J , Koch L , Gorge G , Meyer J. Assessment of spontaneous coronary artery dissection by intravascular ultrasound in a patient with unstable angina . Cathet Cardiovasc Diagn 1994 ; 32 : 58 – 61 . Google Scholar Crossref Search ADS PubMed 165 Maehara A , Mintz GS , Castagna MT , Pichard AD , Satler LF , Waksman R , Suddath WO , Kent KM , Weissman NJ. Intravascular ultrasound assessment of spontaneous coronary artery dissection . Am J Cardiol 2002 ; 89 : 466 – 468 . Google Scholar Crossref Search ADS PubMed 166 Alfonso F , Canales E , Aleong G. Spontaneous coronary artery dissection: diagnosis by optical coherence tomography . Eur Heart J 2009 ; 30 : 385. Google Scholar Crossref Search ADS PubMed 167 Satogami K , Ino Y , Kubo T , Shiono Y , Nishiguchi T , Matsuo Y , Orii M , Yamano T , Yamaguchi T , Hirata K , Tanaka A , Akasaka T. Successful stenting with optical frequency domain imaging guidance for spontaneous coronary artery dissection . JACC Cardiovasc Interv 2015 ; 8 : e83 – e85 . Google Scholar Crossref Search ADS PubMed 168 Bento AP , Fernandes RG , Neves DC , Patricio LM , de Aguiar JE. Spontaneous coronary dissection: “Live Flash” optical coherence tomography guided angioplasty . Case Rep Cardiol 2016 ; 2016 : 5643819. Google Scholar PubMed 169 Mori H , Kutys R , Romero M , Virmani R , Finn AV. Stenting of spontaneous coronary artery dissection from a pathological point of view . Circ Cardiovasc Interv 2016 ; 9 : e004549. Google Scholar Crossref Search ADS PubMed 170 Combaret N , Souteyrand G , Amonchot A , Coupez E , Motreff P. Contribution of guidance by optical coherence tomography (OCT) in rescue management of spontaneous coronary artery dissection . Eur Heart J Cardiovasc Imaging 2013 ; 14 : 714. Google Scholar Crossref Search ADS PubMed 171 Cade J , Mintz GS , Silva RMF , Caixeta A. Spontaneous coronary artery dissection and healing documented by optical coherence tomography . Einstein (Sao Paulo) 2016 ; 14 : 435 – 436 . Google Scholar Crossref Search ADS PubMed 172 Kwon TG , Gulati R , Matsuzawa Y , Aoki T , Guddeti RR , Herrmann J , Lennon RJ , Ritman EL , Lerman LO , Lerman A. Proliferation of coronary adventitial vasa vasorum in patients with spontaneous coronary artery dissection . JACC Cardiovasc Imaging 2016 ; 9 : 891 – 892 . Google Scholar Crossref Search ADS PubMed 173 Eleid MF , Tweet MS , Young PM , Williamson E , Hayes SN , Gulati R. Spontaneous coronary artery dissection: challenges of coronary computed tomography angiography . Eur Heart J Acute Cardiovasc Care 2017 ;2048872616687098. 174 Torres-Ayala SC , Maldonado J , Bolton JS , Bhalla S. Coronary computed tomography angiography of spontaneous coronary artery dissection: a case report and review of the literature . Am J Case Rep 2015 ; 16 : 130 – 135 . Google Scholar Crossref Search ADS PubMed 175 Alzand BS , Vanneste L , Fonck D , Van Mieghem C. Spontaneous coronary artery dissection undissolved using cardiac computed tomography . Int J Cardiol 2016 ; 222 : 1040 – 1041 . Google Scholar Crossref Search ADS PubMed 176 Conrotto F , D'Ascenzo F , Cerrato E , Fernandez-Ortiz A , Gonzalo N , Macaya F , Tamburino C , Barbanti M , van Lavieren M , Piek JJ , Applegate RJ , Latib A , Spinnler MT , Marzullo R , Iannaccone M , Pavani M , Crimi G , Fattori R , Chinaglia A , Presbitero P , Varbella F , Gaita F , Escaned J. Safety and efficacy of drug eluting stents in patients with spontaneous coronary artery dissection . Int J Cardiol 2017 ; 238 : 105 – 109 . Google Scholar Crossref Search ADS PubMed 177 Martins JL , Afreixo V , Santos L , Costa M , Santos J , Goncalves L. Medical treatment or revascularisation as the best approach for spontaneous coronary artery dissection: a systematic review and meta-analysis . Eur Heart J Acute Cardiovasc Care 2017 ;2048872617706502. 178 Arrivi A , Milici C , Bock C , Placanica A , Boschetti E , Dominici M. Idiopathic, serial coronary vessels dissection in a young woman with psychological stress: a case report and review of the literature . Case Rep Vasc Med 2012 ; 2012 : 1. Google Scholar Crossref Search ADS 179 Dashwood AM , Saw J , Dhillon P , Murdoch D. Use of a three-stent technique for a case of spontaneous coronary artery dissection . Can J Cardiol 2017 ; 33 : 830.e13 – 830.e15 . Google Scholar Crossref Search ADS 180 Walsh SJ , Jokhi PP , Saw J. Successful percutaneous management of coronary dissection and extensive intramural haematoma associated with ST elevation MI . Acute Card Care 2008 ; 10 : 231 – 233 . Google Scholar Crossref Search ADS PubMed 181 Alfonso F , Bastante T , Garcia-Guimaraes M , Pozo E , Cuesta J , Rivero F , Benedicto A , Antuna P , Alvarado T , Gulati R , Saw J. Spontaneous coronary artery dissection: new insights into diagnosis and treatment . Coron Artery Dis 2016 ; 27 : 696 – 706 . Google Scholar Crossref Search ADS PubMed 182 Ito T , Shintani Y , Ichihashi T , Fujita H , Ohte N. Non-atherosclerotic spontaneous coronary artery dissection revascularized by intravascular ultrasonography-guided fenestration with cutting balloon angioplasty . Cardiovasc Interv Ther 2017 ; 32 : 241 – 243 . Google Scholar Crossref Search ADS PubMed 183 Alkhouli M , Cole M , Ling FS. Coronary artery fenestration prior to stenting in spontaneous coronary artery dissection . Catheter Cardiovasc Interv 2016 ; 88 : E23 – E27 . Google Scholar Crossref Search ADS PubMed 184 Motreff P , Barber-Chamoux N , Combaret N , Souteyrand G. Coronary artery fenestration guided by optical coherence tomograhy before stenting: new interventional option in rescue management of compressive spontaneous intramural hematoma . Circ Cardiovasc Interv 2015 ; 8 : e002266. Google Scholar Crossref Search ADS PubMed 185 Yumoto K , Sasaki H , Aoki H , Kato K. Successful treatment of spontaneous coronary artery dissection with cutting balloon angioplasty as evaluated with optical coherence tomography . JACC Cardiovasc Interv 2014 ; 7 : 817 – 819 . Google Scholar Crossref Search ADS PubMed 186 Watt J , Egred M , Khurana A , Bagnall AJ , Zaman AG. 1-year follow-up optical frequency domain imaging of multiple bioresorbable vascular scaffolds for the treatment of spontaneous coronary artery dissection . JACC Cardiovasc Interv 2016 ; 9 : 389 – 391 . Google Scholar Crossref Search ADS PubMed 187 Sengottuvelu G , Rajendran R. Full polymer jacketing for long-segment spontaneous coronary artery dissection using bioresorbable vascular scaffolds . JACC Cardiovasc Interv 2014 ; 7 : 820 – 821 . Google Scholar Crossref Search ADS PubMed 188 Panoulas VF , Ielasi A. Bioresorbable scaffolds and drug-eluting balloons for the management of spontaneous coronary artery dissections . J Thorac Dis 2016 ; 8 : E1328 – E1330 . Google Scholar Crossref Search ADS PubMed 189 Serruys PW , Chevalier B , Sotomi Y , Cequier A , Carrié D , Piek JJ , Van Boven AJ , Dominici M , Dudek D , McClean D , Helqvist S , Haude M , Reith S , de Sousa Almeida M , Campo G , Iñiguez A , Sabaté M , Windecker S , Onuma Y. Comparison of an everolimus-eluting bioresorbable scaffold with an everolimus-eluting metallic stent for the treatment of coronary artery stenosis (ABSORB II): a 3 year, randomised, controlled, single-blind, multicentre clinical trial . Lancet 2016 ; 388 : 2479 – 2491 . Google Scholar Crossref Search ADS PubMed 190 Wehman B , Lehr EJ , Mukherjee R , Grigore A , Griffith B , Bonatti J. Robotic totally endoscopic coronary artery bypass grafting for spontaneous coronary artery dissection . Int J Med Robot 2012 ; 8 : 166 – 168 . Google Scholar Crossref Search ADS PubMed 191 Abu-Laban RB , Migneault D , Grant MR , Dhingra V , Fung A , Cook RC , Sweet D. Extracorporeal membrane oxygenation after protracted ventricular fibrillation cardiac arrest: case report and discussion . CJEM 2015 ; 17 : 210 – 216 . Google Scholar Crossref Search ADS PubMed 192 Aliyary S , Mariani MA , Verhorst PM , Hartmann M , Stoel MG , von Birgelen C. Staged therapeutic approach in spontaneous coronary dissection . Ann Thorac Surg 2007 ; 83 : 1879 – 1881 . Google Scholar Crossref Search ADS PubMed 193 Farhat F , Sassard T , Maghiar A , Jegaden O. Primary spontaneous coronary artery dissection complicated by iatrogenous aortic dissection: from David procedure to full arterial coronary revascularization . Interact Cardiovasc Thorac Surg 2006 ; 5 : 149 – 152 . Google Scholar Crossref Search ADS PubMed 194 Jacob JC , Kiernan FJ , Patel N , Rock J , Hammond J Jr. , Wencker D , Lasala AF. SCAD: a rare case of cardiac arrest in a young female . Conn Med 2011 ; 75 : 147 – 152 . Google Scholar PubMed 195 Jorge-Pérez P , García-González MJ , Ávalos-Pinto RM , G-Cosio-Carmena MD , Renes-Carreño E , Delgado JF , Yanes-Bowden G , Ferrer-Hita JJ. Spontaneous coronary dissection and cardiogenic shock requiring mechanical circulatory support in a non-transplant center . Int J Cardiol 2016 ; 221 : 629 – 630 . Google Scholar Crossref Search ADS PubMed 196 Julia I , Tauron M , Munoz-Guijosa C. Postpartum acute coronary syndrome due to intramural hematoma and coronary artery dissection . Thorac Cardiovasc Surg 2012 ; 61 : 085 – 087 . Google Scholar Crossref Search ADS 197 Paez M , Buisan F , Herrero E. Spontaneous dissection of the left coronary artery trunk during the postpartum period treated with revascularization surgery, ventricular assistance and a successful heart transplant . Acta Anaesthesiol Scand 2007 ; 51 : 960 – 961 . Google Scholar Crossref Search ADS PubMed 198 Rahman S , Abdul-Waheed M , Helmy T , Huffman LC , Koshal V , Guitron J , Merrill WH , Lewis DF , Dunlap S , Shizukuda Y , Weintraub NL , Meyer C , Cilingiroglu M. Spontaneous left main coronary artery dissection complicated by pseudoaneurysm formation in pregnancy: role of CT coronary angiography . J Cardiothorac Surg 2009 ; 4 : 15. Google Scholar Crossref Search ADS PubMed 199 Weinberg L , Ong M , Tan CO , McDonnell NJ , Lo C , Chiam E. Spontaneous coronary artery dissection in pregnancy requiring emergency caesarean delivery followed by coronary artery bypass grafting . Anaesth Intensive Care 2013 ; 41 : 251 – 255 . Google Scholar PubMed 200 Bashir M , Mustafa H , Singh H , Bonser R. Cardiac transplantation for spontaneous coronary artery dissection . Interact Cardiovasc Thorac Surg 2013 ; 16 : 91 – 92 . Google Scholar Crossref Search ADS PubMed 201 Evans R. Post-partum spontaneous coronary artery dissection and the use of veno-arterial extra-corporeal membrane oxygenation . Nurs Crit Care 2014 ; 19 : 304 – 309 . Google Scholar Crossref Search ADS PubMed 202 Knapp KE , Weis RA , Cubillo EI , Chapital AB , Ramakrishna H. Spontaneous, postpartum coronary artery dissection and cardiogenic shock with extracorporeal membrane oxygenation assisted recovery in a 30-year-old patient . Case Rep Cardiol 2016 ; 2016 : 1. Google Scholar Crossref Search ADS 203 Martins RP , Leurent G , Corbineau H , Fouquet O , Seconda S , Baruteau AE , Moreau O , Le Breton H , Bedossa M. Coronary angiography of pregnancy-associated coronary artery dissection: a high-risk procedure . Cardiovasc Revasc Med 2010 ; 11 : 182 – 185 . Google Scholar Crossref Search ADS PubMed 204 Patane F , Boffini M , Sansone F , Campanella A , Rinaldi M. ECMO as a bridge to transplantation in biventricular dysfunction due to primary spontaneous coronary artery dissection . Transpl Int 2009 ; 22 : 500 – 502 . Google Scholar Crossref Search ADS PubMed 205 Ponikowski P , Voors AA , Anker SD , Bueno H , Cleland JG , Coats AJ , Falk V , Gonzalez-Juanatey JR , Harjola VP , Jankowska EA , Jessup M , Linde C , Nihoyannopoulos P , Parissis JT , Pieske B , Riley JP , Rosano GM , Ruilope LM , Ruschitzka F , Rutten FH , van der Meer P. Authors/Task Force M . 2016 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure: the Task Force for the diagnosis and treatment of acute and chronic heart failure of the European Society of Cardiology (ESC) Developed with the special contribution of the Heart Failure Association (HFA) of the ESC . Eur Heart J 2016 ; 37 : 2129 – 2200 . Google Scholar Crossref Search ADS PubMed 206 Roffi M , Patrono C , Collet JP , Mueller C , Valgimigli M , Andreotti F , Bax JJ , Borger MA , Brotons C , Chew DP , Gencer B , Hasenfuss G , Kjeldsen K , Lancellotti P , Landmesser U , Mehilli J , Mukherjee D , Storey RF , Windecker S , Baumgartner H , Gaemperli O , Achenbach S , Agewall S , Badimon L , Baigent C , Bueno H , Bugiardini R , Carerj S , Casselman F , Cuisset T , Erol C , Fitzsimons D , Halle M , Hamm C , Hildick-Smith D , Huber K , Iliodromitis E , James S , Lewis BS , Lip GY , Piepoli MF , Richter D , Rosemann T , Sechtem U , Steg PG , Vrints C , Luis Zamorano J. Management of Acute Coronary Syndromes in Patients Presenting without Persistent STSEotESoC . 2015 ESC Guidelines for the management of acute coronary syndromes in patients presenting without persistent ST-segment elevation: task Force for the Management of Acute Coronary Syndromes in Patients Presenting without Persistent ST-Segment Elevation of the European Society of Cardiology (ESC) . Eur Heart J 2016 ; 37 : 267 – 315 . Google Scholar Crossref Search ADS PubMed 207 Ibanez B , James S , Agewall S , Antunes MJ , Bucciarelli-Ducci C , Bueno H , Caforio ALP , Crea F , Goudevenos JA , Halvorsen S , Hindricks G , Kastrati A , Lenzen MJ , Prescott E , Roffi M , Valgimigli M , Varenhorst C , Vranckx P , Widimsky P. 2017 ESC Guidelines for the management of acute myocardial infarction in patients presenting with ST-segment elevation . Rev Esp Cardiol (Engl Ed) 2017 ; 70 : 1082 . Google Scholar Crossref Search ADS PubMed 208 Priori SG , Blomström-Lundqvist C , Mazzanti A , Blom N , Borggrefe M , Camm J , Elliott PM , Fitzsimons D , Hatala R , Hindricks G , Kirchhof P , Kjeldsen K , Kuck K-H , Hernandez-Madrid A , Nikolaou N , Norekvål TM , Spaulding C , Van Veldhuisen DJ. 2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: the Task Force for the Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death of the European Society of Cardiology (ESC). Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC) . Eur Heart J 2015 ; 36 : 2793 – 2867 . Google Scholar Crossref Search ADS PubMed 209 Jovic Z , Obradovic S , Djenic N , Mladenovic Z , Djuric P , Spasic M , Tavicovski D. Does thrombolytic therapy harm or help in ST elevation myocardial infarction (STEMI) caused by the spontaneous coronary dissection? Vojnosanit Pregl 2015 ; 72 : 536 – 540 . Google Scholar Crossref Search ADS PubMed 210 Andreou AY , Georgiou PA , Georgiou GM. Spontaneous coronary artery dissection: report of two unsuspected cases initially treated with thrombolysis . Exp Clin Cardiol 2009 ; 14 : e89 – e92 . Google Scholar PubMed 211 Shamloo BK , Chintala RS , Nasur A , Ghazvini M , Shariat P , Diggs JA , Singh SN. Spontaneous coronary artery dissection: aggressive vs. conservative therapy . J Invasive Cardiol 2010 ; 22 : 222 – 228 . Google Scholar PubMed 212 Zupan I , Noc M , Trinkaus D , Popovic M. Double vessel extension of spontaneous left main coronary artery dissection in young women treated with thrombolytics . Catheter Cardiovasc Interv 2001 ; 52 : 226 – 230 . Google Scholar Crossref Search ADS PubMed 213 Goh AC , Lundstrom RJ. Spontaneous coronary artery dissection with cardiac tamponade . Tex Heart Inst J 2015 ; 42 : 479 – 482 . Google Scholar Crossref Search ADS PubMed 214 Maas AH , Euler M , Bongers MY , Rolden HJ , Grutters JP , Ulrich L , Schenck-Gustafsson K. Practice points in gynecardiology: abnormal uterine bleeding in premenopausal women taking oral anticoagulant or antiplatelet therapy . Maturitas 2015 ; 82 : 355 – 359 . Google Scholar Crossref Search ADS PubMed 215 Tweet MS , Olin JW. Insights into spontaneous coronary artery dissection: can recurrence be prevented? J Am Coll Cardiol 2017 ; 70 : 1159 – 1161 . Google Scholar Crossref Search ADS PubMed 216 Colletti PM , Lee KH , Elkayam U. Cardiovascular imaging of the pregnant patient . AJR Am J Roentgenol 2013 ; 200 : 515 – 521 . Google Scholar Crossref Search ADS PubMed 217 Tweet MS , Hayes SN , Gulati R , Rose CH , Best PJ. Pregnancy after spontaneous coronary artery dissection: a case series . Ann Intern Med 2015 ; 162 : 598 – 600 . Google Scholar Crossref Search ADS PubMed 218 Guo LQ , Wasfy MM , Hedgire S , Kalra M , Wood M , Prabhakar AM , Ghoshhajra BB. Multimodality imaging of spontaneous coronary artery dissection: case studies of the Massachusetts General Hospital . Coron Artery Dis 2016 ; 27 : 70 – 71 . Google Scholar Crossref Search ADS PubMed 219 Bhatt DD , Kachru R , Gupta S , Kaul U. Recurrent chest pain after treatment of spontaneous coronary artery dissection: an enigma . Indian Heart J 2015 ; 67 : S18 – S20 . Google Scholar Crossref Search ADS PubMed 220 Chou AY , Prakash R , Rajala J , Birnie T , Isserow S , Taylor CM , Ignaszewski A , Chan S , Starovoytov A , Saw J. The first dedicated cardiac rehabilitation program for patients with spontaneous coronary artery dissection: description and initial results . Can J Cardiol 2016 ; 32 : 554 – 560 . Google Scholar Crossref Search ADS PubMed 221 Krittanawong C , Tweet MS , Hayes SE , Bowman MJ , Gulati R , Squires RW , Hayes SN. Usefulness of cardiac rehabilitation after spontaneous coronary artery dissection . Am J Cardiol 2016 ; 117 : 1604 – 1609 . Google Scholar Crossref Search ADS PubMed 222 Liang JJ , Tweet MS , Hayes SE , Gulati R , Hayes SN. Prevalence and predictors of depression and anxiety among survivors of myocardial infarction due to spontaneous coronary artery dissection . J Cardiopulm Rehabil Prev 2014 ; 34 : 138 – 142 . Google Scholar Crossref Search ADS PubMed Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2018. For permissions, please email: journals.permissions@oup.com. This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/open_access/funder_policies/chorus/standard_publication_model) http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png European Heart Journal Oxford University Press

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Abstract

ESC-ACCA Position Paper on spontaneous coronary artery dissection  Contents Introduction 3354 Definition 3354 Pathology 3354 Epidemiology 3356 Incidence 3356 Demographics 3356 Pathophysiology: risk factors and associations 3356 Female sex and pregnancy 3356 Fibromuscular dysplasia 3357 Inflammatory conditions 3358 Atherosclerotic risk factors 3358 Mechanical stressors and exercise 3359 Emotional stressors 3359 Inherited connective tissue disorders 3359 Genetics 3359 Clinical presentation 3359 Diagnosis 3359 Coronary angiographic classification 3360 Additional possible angiographic findings 3360 Multi-vessel spontaneous coronary artery dissection 3361 Secondary iatrogenic dissection in spontaneous coronary artery dissection 3361 Intracoronary imaging 3361  Intravascular ultrasound 3361  Optical coherence tomography 3362 Computed tomography coronary angiography 3363 Acute management 3364 Conservative management 3364 Percutaneous coronary intervention 3364 Coronary artery bypass grafting 3365 Adjunctive supportive devices and transplant 3365 Medical management 3365  Thrombolysis 3365  Antiplatelet therapies 3365  Anticoagulant therapies 3365  Angiotensin converting enzyme inhibitors, angiotensin receptor antagonists, mineralocorticoid receptor antagonists, beta-blockers and vasodilator therapies 3365  Statins 3366  Contraception and hormone replacement therapy 3366 Pregnancy 3366 Pregnancy-associated spontaneous coronary artery dissection: special considerations 3366 Pregnancy after spontaneous coronary artery dissection 3366 Outcomes and follow-up 3366 Follow-up imaging 3366  Echocardiography/cardiac magnetic resonance imaging 3366  Computed tomography-peripheral angiography or magnetic resonance-angiography 3366  Coronary angiography or CT-coronary angiography 3367 Prognosis: mortality, recurrence risk and major adverse cardiac events 3367 Post spontaneous coronary artery dissection chest pain and its management 3367 Cardiac rehabilitation and exercise 3368 Post-traumatic stress disorder and the emotional and psychological consequences of spontaneous coronary artery dissection 3368 Management considerations 3368 Research priorities 3368 Introduction Spontaneous coronary artery dissection (SCAD) has long been recognized as a cause of acute coronary syndromes (ACS). Initially considered very rare and associated primarily with pregnancy and the peripartum period, the use of higher sensitivity Troponin assays and early angiography in ACS, coupled with greater awareness of the condition, has led to increased diagnosis, and it is now understood that SCAD represents a significant cause of ACS in predominantly young to middle-aged women, with most cases occurring outside the context of recent pregnancy.1,2 Although there are no randomized controlled trials in SCAD, knowledge has further advanced in the last 5-years as a result of an international research effort primarily focused on building and studying national SCAD registries.3–19 These studies have demonstrated, not only that SCAD is a distinct pathophysiological entity, but that there are key differences in management and outcomes compared to ACS of atherosclerotic aetiology. This position paper aims to set-out current knowledge on SCAD for the benefit of practicing clinicians caring for patients with this condition. It presents the consensus on contemporary management and areas of controversy and uncertainty, which remain a focus of ongoing research. The information is provided to support clinical care providers but is not intended to replace individualized decision-making by clinicians and other health care professionals. Definition For the purposes of this article, SCAD refers to the acute development of a false lumen within the coronary artery wall which may compromise coronary flow by external compression of the true lumen. Dissections arising from coronary instrumentation (iatrogenic), trauma and as a consequence of a penetrating ulcer or plaque rupture secondary to atherosclerotic disease or primary aortic dissection are not considered here. Pathology Spontaneous coronary artery dissection is a recognized, relatively rare cause of sudden cardiac death, presumably as a result of ventricular arrhythmia triggered by myocardial ischaemia or infarction.20 Accurate diagnosis at autopsy can be challenging and the condition is likely under-represented in post mortem series.21,22 A high index of suspicion for SCAD is recommended in all potential cases with careful assessment of coronary histopathology, particularly of the mid-distal vessels which are predominantly affected in SCAD and examination of the peripheral arterial system for associated arteriopathies [such as fibromuscular dysplasia (FMD)]. Spontaneous coronary artery dissection results from the development of a false lumen, generally in the outer third of the tunica media (Figure 1A).21–27 The primary cause of false lumen formation is unclear with two potential mechanisms proposed: the ‘inside-out’ model (Figure 1B), where the causal event is the development of an endothelial and intimal discontinuity or ‘tear’, allowing blood to cross the internal elastic lamina and accumulate in the media; and the ‘outside-in’ mechanism (Figure 1C) where the causal event is the primary disruption of a vasa vasorum micro-vessel leading to haemorrhage directly into the tunica media.1,28,29 In either case blood propagates axially as the false lumen extends leading to compression of the true lumen. It remains unclear if there is a single dominant mechanism in SCAD or if both causal events are possible. However, a recent intracoronary imaging study with high resolution optical coherence tomography (OCT) has shown case examples where there is no demonstrable communication between false and true lumens,13,30,31 suggesting the ‘outside-in’ mechanism is likely in at least some cases. Figure 1 View largeDownload slide Schematic illustration of spontaneous coronary artery dissection. Accumulation and axial propagation of blood forms a false lumen in the outer third of the tunica media leading to external compression of the true lumen (A). Blood may enter through an endothelial-intimal disruption or ‘tear’ (B) or as a result of bleeding from a microvessel within the vessel wall (C) leading to an expanding and compressing false lumen (dotted arrows). Figure 1 View largeDownload slide Schematic illustration of spontaneous coronary artery dissection. Accumulation and axial propagation of blood forms a false lumen in the outer third of the tunica media leading to external compression of the true lumen (A). Blood may enter through an endothelial-intimal disruption or ‘tear’ (B) or as a result of bleeding from a microvessel within the vessel wall (C) leading to an expanding and compressing false lumen (dotted arrows). Histologically, fibrin-rich haematoma is present in the false lumen (Figure 2) with a neutrophil infiltrate extending into the media. There are frequent reports of a peri-adventitial inflammatory infiltrate with a predominance of eosinophils.21,23–25,27,32–36 The clot reorganizes and attaches to the media with granulation tissue formation and recanalization. Although this may not be specific to SCAD, it can be useful in distinguishing SCAD from post mortem artefact where fissure formation may occur but only red blood cells are present.21 Some early cases report features of cystic medial necrosis37–39 but more recent reports do not concur.32,34,40 Significant co-existent atherosclerotic coronary artery disease in SCAD is uncommon.21 Key Messages Spontaneous coronary artery dissection should be actively considered in the post-mortem differential diagnosis of unexplained sudden cardiac death with careful assessment of the entire coronary tree. Figure 2 View largeDownload slide Gross pathology (A) and histopathology (B) showing spontaneous coronary artery dissection with external compression of the true lumen by blood within a false lumen in the outer third of the tunica media. Figure 2 View largeDownload slide Gross pathology (A) and histopathology (B) showing spontaneous coronary artery dissection with external compression of the true lumen by blood within a false lumen in the outer third of the tunica media. Epidemiology Incidence The true incidence of SCAD is unknown as this condition remains under-diagnosed.2 Spontaneous coronary artery dissection was historically considered very rare but contemporary angiographic series report SCAD diagnosis rates of 0.07–0.2% of all angiograms and 2–4% of angiograms performed for ACS14,41,42 (although the study with the highest ACS prevalence did not exclude all atherosclerotic cases42). Furthermore, in younger women SCAD is reported to account for a much higher proportion of ACS presentations. In a Canadian series of women less than 50 years with myocardial infarction, SCAD accounted for 24% of cases.43 Likewise a Japanese registry reported SCAD in 35% of females patients under 50 years presenting with acute myocardial infarction (AMI),8 a French series reported SCAD in 36% of women under 60 years with ACS and one or fewer conventional cardiovascular risk factors13 and a smaller Australian series describes a SCAD prevalence of 23% in women under 60 years presenting with ACS.16 Pregnant and peripartum cases (P-SCAD) account for a minority of these cases (around 10% in most contemporary series)3–11,13–16,18,44,45 and SCAD should no longer be considered primarily a peripartum condition. However, 21–27% of myocardial infarctions in pregnancy and 50% of post-partum coronary events are reportedly due to SCAD.18,46 Demographics Previously considered primarily a disease of young adults, SCAD has now been described in patients aged 18–84 years4,44 with the mean age in large contemporary series ranging from 44 to 53 years.3–5,7–16 No ethnic variations have been reported but there is a strong female predominance (female sex and pregnancy section). The demographics of SCAD patients from 14 contemporary series with at least 20 patients are summarized in Table 1.3–16 Table 1 Demographics and risk factors of patients with spontaneous coronary artery dissection (SCAD) in contemporary case series (studies with n > 20) Max N Age (years) Gender (female, %) HTN (%) Chol (%) Smoking (%) DM (%) FH (%) P-SCAD (%) Mayo Clinic3 189 44 ± 9 92 31 22 15 2 NA 15 Saw4 168 52 ± 9 92 39 24 13 5 29 2 Lettieri5 134 52 ± 11 81 51 33 34 2 25 NA Faden6 79 33 ± 5 100 17 18 17 11 NA 100 Rogowski7 64 53 ± 11 94 45 52 28 0 19 5 Nakashima8 63 46 ± 10 94 33 23 32 0 8 8 Motreff13 55 50 100 27 11 22 4 22 4 McGrath-Cadell9 40 45 ± 10 95 18 10 8 5 28 8 Roura10 34 47 ± 12 94 NA NA NA NA 15 Alfonso11 27 52 ± 10 85 37 33 52 4 NA 4 Ito12 23 45 ± 11 100 57 22 30 4 NA 30 Vanzetto14 23 46 ± 9 74 26 39 43 13 13 0 Mortensen15 22 49 ± 9 81 38 NA 57 0 40 10 Rashid16 21 53 ± 9 95 48 48 47 5 24 0 Max N Age (years) Gender (female, %) HTN (%) Chol (%) Smoking (%) DM (%) FH (%) P-SCAD (%) Mayo Clinic3 189 44 ± 9 92 31 22 15 2 NA 15 Saw4 168 52 ± 9 92 39 24 13 5 29 2 Lettieri5 134 52 ± 11 81 51 33 34 2 25 NA Faden6 79 33 ± 5 100 17 18 17 11 NA 100 Rogowski7 64 53 ± 11 94 45 52 28 0 19 5 Nakashima8 63 46 ± 10 94 33 23 32 0 8 8 Motreff13 55 50 100 27 11 22 4 22 4 McGrath-Cadell9 40 45 ± 10 95 18 10 8 5 28 8 Roura10 34 47 ± 12 94 NA NA NA NA 15 Alfonso11 27 52 ± 10 85 37 33 52 4 NA 4 Ito12 23 45 ± 11 100 57 22 30 4 NA 30 Vanzetto14 23 46 ± 9 74 26 39 43 13 13 0 Mortensen15 22 49 ± 9 81 38 NA 57 0 40 10 Rashid16 21 53 ± 9 95 48 48 47 5 24 0 Data are given as mean ± standard deviation or percentages. HTN, hypertension; Chol, dyslipidaemia; DM, diabetes mellitus; FH, family history of coronary artery disease; NA, not available; P-SCAD, pregnancy-associated coronary artery dissection. Table 1 Demographics and risk factors of patients with spontaneous coronary artery dissection (SCAD) in contemporary case series (studies with n > 20) Max N Age (years) Gender (female, %) HTN (%) Chol (%) Smoking (%) DM (%) FH (%) P-SCAD (%) Mayo Clinic3 189 44 ± 9 92 31 22 15 2 NA 15 Saw4 168 52 ± 9 92 39 24 13 5 29 2 Lettieri5 134 52 ± 11 81 51 33 34 2 25 NA Faden6 79 33 ± 5 100 17 18 17 11 NA 100 Rogowski7 64 53 ± 11 94 45 52 28 0 19 5 Nakashima8 63 46 ± 10 94 33 23 32 0 8 8 Motreff13 55 50 100 27 11 22 4 22 4 McGrath-Cadell9 40 45 ± 10 95 18 10 8 5 28 8 Roura10 34 47 ± 12 94 NA NA NA NA 15 Alfonso11 27 52 ± 10 85 37 33 52 4 NA 4 Ito12 23 45 ± 11 100 57 22 30 4 NA 30 Vanzetto14 23 46 ± 9 74 26 39 43 13 13 0 Mortensen15 22 49 ± 9 81 38 NA 57 0 40 10 Rashid16 21 53 ± 9 95 48 48 47 5 24 0 Max N Age (years) Gender (female, %) HTN (%) Chol (%) Smoking (%) DM (%) FH (%) P-SCAD (%) Mayo Clinic3 189 44 ± 9 92 31 22 15 2 NA 15 Saw4 168 52 ± 9 92 39 24 13 5 29 2 Lettieri5 134 52 ± 11 81 51 33 34 2 25 NA Faden6 79 33 ± 5 100 17 18 17 11 NA 100 Rogowski7 64 53 ± 11 94 45 52 28 0 19 5 Nakashima8 63 46 ± 10 94 33 23 32 0 8 8 Motreff13 55 50 100 27 11 22 4 22 4 McGrath-Cadell9 40 45 ± 10 95 18 10 8 5 28 8 Roura10 34 47 ± 12 94 NA NA NA NA 15 Alfonso11 27 52 ± 10 85 37 33 52 4 NA 4 Ito12 23 45 ± 11 100 57 22 30 4 NA 30 Vanzetto14 23 46 ± 9 74 26 39 43 13 13 0 Mortensen15 22 49 ± 9 81 38 NA 57 0 40 10 Rashid16 21 53 ± 9 95 48 48 47 5 24 0 Data are given as mean ± standard deviation or percentages. HTN, hypertension; Chol, dyslipidaemia; DM, diabetes mellitus; FH, family history of coronary artery disease; NA, not available; P-SCAD, pregnancy-associated coronary artery dissection. Key Messages Whilst SCAD has been described across a broad demographic, it is a frequent cause of ACS in young- to middle-aged women and patients with myocardial infarction in egnancy or post-partuprm. Pregnancy-associated SCAD accounts for a minority of cases. Pathophysiology: risk factors and associations The pathophysiology of SCAD remains unknown. It is likely that a combination of predisposing factors increase susceptibility such that a relatively minor trigger event is sufficient to precipitate SCAD. There are a large number of reported associations with SCAD (Supplementary material online, Table S2).4,8,9,33,38,44,47–91,117–124,127–133,140–143 For some (e.g. female sex, pregnancy, and FMD), a link with SCAD has been established in multiple series. Other associations are based on anecdotal case reports, and in this context causality is harder to determine. Female sex and pregnancy The vast majority of SCAD patients (∼90%) are women.3–11,14–18 There is some evidence from a Canadian series that male SCAD patients are different from female cases, being slightly younger and with higher rates of preceding isometric exercise and lower prior emotional stress levels.92 The predilection of SCAD for female patients and the association with pregnancy suggest a pathophysiological role for female sex hormones. The precise nature of this association remains to be elucidated but may relate to hormonal influences on vascular connective tissue and/or the vessel microvasculature. Data from most contemporary SCAD series suggest that P-SCAD represents approximately 10% of SCAD cases. Multi-parity, fertility hormones and pre-eclampsia may increase risk.45,93–95 Pregnant and peripartum cases has been reported antepartum as early as the 5th week of pregnancy,96 although most events reported during pregnancy are in the third trimester.97,–99 It also occurs in the early (<6 weeks), late (6 weeks to 12 months), and very late post-partum (12 to 24 month) periods, with the peak occurring in the early post-partum period.95,97–99 Anecdotally, late post-partum SCAD may occur in association with breastfeeding.96 A Canadian study identified 79 P-SCAD cases from a nationwide cohort study of 4.4 million pregnancies between 2008 and 2012 (incidence 1.8 SCAD cases per 100 000 pregnancies),6 a higher incidence of P-SCAD compared to the historical literature. This study suggested the P-SCAD presentation may be more severe than SCAD outside the context of pregnancy, with ST-elevation myocardial infarction (STEMI) in 64%, cardiogenic shock in 24%, cardiac arrest in 14%, and maternal death in 4.5%. Moreover, P-SCAD was more likely to involve the proximal coronaries and was associated with a reduction in post-infarct ejection fraction. A recent report of P-SCAD cases from the US Mayo Clinic Series also reported increased STEMI, proximal or mutivessel disease and worse left ventricular function compared to non-P-SCAD. The findings of a more severe P-SCAD phenotype were confirmed in an analysis of contemporary published cases99 and a small retrospective study comparing 7 P-SCAD and 16 SCAD cases.12 Fibromuscular dysplasia Spontaneous coronary artery dissection has been associated with various predisposing arteriopathies (Figure 3).1,2,92 The most frequent is FMD, a non-atherosclerotic, non-inflammatory disease of arterial walls, which also occurs predominantly in middle-aged females with few cardiovascular risk factors. Fibromuscular dysplasia may lead to stenosis, dissections, and aneurysms of medium-sized arteries, including but not limited to renal, cervico-cephalic, and visceral arteries.100,101 It is currently classified by angiography into two subtypes, multifocal and (uni)focal. Multifocal FMD, with the typical ‘string-of-beads’ pattern, is the angiographic presentation of medial FMD and is at least four times more frequent than unifocal FMD.101–103 Figure 3 View largeDownload slide Extracoronary abnormalities in spontaneous coronary artery dissection including renal (A and D) and femoral (B) fibromuscular dysplasia, carotid and vertebrobasilar aneurysms and tortuosity (C and F) and a localised iliac dissection (E). Figure 3 View largeDownload slide Extracoronary abnormalities in spontaneous coronary artery dissection including renal (A and D) and femoral (B) fibromuscular dysplasia, carotid and vertebrobasilar aneurysms and tortuosity (C and F) and a localised iliac dissection (E). Since the first case series reporting the association of SCAD with extra-coronary FMD in 2012,104,105 FMD of extra-coronary vascular beds has been documented in 11–86% of patients with SCAD,4,9,44,47,50,106–108 with a narrower range of 41–86% after exclusion of three studies in which <50% of patients were screened9,44,47 (Figure 4). The prevalence of extra-coronary FMD in various cohorts may differ according to the proportion of patients screened, the screening protocol used (e.g. imaging technique, vascular beds, screening according to symptoms vs. systematic assessment etc.) and diagnostic criteria of FMD. Fibromuscular dysplasia of extra-coronary vascular beds may also be more frequent in SCAD patients with a higher coronary tortuosity score.47 Figure 4 View largeDownload slide Prevalence of extracoronary lesions of fibromuscular dysplasia in contemporary series of spontaneous coronary artery dissection patients. Figure 4 View largeDownload slide Prevalence of extracoronary lesions of fibromuscular dysplasia in contemporary series of spontaneous coronary artery dissection patients. In current series, the most commonly affected vascular beds are renal, cervico-cephalic and iliac arteries.4,50,106–108 In three studies in which the information was available,104,106,107 29–70% of SCAD patients with extra-coronary FMD had involvement of two or more vascular beds. While Saw et al.4 focused their analysis on the presence of the ‘string-of-beads’ appearance, almost pathognomonic of multifocal FMD, Prasad et al.107 extended vascular screening to isolated stenosis possibly due to unifocal FMD. Notably however, the 52 cases of extra-coronary FMD documented in their series of 115 SCAD patients were exclusively of the multifocal subtype. Finally, besides typical FMD lesions, other extra-coronary vascular abnormalities (EVAs) such as aneurysms, dissections, irregularities, undulations, and/or tortuosity have been reported.106,107 Whether the latter correspond to subtle forms of FMD or a different process such as a form of connective tissue disorder remains to be established107 The clinical implications of the association between EVAs and SCAD are described in the ‘Computed tomography-peripheral angiography or magnetic resonance-angiography section' below. In view of the high prevalence of extra-coronary FMD in patients with SCAD, it is tempting to speculate that SCAD is a complication of underlying coronary FMD in a substantial proportion of cases.108 Pathological demonstration of coronary FMD lesions in at least four cases of SCAD37,109–111 adds weight to this hypothesis. However, at present, a degree of caution is required in equating SCAD with FMD, as (i) current data on the association of SCAD with extra-coronary FMD mostly derive from two centres in Canada4,104 and the USA,44,47,50,106,107 with repeat publications from the same group likely representing different stages of recruitment and exploration of these two cohorts; (ii) typical FMD coronary lesions are rare112,113 and description of the angiographic characteristics of coronary FMD is only in its infancy114,115; (iii) the prevalence of coronary artery dissection is very low (<3%) in the US FMD registry116; and (iv) a proportion of cases of SCAD remain idiopathic or are associated with other vascular diseases. Inflammatory conditions Spontaneous coronary artery dissection has been associated with systemic inflammatory conditions (Supplementary material online, Table S2).33,68–77,117–124 While the prevalence of inflammatory systemic diseases was 8.9% in a Canadian series of 168 cases,4 this is not yet widely corroborated in other series.11 A clear mechanistic link between systemic inflammation and SCAD remains to be elucidated. Atherosclerotic risk factors Significant atherosclerosis is rare in typical SCAD (although this may partly reflect the criteria used to define cases). Spontaneous coronary artery dissection patients have fewer traditional cardiovascular risk factors for ischaemic heart disease than patients with atherosclerotic coronary artery disease,44 and some patients have no such risk factors at all. However, in contrast to the previous perception, many patients do have some risk factors for ischaemic heart disease including hypertension, smoking, and dyslipidaemia3–18 (Table 1), although there is no evidence these contribute directly to the risk of SCAD. For example, the mean low-density lipoprotein cholesterol (LDL-C) plasma concentration in the case series by Rogowski et al.7 was 3.3 mmol/L and some cases of SCAD have been reported in patients with severe hypertension.125,126 In contrast, diabetes seems to be rare in SCAD patients. In keeping with the wider population, coronary risk factors tend to be more prevalent in older patients with SCAD. Mechanical stressors and exercise A number of mechanical triggers have been linked to SCAD events, including extreme Valsalva-type manoeuvres and the provocation of coronary spasm (Supplementary material online, Table S2).4,9,79–91,127–133 Dissections have also been reported following isometric or extreme exercise,134–138 reported in 11.9% of cases in a recent prospective series19 and occurring more commonly in men.92 A mechanism linked to increased coronary wall shear stress has been proposed. There are also patients in whom there is a significant lag time between the last exercise event and the onset of symptoms suggesting a higher exercise performance capability may be a feature of the SCAD phenotype in some patients (potentially relating to a mild connective tissue phenotype).139 Emotional stressors Antecedent emotional stresses such as a bereavement or major personal crisis have been reported in a higher than expected proportion of SCAD cases, particularly in women.4,8,92 Inherited connective tissue disorders Although SCAD has frequently been reported in association with connective tissue disorders (Supplementary material online, Table S2),38,44,50–58,140–143 large contemporary series suggest such cases constitute a tiny minority of prevalent cases (around 1–2% in the US, Canadian, and Japanese series)4,107 and until now the yield from genetic screening of SCAD-survivors for associated genes has been reported to be very low.50 Genetics The genetics of SCAD remains to be clearly elucidated with very few focused studies to date. Whilst a number of sibling–sibling pairs and mother–daughter pairs have been described,144 SCAD outside the rare context of known connective tissue disorders does not appear to be a strongly inherited condition, with only 1.2% of 412 patients in one series describing a family history of SCAD.144 Hence whilst a thorough clinical assessment for rare connective tissue disorders is required in all SCAD-survivors, whether more systematic genetic screening is useful and cost-effective remains to be demonstrated.50,115 Key Messages The strong female predisposition and association with pregnancy suggest a role for female sex-hormones in the pathogenesis of SCAD but the mechanism remains unknown. Spontaneous coronary artery dissection is frequently associated with extra-coronary arteriopathies including FMD. Spontaneous coronary artery dissection is not a strongly inherited condition. Research is needed to better understand the pathophysiology of SCAD. Clinical presentation There is considerable evidence that SCAD remains under-diagnosed.2 Some patients fail to act on their symptoms and never present to medical services and a few cases may present with sudden cardiac death (see pathology section). However, of those who do present to medical services, missed or delayed diagnosis is common.1,145 Some patients are not referred for coronary investigations, primarily because most acute medical and cardiology services are focused on the identification of patients at high risk of obstructive atherosclerotic ACS and patients with SCAD typically fall into the lowest risk groups on the basis of traditional risk scores for ischaemic heart disease. A high index of suspicion in typical patients, coupled with familiarity with the angiographic variants of SCAD is key to minimizing missed or delayed diagnoses. Patients with SCAD usually present with an ACS associated with positive biomarkers of myocardial necrosis (e.g. high sensitivity troponin). The proportion of cases presenting with STEMI (26–55%) vs. non-ST-elevation myocardial infarction (NSTEMI) varies in the larger published series.3–5,8,13,45 This likely reflects differences in patient selection for these registries. A minority present with ventricular arrhythmia (2.8–10%),3–5,8,45 Chest pain is the most frequently described presenting symptom,15,44,146 an association which may be more common in SCAD than atherosclerotic ACS patients (chest pain reported in 60–90% of SCAD patients).146 This may be because, in addition to pain arising from myocardial ischaemia and infarction, dissection per se is inherently painful.145 In the Canadian series, chest pain was reportedly associated with radiation to the arm (49.5%) and neck (22.1%), nausea and vomiting (23.4%), diaphoresis (20.9%), dyspnoea (19.3%), and back pain (12.2%).146 Despite the strong association with chest pain, the nature of the pain may be atypical with ‘burning’ (9%), ‘pleuritic’ (3.0%), ‘tearing’ (1.0%), and ‘positional’ (1%) descriptors in a minority of patients.146 Key Messages Patients with SCAD usually present with ACS. Delayed diagnosis is common and SCAD should be actively considered in the differential diagnosis of ACS presentations in low risk patients. Diagnosis The differential diagnosis for SCAD includes atherosclerotic ACS, coronary artery spasm, Takotsubo cardiomyopathy, coronary thromboembolism, and myocardial infarction with non-obstructed coronary arteries (MINOCA). There are no currently identified specific blood biomarkers for SCAD. Coronary angiography represents the principal tool for the diagnosis of SCAD in clinical practice.1,145,147 Intracoronary nitroglycerin should be given, where blood pressure allows, to ensure complete vasodilation and to rule out the possibility of associated coronary spasm. With experience most SCAD cases can be diagnosed on angiography alone, with intracoronary imaging reserved for cases where diagnostic uncertainty exists.145 However, it is important to appreciate that the appearances of a radiolucent flap, dual lumen, and contrast hold-up seen with iatrogenic dissections148 and familiar to most interventional cardiologists are present in only a minority of SCAD angiograms. Coronary angiographic classification The most commonly used angiographic classification of SCAD has been adapted from Saw et al.149,150 (Figure 5), although other approaches are also proposed (Supplementary material online, Figure S6).13 From the Saw classification, Type 1 represents the classical angiographic radiolucent ‘flap’ and linear double lumen often associated with contrast hold-up (Figure 5A). This reportedly occurs in 29–48% of cases.4,7,8 However these features are absent in the commoner Type 2 pattern (52–67%)4,7,8 characterized by a long diffuse and smooth stenosis predominantly located in mid-to-distal segments. This has been divided into Type 2a (Figure 5B) where there is recrudescence of a normal calibre distal vessel and Type 2b (Figure 5C) where the stenosis extends angiographically to the end of the vessel. Intermediate appearances (between Type 1 and 2) are also seen where a typical Type 2 pattern is seen but with a short segment of dual lumen or contrast hold-up in keeping with a localized fenestration between true and false lumen (Figure 5F). Type 3 lesions (Figure 5D) are defined as angiographically indistinguishable from a focal atherosclerotic stenosis requiring diagnostic confirmation by intracoronary imaging (see intracoronary imaging section). However, these account for a small minority of cases (0–3.9%).4,7,8 Type 4 SCAD is described as a total occlusion, usually of a distal vessel (Figure 5E).145 In this uncommon circumstance, the diagnosis is particularly challenging and frequently can only be established during an ensuing coronary intervention once coronary flow is re-established or inferred by subsequent vessel healing and the exclusion of an embolic cause. Figure 5 View largeDownload slide Angiographic classification of spontaneous coronary artery dissection. Type 1 spontaneous coronary artery dissection (A), Type 2A spontaneous coronary artery dissection (B), Type 2B spontaneous coronary artery dissection (C), Type 3 spontaneous coronary artery dissection (D), Type 4 spontaneous coronary artery dissection (E), and Intermediate Type1/2 spontaneous coronary artery dissection (F). Figure 5 View largeDownload slide Angiographic classification of spontaneous coronary artery dissection. Type 1 spontaneous coronary artery dissection (A), Type 2A spontaneous coronary artery dissection (B), Type 2B spontaneous coronary artery dissection (C), Type 3 spontaneous coronary artery dissection (D), Type 4 spontaneous coronary artery dissection (E), and Intermediate Type1/2 spontaneous coronary artery dissection (F). Additional possible angiographic findings Other angiographic features reported in association with SCAD include: Increased coronary tortuosity47 Predilection for more distal coronary segments (in contrast to atherosclerotic disease)4,5,44,45 Predominant involvement of the left anterior descending coronary artery and its branches reported in most4,5,8,13,44 but not all45 series False lumen starting and/or ending at a side branch13 Absence or reduced incidence of co-existent atherosclerosis—unaffected coronaries are usually normal or near-normal13 Coronary FMD (see fibromuscular dysplasia section) Association of sites of dissection with myocardial bridging.151 Multi-vessel spontaneous coronary artery dissection Multi-vessel SCAD (Figure 6) is defined as simultaneous dissections occurring in more than one artery, without continuity, and is thus distinct from recurrent SCAD or a continuous dissection which extends into different coronary territories. It is well recognized152–156 with a reported frequency of 5–13%3–5,8,13,45 in the larger series and careful angiographic assessment of apparent non-culprit vessels during index angiography is recommended. There are no current specific studies of multi-vessel SCAD; however, the approach to management for multi-vessel SCAD should not differ from single vessel disease with each lesion being assessed for intervention on its individual merits and a preference for conservative management where possible (see conservative management section). Figure 6 View largeDownload slide Multivessel spontaneous coronary artery dissection affecting two branches of the circumflex coronary artery, the left anterior descending coronary artery and its diagonal branch (arrows). Figure 6 View largeDownload slide Multivessel spontaneous coronary artery dissection affecting two branches of the circumflex coronary artery, the left anterior descending coronary artery and its diagonal branch (arrows). Secondary iatrogenic dissection in spontaneous coronary artery dissection A single study has reported an increased risk of secondary iatrogenic dissections during angiography and percutaneous coronary intervention (PCI) in SCAD patients (2% risk during coronary angiography vs. 0.2% during non-SCAD angiography, 14.3% during PCI).157 High rates of secondary iatrogenic dissection were also reported in a second series (11 of 189 SCAD cases) but without a comparator population.3 For this reason, a meticulous co-axial catheter technique and avoidance of aggressive or deeply engaging guiding catheter designs is advised. Intracoronary imaging Most SCAD can be diagnosed angiographically and in scenarios where a conservative approach to management is feasible, coronary instrumentation should, if possible, be avoided.19,106,108 However, where diagnostic uncertainty exists or to guide coronary intervention when required, careful intracoronary imaging can be invaluable and appears safe.13,30 Because SCAD frequently affects more distal coronary segments, complete imaging of the axial extent of the false lumen may not be possible (e.g. for Type 2b SCAD). In one series only 5/11 affected cases could be imaged across the entire affected length.30 However imaging of shorter proximal segments may be sufficient for diagnostic purposes.30 Intravascular ultrasound (IVUS) and OCT provide tomographic images of the vessel wall and the coronary lumen that have proved to be of major value in the diagnosis of SCAD.13,30,31,158,159 Intracoronary imaging can also help to guide decision-making on stent size. The length of the stent may be planned according to the longitudinal extent of the false lumen with the aim of minimizing propagation of intramural haematoma (see percutaneous coronary intervention section). The reported risk of late mal-apposition on haematoma resorption due to stent under-sizing in the presence of extensive haematomas160 can also potentially be minimized by careful assessment of proximal and distal vessel dimensions with IVUS/OCT. Although there are relative advantages to each technology, OCT is generally favoured for SCAD imaging because of its higher spatial resolution.1,145 Intravascular ultrasound Intravascular ultrasound (axial resolution 150 μm) is able to differentiate atherosclerotic plaques from SCAD (Figure 7A).31,158,159,161–165 This technique readily depicts the true and the false coronary lumens and is able to demonstrate the extent of false lumen thrombosis. The principal advantage of IVUS over OCT is firstly, that blood clearance (and therefore pressurized contrast injection) is not required and secondly, IVUS has superior depth penetration, enabling complete visualization of the vessel wall to the external elastic lamina, including imaging through thrombus. The main limitation is poor spatial resolution which can limit identification of small structures associated with SCAD such as the intimal-medial membrane and localized fenestrations connecting true and false lumens.145 Figure 7 View largeDownload slide Intracoronary imaging of spontaneous coronary artery dissection by intravascular ultrasound (with outer border of false lumen arrowed, A), and optical coherence tomography showing partial (B) and circumferential (C) false lumens; the site of a fenestration (D) connecting true and false lumens and reduced light penetration through the false lumen (E). Three-dimensional image derived from segmentation of optical coherence tomography image showing how the false lumen tracks around the true lumen and is influenced (and frequently bounded) by side branches (F). Figure 7 View largeDownload slide Intracoronary imaging of spontaneous coronary artery dissection by intravascular ultrasound (with outer border of false lumen arrowed, A), and optical coherence tomography showing partial (B) and circumferential (C) false lumens; the site of a fenestration (D) connecting true and false lumens and reduced light penetration through the false lumen (E). Three-dimensional image derived from segmentation of optical coherence tomography image showing how the false lumen tracks around the true lumen and is influenced (and frequently bounded) by side branches (F). Optical coherence tomography Optical coherence tomography has the advantage of much higher spatial resolution (axial resolution 15 μm), and characteristic images of SCAD are well described (Figure 7B–E).30,42,150,159,166 Imaging with OCT necessitates blood clearance requiring a high pressure contrast injection which carries a potential risk of extension of the false lumen, especially in proximal Type I SCAD. However, reports to date suggest, with care, OCT imaging in SCAD can be conducted safety.13,30,150 Depth penetration is more limited with OCT, exacerbated by shadowing or attenuation e.g. by thrombus or haematoma. However this generally does not limit accurate diagnosis. Optical coherence tomography enables detailed characterization of: the true lumen including any associated thrombus; the size, nature, and extent of the false lumen including points where the false lumen surrounds the true lumen circumferentially; the relationship of the false lumen with side-branches; fenestrations or the more classical ‘entry tear’ connecting true and false lumens.30 Accurate measurement of key features is also practical including the thickness of the intimal-medial membrane (mean 350 μm)30 and the dimensions of the compressed true lumen. Optical coherence tomography has also been used to report areas characterized as ‘coronary FMD’ (see fibromuscular dysplasia section). It is important to recognize that the light attenuation (blackness) of the false lumen is highly variable (presumably reflecting phases in the maturation of the intramural thrombus and variability in contrast penetration of the false lumen).145 Careful assessment of the images is required to identify the classical crescentic semi-lunar false lumen (Figure 7B–E) and ensure an accurate OCT diagnosis. Optical coherence tomography is also of value in the subset of SCAD patients requiring coronary revascularization.30,167 Before any intervention it is critical to confirm, if there is any uncertainty, that the guidewire is located in the true lumen (Figure 8) as stenting into the false lumen can have serious consequences.168,–170 Long-term follow-up OCT has also been reported in a few cases showing the stages of vessel healing. In most cases restitutio ad integrum of the previously affected vessel wall can be appreciated.171 One report described an increase in the vasa vasorum density during the healing phase (median 44 days after presentation) of SCAD.172 Given the reported increased risk of iatrogenic dissection in SCAD patients, repeat imaging should only be considered where clinically necessary. Figure 8 View largeDownload slide Guidewire passage into the false lumen demonstrated by optical coherence tomography, with both wire (GW) and optical coherence tomography catheter (C) seen in the false lumen outside the compressed true lumen. Figure 8 View largeDownload slide Guidewire passage into the false lumen demonstrated by optical coherence tomography, with both wire (GW) and optical coherence tomography catheter (C) seen in the false lumen outside the compressed true lumen. Computed tomography coronary angiography There is currently limited data to support the use of computed tomography coronary angiography (CTCA) for the diagnosis of acute SCAD. However, this imaging modality is increasingly used clinically to assess acute chest pain presentations. For SCAD, it has the advantage of being non-invasive (allowing coronary assessment without the increased risk of iatrogenic dissection reported with invasive angiography. However it is limited by lower spatial resolution, of particular importance for accurate assessment and interpretation of the smaller mid-to-distal coronary territories for which SCAD has a predilection.173 Typical CTCA appearances have been described147,174 (Figure 9A) and are consistent with angiographic and OCT findings where some cases show clear-cut contrast penetration of the false lumen leading to a ‘classical’ dual lumen but many show compression of the true-lumen by haematoma but without contrast penetration. The sensitivity and specificity of CTCA as a primary diagnostic investigation for SCAD are not known and false negative findings are reported.173,175 In this context at present, patients in whom SCAD is suspected (e.g. low atherosclerotic risk young- to middle-aged females with typical symptoms and positive biomarkers) are recommended to undergo coronary angiography as the primary diagnostic investigation of choice. CTCA may however have a role in the follow-up assessment of SCAD (Figure 9B, see coronary angiography or computed tomography-coronary angiography section). Key Messages Most SCAD can be diagnosed by coronary angiography and a working knowledge of the typical angiographic findings is key. Intracoronary imaging with OCT appears safe and should be considered where there is diagnostic uncertainty. Multi-vessel SCAD is common and careful assessment for this is required during angiography. Figure 9 View largeDownload slide Coronary computed tomography findings with parallel angiographic images for a Type 2A dissection of the left anterior descending coronary artery at presentation (A) and following healing after 3-months follow-up (B). Figure 9 View largeDownload slide Coronary computed tomography findings with parallel angiographic images for a Type 2A dissection of the left anterior descending coronary artery at presentation (A) and following healing after 3-months follow-up (B). Acute management Conservative management There is good evidence that the majority of SCAD will first stabilize and then heal completely over time if managed conservatively (Supplementary material online, Figure S6).3,4,7,8,11 Revascularization in patients with SCAD is very challenging due to the presence of an underlying disrupted and friable coronary vessel wall. This is widely reported to lead to worse outcomes for PCI than in atherosclerotic coronary disease.3–5,7,8 For this reason where revascularization is not mandated (i.e. in haemodynamically stable patients with maintained distal flow in the culprit coronary and without demonstrable ongoing ischaemia) a conservative strategy is generally favoured.1,2,145 The proportion of healed cases described in contemporary series depends on the number of cases undergoing follow-up angiography and the timing and indication for repeat assessment. The US Mayo Clinic series reported on 59 patients (from 95 managed conservatively) who underwent repeat angiography for a range of reasons a median of 2.4 years after the index event. In all, 73% (43/59) were described as ‘healed’.3 Likewise, the Canadian series reported late revascularization in 3/134 conservatively managed cases but complete healing in all 79 of the remaining cases who underwent repeat angiography73; a Japanese series reported 68% healing in 28 conservatively managed patients assessed early by CTA a median of 3.4 months post event8; a Swiss series reported healing in all but one of 36 from 56 conservatively managed patients who underwent repeat angiography 6 months after the event.7 It does appear that a small number of cases initially managed conservatively subsequently require revascularization. In a recent prospective series, 9 of 272 (3.3%) patients managed conservatively required subsequent in-hospital revascularization.19 This may depend in part on the threshold for repeat investigation and intervention. It should be noted that dissections are often inherently painful and careful consideration should be given as to whether ongoing symptomatic chest pain is ischaemic in origin before proceeding to repeat angiography or intervention on a symptomatic basis.145 As the majority of cases failing a conservative management strategy occur early during follow-up, prolonged inpatient monitoring (∼5 days) in conservatively managed SCAD is suggested.1,2,148 Further prospective research is required to better understand vascular healing after SCAD and the characteristics and management approach to delayed or failed healing. Percutaneous coronary intervention Published studies consistently show an increased risk of coronary complications with PCI.3–5,7,8 In the Canadian series, revascularization procedural success was only achieved in 64% of patients and, in addition to that, only 30% of patients maintained durable results at long-term follow-up.4 In the large series from the Mayo Clinic, most patients (2/3 of the total cohort) underwent coronary revascularization during initial hospitalization.3 Coronary interventions, however, were associated with high complication rates. Procedural success was only achieved in 57% of cases. Furthermore revascularization was not associated with a reduced long-term risk of repeated revascularization or recurrent SCAD.3,8 Where ongoing ischaemia or infarction mandates intervention, interventional cardiologists should be mindful of specific additional risks associated with SCAD interventions. These include: Increased risk of secondary iatrogenic dissection Guidewire passage into the false lumen3,168–170 (Figure 8) Proximal and/or distal false lumen propagation during stent deployment33,36,38,39,41 (Figure 10) Persistent distal dissection Major side branch restriction or occlusion by propagation of haematoma Figure 10 View largeDownload slide False lumen propagation during stenting. A Type 2A dissection affecting a high obtuse marginal branch of the circumflex but with maintained distal flow (A). Stenting (B) leads to proximal haematoma migration (arrowed C). Two further stents were required in this case to fully exclude the haematoma from luminal restriction. Figure 10 View largeDownload slide False lumen propagation during stenting. A Type 2A dissection affecting a high obtuse marginal branch of the circumflex but with maintained distal flow (A). Stenting (B) leads to proximal haematoma migration (arrowed C). Two further stents were required in this case to fully exclude the haematoma from luminal restriction. Where stents are deployed, second generation drug-eluting stents (DES) are advised. Significant rates of in-stent restenosis are reported in one retrospective series [23/44 target vessel revascularization (TVR) from 87 patients managed by PCI and followed-up for a median 2.3 years], although the drug elution status of the stents used is not described.3 In a recent retrospective multicentre study of 238 SCAD patients,176 108 underwent PCI with DES or bare metal stents (BMS). Overall, 24 patients (22.2%) suffered procedural-related complications. After a median follow-up of 3 years there was a trend towards a reduction in major adverse cardiac events after DES compared with BMS (26 vs. 39%, P = 0.14) mainly driven by TVR (4 vs. 18%, P = 0.08). Although the differences did not achieve statistical significance, the trends are consistent with existing knowledge about the relative risk of BMS vs. DES in non-SCAD PCI. A recent meta-analysis also suggested an additional TVR risk of 6.3% in patients treated with revascularization.177 A single case has been reported of late strut mal-apposition purportedly due to the effects of haematoma resorption160 although to date an increased incidence of stent thrombosis has not been confirmed. Adequate stent sizing and expansion is therefore likely to be important but stent post-dilation/optimization is a balance between the risk of haematoma propagation and ensuring optimal strut expansion. Given the increased risk of adverse outcomes with PCI in SCAD, a number of less conventional interventional approaches have been reported. These include: Minimal plain old balloon angioplasty (POBA) to restore flow followed by a conservative strategy178 Extended stent lengths to reduce the chances of proximal or distal haematoma propagation Sealing the proximal and distal extremes of the affected segments with short stents to restrict the haematoma before stenting the intermediate segments.179,180 Targeting an intimal tear or ‘flap’ for focal stenting or stenting just the proximal extent of the dissection to prevent proximal propagation.8,181 Cutting balloon inflation to fenestrate the intimal-medial membrane and depressurize the false lumen as a stand-alone strategy or prior to stenting.182–185 Use of bioresorbable coronary scaffolds.184,186–188 These anecdotal case reports are subject to publication bias. Additionally, the role of bioresorbable coronary scaffolds more generally has been the subject of recent scrutiny.189 In the absence of randomized data, no specific alternative PCI strategy can at present be specifically recommended. Coronary artery bypass grafting Coronary artery bypass grafting (CABG) in SCAD is generally used as a bail-out strategy either for a failure of PCI with ongoing ischaemia or infarction of a significant at-risk myocardial territory (e.g. failure to wire the true lumen distal to a SCAD occlusion) or because the site and extent of the dissection (usually involving the left main stem or the presence of multiple dissections in different vessels) is felt to pose a prohibitive risk with either a conservative or a PCI strategy. Successful grafting may be challenging where the dissection extends beyond the graft anastomosis site and great care must be taken to ensure anastomosis to the true lumen.190 Coronary artery bypass grafting using arterial or venous conduits and both off-pump and robotic techniques are described but the literature on CABG in SCAD is limited to case reports and small case series (5–23 cases).3,6,12,14 Early outcomes from these limited data on CABG are reported to be good but the Mayo Clinic Series reported high rates of graft failure at follow-up, perhaps due to healing of the native coronary leading to competitive flow and conduit thrombosis.3 Adjunctive supportive devices and transplant For details, see Supplementary material online.191–,208 Medical management There are to date no randomized controlled trials comparing different pharmacological treatment strategies for SCAD. Current practice is therefore based on case and registry observations, clinical experience and the extrapolation (where appropriate) of guidelines for non-SCAD ACS treatment. Thrombolysis Although individual historical cases of SCAD managed apparently successfully with thrombolysis have been described,209 there are also reports of dissection extension and even coronary rupture leading to cardiac tamponade following lytic therapy.210–213 Thrombolysis is therefore contraindicated for the acute management of SCAD. Antiplatelet therapies The use of antiplatelet therapies and the duration of treatment remains an area of controversy and divergent practice in SCAD. This results from an apparent conflict between the strength of existing data of efficacy from non-SCAD ACS vs. an inherent concern (albeit unproven) about using medications that prolong bleeding time for a condition whose primary pathophysiology may be an intramural bleed.145 This may be further complicated by problematic menorrhagia which can be an issue in SCAD-survivors of menstrual age taking antiplatelet therapies.214 Patients who undergo stenting should receive dual antiplatelet therapy for 12 months and prolonged or lifelong monotherapy (usually with aspirin) in accordance with current ACS guidelines.206,207 In patients managed conservatively, there is evidence from OCT studies of high grade stenosis sometimes with true luminal thrombus in association with SCAD.30,166 This provides justification for antiplatelet therapy in the acute phase and most authors advocate acute dual antiplatelet therapy (usually with aspirin and clopidogrel rather than the newer P2Y12 inhibitors and avoiding intravenous antiplatelet therapies).19,106,108 The optimal duration of dual and subsequent monotherapy remains unknown with some authors advocating lifelong aspirin19,108 and others questioning this approach.106 Anticoagulant therapies The same concerns about the potential adverse impact described for antiplatelet therapies also apply to anticoagulant treatments. Anticoagulation should probably be limited to acute administration during revascularization procedures while chronic use should be restricted to situations where there is an unequivocal clinical indication (such as left ventricular thrombus or thromboembolism) which should over-ride what is at present a theoretical risk.145 Angiotensin converting enzyme inhibitors, angiotensin receptor antagonists, mineralocorticoid receptor antagonists, beta-blockers, and vasodilator therapies Medical management of SCAD patients with significant impairment of left ventricular systolic function should follow current guidelines aimed at maximizing angiotensin converting enzyme (ACE) or angiotensin receptor blocker (ARB) and β-blocker doses and adding in a mineralocorticoid receptor antagonist (MRA) as indicated,206,207 although hypotension frequently limits dose escalation in this younger population. More controversial is the management of SCAD-survivors without significant impairment of left ventricular systolic function. One recent multivariate analysis from a prospective cohort reported an association between hypertension and an increased risk of recurrent SCAD and β-blocker treatment with a reduced risk of recurrence.19 These findings are not from a randomized study and await validation in other cohorts215 but if confirmed provide the first evidence that SCAD recurrence risk may be reduced therapeutically. Vasodilatory therapies (e.g. nitrates or calcium channel blockers) are reserved for the empirical treatment of chest pain during the acute phase and recurrent chest pain following the index event (see post-spontaneous coronary artery dissection chest pain and its management section). Statins The rationale for prescribing statins for a condition whose pathophysiology has no known association with cholesterol is unclear. One small study reported higher statin use in patients with SCAD recurrence44 but this non-randomized finding should be interpreted with caution and an adverse signal was not reported in a larger prospective cohort.19 In general, statins are reserved for patients with conventional indications for treatment independent of their SCAD event. Contraception and hormone replacement therapy Concerns about hormonal contraception62,63 and hormone replacement therapy30 following SCAD are largely based on the presumed pathophysiological association between SCAD and female sex hormones arising from the female sex predominance and the known association of SCAD with the peripartum period.1,2 However, the exact nature of this association remains to be elucidated. A number of cases have been reported of SCAD in association with exogenous sex hormones but given the high prevalence of use in the population, a causative link has yet to be determined. At present a reasonable strategy may be to avoid hormonal contraception where possible. In patients with recurrent cyclical chest pain following SCAD, low dose local hormone delivery intrauterine contraceptive devices have been anecdotally reported to be helpful.145 The use of the levonorgestrel releasing intra uterine system (LNG-IUS) or endometrial ablation may also be considered in women with prolonged, severe menorrhagia (e.g. where antiplatelet therapies are mandated in the context of previous stenting). Key Messages Coronary revascularization is associated with an increased risk of complications and adverse outcomes compared with atherosclerotic coronary disease. Conservatively (without revascularization) managed SCAD usually heals completely over a few months. Where flow is maintained and in the absence of ongoing ischaemia or infarction, a conservative approach should be considered followed by a period of inpatient observation. Further research is needed to clarify the optimal PCI strategy in cases where revascularization is necessary. The acute and convalescent medical strategy in SCAD may have key differences from post atherosclerotic AMI and further research is needed to establish the optimal treatment approach. Pregnancy Pregnancy-associated spontaneous coronary artery dissection: special considerations Special considerations for the management of P-SCAD include the avoidance of teratogenic drugs, minimization of exposure of the unborn foetus to ionizing radiation216 and timing of delivery to minimize as much as possible risk to both mother and baby. Obstetric issues are reviewed elsewhere96 and are not considered in detail here but coronary management should not differ greatly from other SCAD patients with diagnosis by limited coronary angiography and a management preference for a conservative approach to revascularization where practical. Pregnancy after spontaneous coronary artery dissection There is a single published report of nine pregnancies in SCAD-survivors with one recurrence occurring in a patients whose first event was not peripartum.217 The degree of left ventricular impairment post-SCAD will also contribute independently to the risk. Some authors have advocated a blanket recommendation to avoid pregnancy in SCAD-survivors.1,2 Patients should certainly be carefully counselled before contemplating pregnancy, potentially teratogenic drugs discontinued, and any planned or un-planned pregnancy should be considered high risk and monitored accordingly. Key Messages P-spontaneous coronary artery dissection should be managed by a multidisciplinary team with individualized cardiovascular and obstetric management. There is limited data on the risk of pregnancy in SCAD-survivors but at present pregnancy should be considered high risk pending further research to better quantify this risk. Outcomes and follow-up Follow-up imaging Echocardiography/cardiac magnetic resonance imaging Following SCAD, as with myocardial infarction of other causes, an assessment of left ventricular systolic function is mandatory to guide medical and potentially device therapy.206,207 Computed tomography-peripheral angiography or magnetic resonance-angiography In view of the association between SCAD, FMD and other underlying EVAs, affecting multiple vascular beds (see fibromuscular dysplasia section) and the potential implications for patient management and follow-up,106 imaging of extra-coronary vascular beds in patients with SCAD is advised.101,103,106 Saw et al.1 have advocated non-selective angiography of the corresponding arteries with a pigtail catheter on the occasion of the index coronary angiography. However, this strategy has the potential risks of additional vascular imaging, catheter manipulation and prolonged procedural times in unstable patients, with possibly increased vascular fragility.106 Furthermore, this approach requires separate non-invasive imaging of cerebral aneurysms, which have been identified in 8 to 14% of cases despite a low screening rate, sometimes in the absence of typical FMD lesions.4,104,107 Alternatively, Liang et al.106 have shown the feasibility of a dedicated CTA protocol from the neck to the pelvis, with low-osmolar contrast agents and radiation dose-limiting techniques. While the sensitivity of CTA is lower than that of conventional angiography, it is unlikely to miss clinically relevant FMD lesions. MR-angiography leads to a further decrease in spatial resolution but remains a reasonable, radiation-free alternative,105 and may be preferred in case of diabetes, renal insufficiency or iodine-contrast intolerance. There is currently no specific data on follow-up of EVAs identified in SCAD patients and therefore currently this should follow best practice for similar conditions in non-SCAD patients. Coronary angiography or computed tomography-coronary angiography Whilst it is clear that the majority of conservatively managed SCAD heals completely over time, some cases of persistent dissection are reported (see conservative management section). The value of follow-up coronary imaging to determine SCAD-healing and how this should be used to guide subsequent management remains unclear. It may be relevant, for decision-making about the duration of anti-platelet therapy (see antiplatelet therapies section) or in symptomatic patients where the diagnosis is not clear (see post-spontaneous coronary artery dissection chest pain and its management section). Non-invasive imaging is attractive as invasive angiography in SCAD patients has been associated with an increased risk of iatrogenic dissections (see secondary iatrogenic dissection in spontaneous coronary artery dissection section). Computed tomography coronary angiography has been proposed as an alternative although current data are limited to single case discussions and one small series. Although the issue of spatial resolution in the smaller, more distal, SCAD coronary sites is still relevant (see computed tomography coronary angiography section), CT may have greater utility in assessing healing where the site of dissection has already been determined by angiography (Figure 9B).147,218 Roura et al.10 reported the largest series of patients with SCAD studied by multi-slice CT (MSCT) at follow-up (24 cases). In 83% of cases, complete resolution of SCAD was shown. Further data is required before CTCA can be recommended for follow-up SCAD imaging. Where essential, invasive angiography should be performed with meticulous attention to minimize the risk of iatrogenic dissection. Prognosis: mortality, recurrence risk, and major adverse cardiac events Outcomes following SCAD are summarized in Supplementary material online, Table S3.3,5,7,8,19, In patients surviving SCAD, long-term mortality is low. In the US Mayo Clinic series 10-year survival from Kaplan-Meier estimates is reported at 92%.44 Similarly an Italian series reported 94.4% 6-year survival,5 whilst a Swiss series reported no deaths after the index event in 63 patients followed-up to a median 4.5 years,7 a Japanese series reported one death from 63 patients followed up for a median 34 months8 and a prospective Canadian series report 1.2% mortality at median follow-up 3.1 years.19 However this masks significant morbidity. The overall major adverse cardiac events (MACE) rate in SCAD patients is significant but with considerable variation between published series (47.4% MACE over 10-years from Kaplan–Meier estimates in the US series;44 MACE in the prospective Canadian series was 19.9% over median 3.1 years follow-up;19 5-year MACE in the Japanese series was 37%;8 in the Italian series 6-year MACE was 14.6%).5 This is primarily driven by recurrent dissections and a high rate of target vessel failure in patients undergoing PCI (see percutaneous coronary intervention section).3 Recurrence in SCAD has been widely reported.1,3–5,7,8,44 The US series reported SCAD recurrence in 17% of patients across a median follow-up period of 47 months with a 10-year recurrence rate of 29.4% [the median time to a second event was 2.8 years (ranging from 3 days to 12 years)]44; the Canadian prospective series report recurrent de novo SCAD in 10.4% of 327 patients followed up for a median 3.1 years and a recurrent MI rate of 16.8% in the same cohort19; the Japanese series reported seven recurrent SCAD after the first 30-days from the index event from 63 patients followed up for a median 34 months8; the Swiss series report 3/63 recurrent SCAD followed-up for a median 4.5 years7; the Italian series report 4.7% recurrence over a median 22 month follow-up].5 Recurrence often appears to affect new territories (e.g. in 12/15 patients in the US series)44 and stenting at the time of the first event does not appear to be protective. Although reported rates may represent a slight overestimation of true recurrence rates due to the selection bias inherent to self-referral based cohort studies and prospective data is needed, recurrences are certainly a justified concern in SCAD patients. One study has reported a borderline association between recurrence and increased coronary tortuosity, although it is unclear if tortuosity is a marker of an underlying vasculopathy or provides a mechanism for arterial injury.47 Apart from the potential benefit of betablockers and control of hypertension previously described, no current treatment strategy has, to date, been shown to reduce rates of recurrence. Post spontaneous coronary artery dissection chest pain and its management Recurrent chest pain, often with associated hospital admission, is common after SCAD. In some patients symptoms occur cyclically, usually pre-menstrually.219 Given the SCAD recurrence risk, patients presenting with recurrent chest pain require careful assessment with serial electrocardiography (ECG) and high sensitivity troponin measurement. However, given the reported increased risk of secondary iatrogenic dissection in SCAD patients (see secondary iatrogenic dissection in spontaneous coronary artery dissection section), invasive angiography should be reserved for patients with hard evidence of ischaemia or myocardial necrosis. A role for CTCA to rule out recurrent SCAD, when evaluating post SCAD chest pain, although potentially attractive, remains to be clearly elucidated (see coronary angiography or computed tomography-coronary angiography section). Anecdotally, where recurrent SCAD has been excluded, symptoms may respond to a treatment strategy aimed at reducing vasospasm with vasodilator therapies (where left ventricular function is normal reducing blood pressure lowering medications may be necessary to allow the initiation of vasodilators).145 Likewise it has been reported that cyclical symptoms may respond to low dose contraception (e.g. the progesterone hormonal coil).145 Cardiac rehabilitation and exercise For details, see Supplementary material online.220,221 Post-traumatic stress disorder and the emotional and psychological consequences of spontaneous coronary artery dissection For details, see Supplementary material online.220,222 Key Messages Although the prognosis following SCAD appears good, recurrent SCAD is well recognized. Assessment for extra-coronary arteriopathies is advised in SCAD-survivors. Cardiac rehabilitation should be considered in SCAD patients and a return to full-activity with an avoidance of extreme or isometric exercise encouraged. Recurrent chest pain after SCAD is common and requires careful assessment and management. Management considerations Whilst there are several areas in the management of patients with SCAD where the optimal approach remains uncertain and research is needed to provide evidence to justify firm recommendations (see research priorities section), the following considerations may be useful for clinicians: Early coronary angiography should be considered to exclude the diagnosis of SCAD in patients presenting with clinical features of ACS but at low risk of atherosclerotic AMI, in particular young to middle aged women Where angiographic diagnosis of SCAD is uncertain, intracoronary imaging with OCT should be considered In clinically stable patients with maintained coronary flow, a conservative management strategy is preferred because of the increased risk of adverse outcomes with revascularization In patients with confirmed SCAD imaging for extra-coronary arteriopathies is advised A diagnosis of SCAD should be actively sought at post mortem in unexplained cases of sudden cardiac death by careful assessment of the full length of the coronary tree Research priorities Despite huge progress in the clinical characterization of SCAD from an international effort to register, assess and follow-up patients, this remains a condition for which the pathophysiology is poorly understood and for which, despite a significant recurrence risk, there is no specific disease-modifying therapy. Current international efforts are focused on building large prospectively recruited multicentre cohorts, phenotyped by state-of-the-art imaging for pathophysiological (including genetic) and clinical studies. For example, the European Observational Research Platform (EORP) SCAD study will open for recruitment in the summer of 2018. Prospective studies (including ultimately randomized studies) assessing the best medical therapies (e.g. role and duration of antiplatelet therapy, use of beta-blockers and other secondary prevention drugs) as well as the optimal coronary intervention strategy are urgently needed. It is hoped these ongoing collaborative research efforts will shed new light and expand knowledge on this relatively rare, elusive and challenging clinical entity. Acknowledgements We wish to acknowledge our European patients with SCAD, EURODIS, BeatSCAD; the ESC-ACCA SCAD Study Group and the ACCA secretariat—in particular Eugenie Delaveau and Celine Serio; Dr J. Cade for the image in Figure 9, Dr S Parsons for Figure 2. A Persu is grateful to Prof. Patrick Chenu (Cardiology Department, Cliniques Universitaires Saint Luc, UCL, Brussels, Belgium) for sharing his knowledge and experience on Spontaneous Coronary Artery Dissection (SCAD) and to Mr Xiang Li, medical student at the same University for his contribution to Figure 4 and literature review. Funding British Heart Foundation, the NIHR RD-TRC and the Leicester NIHR Biomedical Research Centre to D.A. Conflict of interest: D.A. declares grants from Astra Zeneca and St Jude Medical (now part of Abbott Vascular) as well as grants from the British Heart Foundation, NIHR RD-TRC and BeatSCAD. H.B. reports grants and personal fees from ASTRA ZENECA, personal fees from DAICHII-SANKYO, personal fees from ELI-LILLY, personal fees from BAYER, personal fees from SANOFI, during the conduct of the study; personal fees from NOVARTIS, personal fees from BMS-PFIZER, from SERVIER, outside the submitted work. Footnotes The opinions expressed in this article are not necessarily those of the Editors of the European Heart Journal or of the European Society of Cardiology. References 1 Saw J , Mancini GB , Humphries KH. Contemporary review on spontaneous coronary artery dissection . J Am Coll Cardiol 2016 ; 68 : 297 – 312 . Google Scholar Crossref Search ADS PubMed 2 Tweet MS , Gulati R , Hayes SN. Spontaneous coronary artery dissection . Curr Cardiol Rep 2016 ; 18 : 60. Google Scholar Crossref Search ADS PubMed 3 Tweet MS , Eleid MF , Best PJ , Lennon RJ , Lerman A , Rihal CS , Holmes DR Jr. , Hayes SN , Gulati R. Spontaneous coronary artery dissection: revascularization versus conservative therapy . Circ Cardiovasc Interv 2014 ; 7 : 777 – 786 . Google Scholar Crossref Search ADS PubMed 4 Saw J , Aymong E , Sedlak T , Buller CE , Starovoytov A , Ricci D , Robinson S , Vuurmans T , Gao M , Humphries K , Mancini GB. Spontaneous coronary artery dissection: association with predisposing arteriopathies and precipitating stressors and cardiovascular outcomes . Circ Cardiovasc Interv 2014 ; 7 : 645 – 655 . Google Scholar Crossref Search ADS PubMed 5 Lettieri C , Zavalloni D , Rossini R , Morici N , Ettori F , Leonzi O , Latib A , Ferlini M , Trabattoni D , Colombo P , Galli M , Tarantini G , Napodano M , Piccaluga E , Passamonti E , Sganzerla P , Ielasi A , Coccato M , Martinoni A , Musumeci G , Zanini R , Castiglioni B. Management and long-term prognosis of spontaneous coronary artery dissection . Am J Cardiol 2015 ; 116 : 66 – 73 . Google Scholar Crossref Search ADS PubMed 6 Faden MS , Bottega N , Benjamin A , Brown RN. A nationwide evaluation of spontaneous coronary artery dissection in pregnancy and the puerperium . Heart 2016 ; 102 : 1974 – 1979 . Google Scholar Crossref Search ADS PubMed 7 Rogowski S , Maeder MT , Weilenmann D , Haager PK , Ammann P , Rohner F , Joerg L , Rickli H. Spontaneous coronary artery dissection: angiographic follow-up and long-term clinical outcome in a predominantly medically treated population . Catheter Cardiovasc Interv 2017 ; 89 : 59 – 68 . Google Scholar Crossref Search ADS PubMed 8 Nakashima T , Noguchi T , Haruta S , Yamamoto Y , Oshima S , Nakao K , Taniguchi Y , Yamaguchi J , Tsuchihashi K , Seki A , Kawasaki T , Uchida T , Omura N , Kikuchi M , Kimura K , Ogawa H , Miyazaki S , Yasuda S. Prognostic impact of spontaneous coronary artery dissection in young female patients with acute myocardial infarction: a report from the Angina Pectoris-Myocardial Infarction Multicenter Investigators in Japan . Int J Cardiol 2016 ; 207 : 341 – 348 . Google Scholar Crossref Search ADS PubMed 9 McGrath-Cadell L , McKenzie P , Emmanuel S , Muller DW , Graham RM , Holloway CJ. Outcomes of patients with spontaneous coronary artery dissection . Open Heart 2016 ; 3 : e000491. Google Scholar Crossref Search ADS PubMed 10 Roura G , Ariza-Sole A , Rodriguez-Caballero IF , Gomez-Lara J , Ferreiro JL , Romaguera R , Teruel L , de Albert M , Gomez-Hospital JA , Cequier A. Noninvasive follow-up of patients with spontaneous coronary artery dissection with CT angiography . JACC Cardiovasc Imaging 2016 ; 9 : 896 – 897 . Google Scholar Crossref Search ADS PubMed 11 Alfonso F , Paulo M , Lennie V , Dutary J , Bernardo E , Jiménez-Quevedo P , Gonzalo N , Escaned J , Bañuelos C , Pérez-Vizcayno MJ , Hernández R , Macaya C. Spontaneous coronary artery dissection: long-term follow-up of a large series of patients prospectively managed with a “conservative” therapeutic strategy . JACC Cardiovasc Interv 2012 ; 5 : 1062 – 1070 . Google Scholar Crossref Search ADS PubMed 12 Ito H , Taylor L , Bowman M , Fry ET , Hermiller JB , Van Tassel JW. Presentation and therapy of spontaneous coronary artery dissection and comparisons of postpartum versus nonpostpartum cases . Am J Cardiol 2011 ; 107 : 1590 – 1596 . Google Scholar Crossref Search ADS PubMed 13 Motreff P , Malcles G , Combaret N , Barber-Chamoux N , Bouajila S , Pereira B , Amonchot A , Citron B , Lusson JR , Eschalier R , Souteyrand G. How and when to suspect spontaneous coronary artery dissection: novel insights from a single-centre series on prevalence and angiographic appearance . EuroIntervention 2017 ; 12 : e2236 – e2243 . Google Scholar Crossref Search ADS PubMed 14 Vanzetto G , Berger-Coz E , Barone-Rochette G , Chavanon O , Bouvaist H , Hacini R , Blin D , Machecourt J. Prevalence, therapeutic management and medium-term prognosis of spontaneous coronary artery dissection: results from a database of 11, 605 patients . Eur J Cardiothorac Surg 2009 ; 35 : 250 – 254 . Google Scholar Crossref Search ADS PubMed 15 Mortensen KH , Thuesen L , Kristensen IB , Christiansen EH. Spontaneous coronary artery dissection: a Western Denmark Heart Registry Study . Catheter Cardiovasc Intervent 2009 ; 74 : 710 – 717 . Google Scholar Crossref Search ADS 16 Rashid HN , Wong DT , Wijesekera H , Gutman SJ , Shanmugam VB , Gulati R , Malaipan Y , Meredith IT , Psaltis PJ. Incidence and characterisation of spontaneous coronary artery dissection as a cause of acute coronary syndrome—a single-centre Australian experience . Int J Cardiol 2016 ; 202 : 336 – 338 . Google Scholar Crossref Search ADS PubMed 17 Barber-Chamoux N , Souteyrand G , Combaret N , Ouedraogo E , Lusson JR , Motreff P. Contribution of optical coherence tomography imaging in management of iatrogenic coronary dissection . Cardiovasc Revasc Med 2016 ; 17 : 138 – 142 . Google Scholar Crossref Search ADS PubMed 18 Elkayam U , Jalnapurkar S , Barakkat MN , Khatri N , Kealey AJ , Mehra A , Roth A. Pregnancy-associated acute myocardial infarction: a review of contemporary experience in 150 cases between 2006 and 2011 . Circulation 2014 ; 129 : 1695 – 1702 . Google Scholar Crossref Search ADS PubMed 19 Saw J , Humphries K , Aymong E , Sedlak T , Prakash R , Starovoytov A , Mancini GBJ. Spontaneous coronary artery dissection: clinical outcomes and risk of recurrence . J Am Coll Cardiol 2017 ; 70 : 1148 – 1158 . Google Scholar Crossref Search ADS PubMed 20 Basso C , Aguilera B , Banner J , Cohle S , d'Amati G , de Gouveia RH , di Gioia C , Fabre A , Gallagher PJ , Leone O , Lucena J , Mitrofanova L , Molina P , Parsons S , Rizzo S , Sheppard MN , Mier MPS , Kim Suvarna S , Thiene G , van der Wal A , Vink A , Michaud K. Association for European Cardiovascular P . Guidelines for autopsy investigation of sudden cardiac death: 2017 update from the Association for European Cardiovascular Pathology . Virchows Arch 2017 ; 471 : 691 – 705 . Google Scholar Crossref Search ADS PubMed 21 Desai S , Sheppard MN. Sudden cardiac death: look closely at the coronaries for spontaneous dissection which can be missed. A study of 9 cases . Am J Forensic Med Pathol 2012 ; 33 : 26 – 29 . Google Scholar Crossref Search ADS PubMed 22 Hill SF , Sheppard MN. Non-atherosclerotic coronary artery disease associated with sudden cardiac death . Heart 2010 ; 96 : 1119 – 1125 . Google Scholar Crossref Search ADS PubMed 23 Mandal R , Brooks EG , Corliss RF. Eosinophilic coronary periarteritis with arterial dissection: the mast cell hypothesis . J Forensic Sci 2015 ; 60 : 1088 – 1092 . Google Scholar Crossref Search ADS PubMed 24 Melez İE , Arslan MN , Melez DO , Akçay A , Büyük Y , Avşar A , Kumral B , Şirin G , Karayel FA , Daş T , Dokudan YE , Şam B. Spontaneous coronary artery dissection: report of 3 cases and literature review hormonal, autoimmune, morphological factors . Am J Forensic Med Pathol 2015 ; 36 : 188 – 192 . Google Scholar Crossref Search ADS PubMed 25 Stoukas V , Dragovic LJ. Sudden deaths from eosinophilic coronary monoarteritis: a subset of spontaneous coronary artery dissection . Am J Forensic Med Pathol 2009 ; 30 : 268 – 269 . Google Scholar Crossref Search ADS PubMed 26 Basso C , Morgagni GL , Thiene G. Spontaneous coronary artery dissection: a neglected cause of acute myocardial ischaemia and sudden death . Heart 1996 ; 75 : 451 – 454 . Google Scholar Crossref Search ADS PubMed 27 Robinowitz M , Virmani R , McAllister HAJ. Spontaneous coronary artery dissection and eosinophilic inflammation: a cause and effect relationship? Am J Med 1982 ; 72 : 923 – 928 . Google Scholar Crossref Search ADS PubMed 28 Alfonso F , Bastante T. Spontaneous coronary artery dissection: novel diagnostic insights from large series of patients . Circ Cardiovasc Interv 2014 ; 7 : 638 – 641 . Google Scholar Crossref Search ADS PubMed 29 Vrints CJ. Spontaneous coronary artery dissection . Heart 2010 ; 96 : 801 – 808 . Google Scholar Crossref Search ADS PubMed 30 Alfonso F , Paulo M , Gonzalo N , Dutary J , Jimenez-Quevedo P , Lennie V , Escaned J , Banuelos C , Hernandez R , Macaya C. Diagnosis of spontaneous coronary artery dissection by optical coherence tomography . J Am Coll Cardiol 2012 ; 59 : 1073 – 1079 . Google Scholar Crossref Search ADS PubMed 31 Paulo M , Sandoval J , Lennie V , Dutary J , Medina M , Gonzalo N , Jimenez-Quevedo P , Escaned J , Banuelos C , Hernandez R , Macaya C , Alfonso F. Combined use of OCT and IVUS in spontaneous coronary artery dissection . JACC Cardiovasc Imaging 2013 ; 6 : 830 – 832 . Google Scholar Crossref Search ADS PubMed 32 Fengping Y , Jue H , Qingchun Y , Fangxing H. A case of sudden death due to spontaneous coronary artery dissection . Am J Forensic Med Pathol 2011 ; 32 : 312 – 313 . Google Scholar Crossref Search ADS PubMed 33 Kanaroglou S , Nair V , Fernandes JR. Sudden cardiac death due to coronary artery dissection as a complication of cardiac sarcoidosis . Cardiovasc Pathol 2015 ; 24 : 244 – 246 . Google Scholar Crossref Search ADS PubMed 34 Lunebourg A , Letovanec I , Eggenberger P , Lehr HA. Images in cardiovascular medicine. Sudden cardiac death due to triple vessel coronary dissection . Circulation 2008 ; 117 : 2038 – 2040 . Google Scholar Crossref Search ADS PubMed 35 Wei JP , Kay D , Fishbein MC. Spontaneous dissection of the distal obtuse marginal coronary artery: a rare cause of sudden death . Am J Forensic Med Pathol 2008 ; 29 : 199 – 201 . Google Scholar Crossref Search ADS PubMed 36 Carreon CK , Esposito MJ. Eosinophilic coronary monoarteritis . Arch Pathol Lab Med 2014 ; 138 : 979 – 981 . Google Scholar Crossref Search ADS PubMed 37 Asuncion CM , Hyun J. Dissecting intramural hematoma of the coronary artery in pregnancy and the puerperium . Obstet Gynecol 1972 ; 40 : 202 – 210 . Google Scholar PubMed 38 Conraads VM , Vorlat A , Colpaert CG , Rodrigus IE , De Paep RJ , Moulijn AC , Vrints CJ. Spontaneous dissection of three major coronary arteries subsequent to cystic medial necrosis . Chest 1999 ; 116 : 1473 – 1475 . Google Scholar Crossref Search ADS PubMed 39 Madu EC , Kosinski DJ , Wilson WR , Burket MW , Fraker TD Jr , Ansel GM. Two-vessel coronary artery dissection in the peripartum period. Case report and literature review . Angiology 1994 ; 45 : 809 – 816 . Google Scholar Crossref Search ADS PubMed 40 Makino Y , Inokuchi G , Yokota H , Hayakawa M , Yajima D , Motomura A , Chiba F , Torimitsu S , Nakatani Y , Iwase H. Sudden death due to coronary artery dissection associated with fibromuscular dysplasia revealed by postmortem selective computed tomography coronary angiography: a case report . Forensic Sci Int 2015 ; 253 : e10 – e15 . Google Scholar Crossref Search ADS PubMed 41 Mortensen KH , Thuesen L , Kristiensen IB , Christiansen EH. Spontaneous coronary artery dissection - a western Denmark heart registry study . Eur Heart J 2009 ; 30 : 787 – 787 . 42 Nishiguchi T , Tanaka A , Ozaki Y , Taruya A , Fukuda S , Taguchi H , Iwaguro T , Ueno S , Okumoto Y , Akasaka T. Prevalence of spontaneous coronary artery dissection in patients with acute coronary syndrome . Eur Heart J Acute Cardiovasc Care 2016 ; 5 : 263 – 270 . Google Scholar Crossref Search ADS PubMed 43 Saw J , Aymong E , Mancini GB , Sedlak T , Starovoytov A , Ricci D. Nonatherosclerotic coronary artery disease in young women . Can J Cardiol 2014 ; 30 : 814 – 819 . Google Scholar Crossref Search ADS PubMed 44 Tweet MS , Hayes SN , Pitta SR , Simari RD , Lerman A , Lennon RJ , Gersh BJ , Khambatta S , Best PJ , Rihal CS , Gulati R. Clinical features, management, and prognosis of spontaneous coronary artery dissection . Circulation 2012 ; 126 : 579 – 588 . Google Scholar Crossref Search ADS PubMed 45 Rogowski S , Maeder MT , Weilenmann D , Haager PK , Ammann P , Rohner F , Joerg L , Rickli H. Spontaneous coronary artery dissection: angiographic follow-up and long-term clinical outcome in a predominantly medically treated population . Catheter Cardiovasc Interv 2017 ; 89 : 59 – 68 . Google Scholar Crossref Search ADS PubMed 46 Bush N , Nelson-Piercy C , Spark P , Kurinczuk JJ , Brocklehurst P , Knight M. Myocardial infarction in pregnancy and postpartum in the UK . Eur J Prev Cardiol 2013 ; 20 : 12 – 20 . Google Scholar Crossref Search ADS PubMed 47 Eleid MF , Guddeti RR , Tweet MS , Lerman A , Singh M , Best PJ , Vrtiska TJ , Prasad M , Rihal CS , Hayes SN , Gulati R. Coronary artery tortuosity in spontaneous coronary artery dissection: angiographic characteristics and clinical implications . Circ Cardiovasc Interv 2014 ; 7 : 656 – 662 . Google Scholar Crossref Search ADS PubMed 48 Huikuri HV , Mallon SM , Myerburg RJ. Cardiac arrest due to spontaneous coronary artery dissection in a patient with coronary ectasia—a case report . Angiology 1991 ; 42 : 148 – 151 . Google Scholar Crossref Search ADS PubMed 49 Mieghem CA , Ligthart JM , Cademartiri F. Images in cardiology . Spontaneous dissection of the left main coronary artery in a patient with Osler-Weber-Rendu disease . Heart 2005 ; 92 : 394. Google Scholar Crossref Search ADS 50 Henkin S , Negrotto SM , Tweet MS , Kirmani S , Deyle DR , Gulati R , Olson TM , Hayes SN. Spontaneous coronary artery dissection and its association with heritable connective tissue disorders . Heart 2016 ; 102 : 876 – 881 . Google Scholar Crossref Search ADS PubMed 51 Sato C , Wakabayashi K , Suzuki H. Natural course of isolated spontaneous coronary artery dissection in Marfan syndrome . Int J Cardiol 2014 ; 177 : 20 – 22 . Google Scholar Crossref Search ADS PubMed 52 Fattori R , Sangiorgio P , Mariucci E , Ritelli M , Wischmeijer A , Greco C , Colombi M. Spontaneous coronary artery dissection in a young woman with Loeys-Dietz syndrome . Am J Med Genet A 2012 ; 158A : 1216 – 1218 . Google Scholar Crossref Search ADS PubMed 53 Hampole CV , Philip F , Shafii A , Pettersson G , Anesi GL , Patel JB , Menon V. Spontaneous coronary artery dissection in Ehlers-Danlos syndrome . Ann Thorac Surg 2011 ; 92 : 1883 – 1884 . Google Scholar Crossref Search ADS PubMed 54 Giugliano GR , Sethi PS. Spontaneous left anterior descending coronary artery dissection in a patient with neurofibromatosis . J Invasive Cardiol 2009 ; 21 : e103 – e105 . Google Scholar PubMed 55 Sibon I , Sommer P , Lamaziere JM , Bonnet J. Lysyl oxidase deficiency: a new cause of human arterial dissection . Heart 2005 ; 91 : e33. Google Scholar Crossref Search ADS PubMed 56 Martín Dávila F , Delgado Portela M , García Rojo M , González García J , Puig Rullán AM , López Pérez R , Carbajo Vicente M. Coronary artery dissection in alpha-1-antitrypsin deficiency . Histopathology 1999 ; 34 : 376 – 378 . Google Scholar Crossref Search ADS PubMed 57 Diez-del Hoyo F , Sanz-Ruiz R , Diez-Villanueva P , Nunez-Garcia A , Casado-Plasencia A , Angulo-Llanos R , Clavero-Olmos M , Elizaga Corrales J , Fernandez-Aviles F. A novel cardiovascular presentation of Alport Syndrome: spontaneous coronary artery dissection . Int J Cardiol 2014 ; 177 : e133 – e134 . Google Scholar Crossref Search ADS PubMed 58 Itty CT , Farshid A , Talaulikar G. Spontaneous coronary artery dissection in a woman with polycystic kidney disease . Am J Kidney Dis 2009 ; 53 : 518 – 521 . Google Scholar Crossref Search ADS PubMed 59 Marcoff L , Rahman E. Menstruation-associated spontaneous coronary artery dissection . J Invasive Cardiol 2010 ; 22 : E183 – E185 . Google Scholar PubMed 60 Iltumur K , Karahan Z , Ozmen S , Danis R , Toprak N. Spontaneous coronary artery dissection during hemodialysis in the post-abortion period . Int J Cardiol 2008 ; 127 : e45 – e47 . Google Scholar Crossref Search ADS PubMed 61 Luceri S , Paolillo V,D , Benedictis M , Scrocca I. Spontaneous dissection of the left coronary tree after an interruption of pregnancy treated with extensive stenting . J Invasive Cardiol 2006 ; 18 : E117 – E120 . Google Scholar PubMed 62 Azam MN , Roberts DH , Logan WF. Spontaneous coronary artery dissection associated with oral contraceptive use . Int J Cardiol 1995 ; 48 : 195 – 198 . Google Scholar Crossref Search ADS PubMed 63 Zehir R , Karabay CY , Kocabay G. Myocardial infarction and spontaneous dissection of coronary artery due to oral contraceptive . J Cardiovasc Med (Hagerstown) 2011 ; 12 : 448 – 450 . Google Scholar Crossref Search ADS PubMed 64 Pan AL , Fergusson D , Hong R , Badawi RA. Spontaneous coronary artery dissection following topical hormone replacement therapy . Case Rep Cardiol 2012 ; 2012 : 1. Google Scholar Crossref Search ADS 65 Abuzeyad FH , Ibnaouf ES , Farras MA. Clomiphene associated inferior STEMI in a young female due to right coronary artery Dissection . Case Rep Emerg Med 2017 ; 2017 : 1. Google Scholar Crossref Search ADS 66 Lempereur M , Grewal J , Saw J. Spontaneous coronary artery dissection associated with beta-HCG injections and fibromuscular dysplasia . Can J Cardiol 2014 ; 30 : 464 e1 – 463 . Google Scholar Crossref Search ADS 67 Mirra M , Kola N , Mattiello G , Morisco C , Spinelli L. Spontaneous coronary artery dissection in a young woman with polycystic ovarian syndrome . Am J Emerg Med 2017 ; 35 : 936 e5 – 936 e7 . Google Scholar Crossref Search ADS 68 Aldoboni AH , Hamza EA , Majdi K , Ngibzadhe M , Palasaidi S , Moayed DA. Spontaneous dissection of coronary artery treated by primary stenting as the first presentation of systemic lupus erythematosus . J Invasive Cardiol 2002 ; 14 : 694 – 696 . Google Scholar PubMed 69 Srinivas M , Basumani P , Muthusamy R , Wheeldon N. Active inflammatory bowel disease and coronary artery dissection . Postgrad Med J 2005 ; 81 : 68 – 70 . Google Scholar Crossref Search ADS PubMed 70 Chu KH , Menapace FJ , Blankenship JC , Hausch R , Harrington T. Polyarteritis nodosa presenting as acute myocardial infarction with coronary dissection . Cathet Cardiovasc Diagn 1998 ; 44 : 320 – 324 . Google Scholar Crossref Search ADS PubMed 71 Hunsaker JC 3rd , O'Connor WN , Lie JT. Spontaneous coronary arterial dissection and isolated eosinophilic coronary arteritis: sudden cardiac death in a patient with a limited variant of Churg-Strauss syndrome . Mayo Clin Proc 1992 ; 67 : 761 – 766 . Google Scholar Crossref Search ADS PubMed 72 Shah AH , Kinnaird TD. Recurrent ST elevation myocardial infarction: what is the aetiology? Heart Lung Circ 2015 ; 24 : e169 – e172 . Google Scholar Crossref Search ADS PubMed 73 Saw J , Aymong E , Sedlak T , Buller CE , Starovoytov A , Ricci D , Robinson S , Vuurmans T , Gao M , Humphries K , Mancini GBJ. Spontaneous coronary artery dissection: association with predisposing arteriopathies and precipitating stressors and cardiovascular outcomes . Circulation 2014 ; 7 : 645 – 655 . Google Scholar PubMed 74 Gerede DM , Yüksel B , Tutar E , Küçükşahin O , Uzun Ç , Atasoy KÇ , Düzgün N , Bengisun U. Spontaneous coronary artery dissection in a male patient with Takayasu’s arteritis and antiphospholipid antibody syndrome . Case Rep Rheumatol 2013 ; 2013 : 1. Google Scholar Crossref Search ADS 75 Ionescu CN , Chrissoheris M , Caraccciolo EA. Spontaneous coronary artery dissection and severe hypothyroidism . J Invasive Cardiol 2009 ; 21 : E60 – E62 . Google Scholar PubMed 76 Bayar N , Çağırcı G , Üreyen ÇM , Kuş G , Küçükseymen S , Arslan Ş. The relationship between spontaneous multi-vessel coronary artery dissection and celiac disease . Korean Circ J 2015 ; 45 : 242 – 244 . Google Scholar Crossref Search ADS PubMed 77 Kay IP , Williams MJ. Spontaneous coronary artery dissection: long stenting in a patient with polycythemia vera . Int J Cardiovasc Intervent 1999 ; 2 : 191 – 193 . Google Scholar Crossref Search ADS PubMed 78 Tsujita K , Miyazaki T , Kaikita K , Chitose T , Takaoka N , Soejima H , Tayama S , Hokimoto S , Sugiyama S , Ogawa H. Premenopausal woman with acute myocardial infarction caused by spontaneous coronary artery dissection and potential association with coronary vasospasm . Cardiovasc Interv Ther 2012 ; 27 : 121 – 126 . Google Scholar Crossref Search ADS PubMed 79 Jaffe BD , Broderick TM , Leier CV. Cocaine-induced coronary-artery dissection . N Engl J Med 1994 ; 330 : 510 – 511 . Google Scholar Crossref Search ADS PubMed 80 Suh SY , Kim JW , Choi CU , Kim EJ , Rha SW , Park CG , Seo HS , Oh DJ. Spontaneous coronary dissection associated with sleep deprivation presenting with acute myocardial infarction . Int J Cardiol 2007 ; 115 : e78 – e79 . Google Scholar Crossref Search ADS PubMed 81 Velusamy M , Fisherkeller M , Keenan ME , Kiernan FJ , Fram DB. Spontaneous coronary artery dissection in a young woman precipitated by retching . J Invasive Cardiol 2002 ; 14 : 198 – 201 . Google Scholar PubMed 82 Schifferdecker B , Pacifico L , Ramsaran EK , Folland ED , Spodick DH , Weiner BH. Spontaneous coronary artery dissection associated with sexual intercourse . Am J Cardiol 2004 ; 93 : 1323 – 1324 . Google Scholar Crossref Search ADS PubMed 83 Tsimikas S , Giordano FJ , Tarazi RY , Beyer RW. Spontaneous coronary artery dissection in patients with renal transplantation . J Invasive Cardiol 1999 ; 11 : 316 – 321 . Google Scholar PubMed 84 Mallon DH , McKenzie D , Dayer M. A spontaneous coronary arterial dissection associated with a calcineurin inhibitor . BMJ Case Rep 2012 ; 2012 : bcr2012006414. Google Scholar Crossref Search ADS PubMed 85 Abbott JD , Curtis JP , Murad K , Kramer HM , Remetz MS , Setaro JF , Brennan JJ. Spontaneous coronary artery dissection in a woman receiving 5-fluorouracil—a case report . Angiology 2003 ; 54 : 721 – 724 . Google Scholar Crossref Search ADS PubMed 86 Goli AK , Koduri M , Haddadin T , Henry PD. Spontaneous coronary artery dissection in a woman on fenfluramine . Rev Cardiovasc Med 2007 ; 8 : 41 – 44 . Google Scholar PubMed 87 Keir ML , Dehghani P. Corticosteroids and spontaneous coronary artery dissection: a new predisposing factor? Can J Cardiol 2016 ; 32 : 395 e7 – 398 . Google Scholar Crossref Search ADS 88 Mahmood MM , Wright RA. Spontaneous coronary artery dissection in a patient on methylphenidate for attention deficit hyperactivity disorder . Int J Cardiol 2016 ; 222 : 830 – 831 . Google Scholar Crossref Search ADS PubMed 89 Garcia Garcia C , Casanovas N , Recasens L , Miranda F , Bruguera J. Spontaneous coronary artery dissection in ergotamine abuse . Int J Cardiol 2007 ; 118 : 410 – 411 . Google Scholar Crossref Search ADS PubMed 90 Saunders SL , Ford SE. Primary coronary artery dissection possibly related to drug hypersensitivity in a male . Can J Cardiol 1991 ; 7 : 138 – 140 . Google Scholar PubMed 91 Karabinos I , Papadopoulos A , Koulouris S , Kranidis A , Korovesis S , Katritsis D. Spontaneous coronary artery dissection during a dobutamine stress echocardiography . Echocardiography 2006 ; 23 : 232 – 234 . Google Scholar Crossref Search ADS PubMed 92 Fahmy P , Prakash R , Starovoytov A , Boone R , Saw J. Pre-disposing and precipitating factors in men with spontaneous coronary artery dissection . JACC Cardiovasc Interv 2016 ; 9 : 866 – 868 . Google Scholar Crossref Search ADS PubMed 93 Vijayaraghavan R , Verma S , Gupta N , Saw J. Pregnancy-related spontaneous coronary artery dissection . Circulation 2014 ; 130 : 1915 – 1920 . Google Scholar Crossref Search ADS PubMed 94 Cade JR , Szarf G , de Siqueira ME , Chaves A , Andrea JC , Figueira HR , Gomes MM Jr , Freitas BP , Filgueiras Medeiros J , Dos Santos MR , Fiorotto WB , Daige A , Goncalves R , Cantarelli M , Alves CM , Echenique L , de Brito FS Jr , Perin MA , Born D , Hecht H , Caixeta A. Pregnancy-associated spontaneous coronary artery dissection: insights from a case series of 13 patients . Eur Heart J Cardiovasc Imaging 2017 ; 18 : 54 – 61 . Google Scholar Crossref Search ADS PubMed 95 Tweet MS , Hayes SN , Codsi E , Gulati R , Rose CH , Best PJM. Spontaneous coronary artery dissection associated with pregnancy . J Am Coll Cardiol 2017 ; 70 : 426 – 435 . Google Scholar Crossref Search ADS PubMed 96 Codsi E , Tweet MS , Rose CH , Arendt KW , Best PJ , Hayes SN. Spontaneous coronary artery dissection in pregnancy: what every obstetrician should know . Obstet Gynecol 2016 ; 128 : 731 – 738 . Google Scholar Crossref Search ADS PubMed 97 Appleby CE , Barolet A , Ing D , Ross J , Schwartz L , Seidelin P , Silversides C , Horlick E. Contemporary management of pregnancy-related coronary artery dissection: a single-centre experience and literature review . Exp Clin Cardiol 2009 ; 14 : e8 – e16 . Google Scholar PubMed 98 Koul AK , Hollander G , Moskovits N , Frankel R , Herrera L , Shani J. Coronary artery dissection during pregnancy and the postpartum period: two case reports and review of literature . Catheter Cardiovasc Interv 2001 ; 52 : 88 – 94 . Google Scholar Crossref Search ADS PubMed 99 Havakuk O , Goland S , Mehra A , Elkayam U. Pregnancy and the risk of spontaneous coronary artery dissection: an analysis of 120 contemporary cases . Circ Cardiovasc Interv 2017 ; 10 : e004941. Google Scholar Crossref Search ADS PubMed 100 Olin JW , Gornik HL , Bacharach JM , Biller J , Fine LJ , Gray BH , Gray WA , Gupta R , Hamburg NM , Katzen BT , Lookstein RA , Lumsden AB , Newburger JW , Rundek T , Sperati CJ , Stanley JC ; American Heart Association Council on Peripheral Vascular Disease, American Heart Association Council on Clinical Cardiology, American Heart Association Council on Cardiopulmonary Critical Care, Perioperative and Resuscitation, American Heart Association Council on Cardiovascular Disease in the Young, American Heart Association Council on Cardiovascular Radiology and Intervention, American Heart Association Council on Epidemiology and Prevention, American Heart Association Council on Functional Genomics and Translational Biology, American Heart Association Council for High Blood Pressure Research, American Heart Association Council on the Kidney in Cardiovascular Disease, American Heart Association Stroke Council. Fibromuscular dysplasia: state of the science and critical unanswered questions: a scientific statement from the American Heart Association . Circulation 2014 ; 129 : 1048 – 1078 . Google Scholar Crossref Search ADS PubMed 101 Persu A , Giavarini A , Touze E , Januszewicz A , Sapoval M , Azizi M , Barral X , Jeunemaitre X , Morganti A , Plouin PF , de Leeuw P ESH Working Group Hypertension and the Kidney . European consensus on the diagnosis and management of fibromuscular dysplasia . J Hypertens 2014 ; 32 : 1367 – 1378 . Google Scholar Crossref Search ADS PubMed 102 Savard S , Steichen O , Azarine A , Azizi M , Jeunemaitre X , Plouin PF. Association between 2 angiographic subtypes of renal artery fibromuscular dysplasia and clinical characteristics . Circulation 2012 ; 126 : 3062 – 3069 . Google Scholar Crossref Search ADS PubMed 103 Persu A , Van der Niepen P , Touze E , Gevaert S , Berra E , Mace P , Plouin PF , Jeunemaitre X. Revisiting fibromuscular dysplasia: rationale of the European fibromuscular dysplasia initiative . Hypertension 2016 ; 68 : 832 – 839 . Google Scholar Crossref Search ADS PubMed 104 Saw J , Poulter R , Fung A , Wood D , Hamburger J , Buller CE. Spontaneous coronary artery dissection in patients with fibromuscular dysplasia: a case series . Circ Cardiovasc Interv 2012 ; 5 : 134 – 137 . Google Scholar Crossref Search ADS PubMed 105 Toggweiler S , Puck M , Thalhammer C , Manka R , Wyss M , Bilecen D , Corti R , Amann-Vesti BR , Lüscher TF , Wyss CA. Associated vascular lesions in patients with spontaneous coronary artery dissection . Swiss Med Wkly 2012 ; 142 : w13538. Google Scholar PubMed 106 Liang JJ , Prasad M , Tweet MS , Hayes SN , Gulati R , Breen JF , Leng S , Vrtiska TJ. A novel application of CT angiography to detect extracoronary vascular abnormalities in patients with spontaneous coronary artery dissection . J Cardiovasc Comput Tomogr 2014 ; 8 : 189 – 197 . Google Scholar Crossref Search ADS PubMed 107 Prasad M , Tweet MS , Hayes SN , Leng S , Liang JJ , Eleid MF , Gulati R , Vrtiska TJ. Prevalence of extracoronary vascular abnormalities and fibromuscular dysplasia in patients with spontaneous coronary artery dissection . Am J Cardiol 2015 ; 115 : 1672 – 1677 . Google Scholar Crossref Search ADS PubMed 108 Saw J , Ricci D , Starovoytov A , Fox R , Buller CE. Spontaneous coronary artery dissection: prevalence of predisposing conditions including fibromuscular dysplasia in a tertiary center cohort . JACC Cardiovasc Interv 2013 ; 6 : 44 – 52 . Google Scholar Crossref Search ADS PubMed 109 Lie JT , Berg KK. Isolated fibromuscular dysplasia of the coronary arteries with spontaneous dissection and myocardial infarction . Hum Pathol 1987 ; 18 : 654 – 656 . Google Scholar Crossref Search ADS PubMed 110 Brodsky SV , Ramaswamy G , Chander P , Braun A. Ruptured cerebral aneurysm and acute coronary artery dissection in the setting of multivascular fibromuscular dysplasia: a case report . Angiology 2007 ; 58 : 764 – 767 . Google Scholar Crossref Search ADS PubMed 111 Mather PJ , Hansen CL , Goldman B , Inniss S , Pina I , Norris R , Jeevanandam V , Bove AA. Postpartum multivessel coronary dissection . J Heart Lung Transplant 1994 ; 13 : 533 – 537 . Google Scholar PubMed 112 Camuglia A , Manins V , Taylor A , Hengel C. Case report and review: epicardial coronary artery fibromuscular dysplasia . Heart Lung Circ 2009 ; 18 : 151 – 154 . Google Scholar Crossref Search ADS PubMed 113 Pate GE , Lowe R , Buller CE. Fibromuscular dysplasia of the coronary and renal arteries? Catheter Cardiovasc Interv 2005 ; 64 : 138 – 145 . Google Scholar Crossref Search ADS PubMed 114 Michelis KC , Olin JW , Kadian-Dodov D , d'Escamard V , Kovacic JC. Coronary artery manifestations of fibromuscular dysplasia . J Am Coll Cardiol 2014 ; 64 : 1033 – 1046 . Google Scholar Crossref Search ADS PubMed 115 Saw J , Bezerra H , Gornik HL , Machan L , Mancini GB. Angiographic and intracoronary manifestations of coronary fibromuscular dysplasia . Circulation 2016 ; 133 : 1548 – 1559 . Google Scholar Crossref Search ADS PubMed 116 Kadian-Dodov D , Gornik HL , Gu X , Froehlich J , Bacharach JM , Chi YW , Gray BH , Jaff MR , Kim ES , Mace P , Sharma A , Kline-Rogers E , White C , Olin JW. Dissection and aneurysm in patients with fibromuscular dysplasia: findings from the U.S. Registry for FMD . J Am Coll Cardiol 2016 ; 68 : 176 – 185 . Google Scholar Crossref Search ADS PubMed 117 Buccheri D , Piraino D , Andolina G. Behcet disease and spontaneous coronary artery dissection: the chicken or the egg? Int J Cardiol 2016 ; 215 : 504 – 505 . Google Scholar Crossref Search ADS PubMed 118 Reddy S , Vaid T , Ganiga Sanjeeva NC , Shetty RK. Spontaneous coronary artery dissection as the first presentation of systemic lupus erythematosus . BMJ Case Rep 2016 ;doi:10.1136/bcr-2016-216344. 119 Rekik S , Lanfranchi P , Jacq L , Bernasconi F. Spontaneous coronary artery dissection in a 35 year-old woman with systemic lupus erythematosus successfully treated by angioplasty . Heart Lung Circ 2013 ; 22 : 955 – 958 . Google Scholar Crossref Search ADS PubMed 120 Nisar MK , Mya T. Spontaneous coronary artery dissection in the context of positive anticardiolipin antibodies and clinically undiagnosed systemic lupus erythematosus . Lupus 2011 ; 20 : 1436 – 1438 . Google Scholar Crossref Search ADS PubMed 121 Kothari D , Ruygrok P , Gentles T , Occleshaw C. Spontaneous coronary artery dissection in an adolescent man with systemic lupus erythematosus . Intern Med J 2007 ; 37 : 342 – 343 . Google Scholar Crossref Search ADS PubMed 122 Sharma AK , Farb A , Maniar P , Ajani AE , Castagna M , Virmani R , Suddath W , Lindsay J. Spontaneous coronary artery dissection in a patient with systemic lupus erythematosis . Hawaii Med J 2003 ; 62 : 248 – 253 . Google Scholar PubMed 123 Canpolat U , Dural M , Atalar E. Acute inferior myocardial infarction in a young female patient with polyarteritis nodosa . Herz 2012 ; 37 : 461 – 463 . Google Scholar Crossref Search ADS PubMed 124 Fernández-Gutiérrez B , Zamorano J , Batlle E , Alfonso F , Conde A , Sánchez-Harguindey L , Jover JA. Coronary dissection associated with hepatitis C virus-related cryoglobulinaemia . Rheumatology (Oxford) 1999 ; 38 : 1299 – 1301 . Google Scholar Crossref Search ADS PubMed 125 Iyisoy A , Agac MT , Celik T , Jata B. Spontaneous dissection of left main coronary artery associated with hypertensive crisis: a probable fatal complication detected by intravascular ultrasound . Int J Cardiol 2010 ; 139 : e5 – e7 . Google Scholar Crossref Search ADS PubMed 126 Greenblatt JM , Kochar GS , Albornoz MA. Multivessel spontaneous coronary artery dissection in a patient with severe systolic hypertension: a possible association. A case report . Angiology 1999 ; 50 : 509 – 513 . Google Scholar Crossref Search ADS PubMed 127 Sivam S , Yozghatlian V , Dentice R , McGrady M , Moriarty C , Di Michiel J , Bye PT , Rees D. Spontaneous coronary artery dissection associated with coughing . J Cyst Fibros 2014 ; 13 : 235 – 237 . Google Scholar Crossref Search ADS PubMed 128 Afzal AM , Sarmast SA , Weber NA , Schussler JM. Spontaneous coronary artery dissection in a 22-year-old man on lisdexamfetamine . Proc (Bayl Univ Med Cent) 2015 ; 28 : 367 – 368 . Google Scholar Crossref Search ADS PubMed 129 Ijsselmuiden A , Verheye S. Cocaine-induced coronary artery dissection . JACC Cardiovasc Interv 2009 ; 2 : 1031. Google Scholar Crossref Search ADS PubMed 130 Kanwar M , Gill N. Spontaneous multivessel coronary artery dissection . J Invasive Cardiol 2010 ; 22 : E5 – E6 . Google Scholar PubMed 131 Katikaneni PK , Akkus NI , Tandon N , Modi K. Cocaine-induced postpartum coronary artery dissection: a case report and 80-year review of literature . J Invasive Cardiol 2013 ; 25 : E163 – E166 . Google Scholar PubMed 132 Wickremaarachchi C , Olinga J , Ooi SY , Cranney G. Complete angiographic resolution of cocaine induced coronary artery dissection within eight days without coronary stenting—a case report . Heart Lung Circ 2016 ; 25 : e24 – e28 . Google Scholar Crossref Search ADS PubMed 133 Reriani M , Sara JD , Flammer AJ , Gulati R , Li J , Rihal C , Lennon R , Lerman LO , Lerman A. Coronary endothelial function testing provides superior discrimination compared with standard clinical risk scoring in prediction of cardiovascular events . Coron Artery Dis 2016 ; 27 : 213 – 220 . Google Scholar Crossref Search ADS PubMed 134 Balakrishnan K , Scott P , Oliver L. A confluence of circumstances: a case of IVF, extreme exercise and spontaneous coronary artery dissection . Int J Cardiol 2016 ; 203 : 76 – 77 . Google Scholar Crossref Search ADS PubMed 135 El-Sherief K , Rashidian A , Srikanth S. Spontaneous coronary artery dissection after intense weightlifting UCSF Fresno Department of Cardiology . Catheter Cardiovasc Interv 2011 ; 78 : 223 – 227 . Google Scholar Crossref Search ADS PubMed 136 Ellis CJ , Haywood GA , Monro JL. Spontaneous coronary artery dissection in a young woman resulting from an intense gymnasium “work-out”. Int J Cardiol 1994 ; 47 : 193 – 194 . Google Scholar Crossref Search ADS PubMed 137 Choi JW , Davidson CJ. Spontaneous multivessel coronary artery dissection in a long-distance runner successfully treated with oral antiplatelet therapy . J Invasive Cardiol 2002 ; 14 : 675 – 678 . Google Scholar PubMed 138 Aghasadeghi K , Aslani A. Spontaneous coronary artery dissection in a professional body builder . Int J Cardiol 2008 ; 130 : e119 – e120 . Google Scholar Crossref Search ADS PubMed 139 Adlam D , Cuculi F , Lim C , Banning A. Management of spontaneous coronary artery dissection in the primary percutaneous coronary intervention era . J Invasive Cardiol 2010 ; 22 : 549 – 553 . Google Scholar PubMed 140 Basile C , Lucarelli K , Langialonga T. Spontaneous coronary artery dissection: one more extrarenal manifestation of autosomal dominant polycystic kidney disease? J Nephrol 2009 ; 22 : 414 – 416 . Google Scholar PubMed 141 Grover P , Fitzgibbons TP. Spontaneous coronary artery dissection in a patient with autosomal dominant polycystic kidney disease: a case report . J Med Case Rep 2016 ; 10 : 62. Google Scholar Crossref Search ADS PubMed 142 Klingenberg-Salachova F , Limburg S , Boereboom F. Spontaneous coronary artery dissection in polycystic kidney disease . Clin Kidney J 2012 ; 5 : 44 – 46 . Google Scholar Crossref Search ADS PubMed 143 Nakamura M , Yajima J , Oikawa Y , Ogasawara K , Uejima T , Abe K , Aizawa T. Vascular Ehlers-Danlos syndrome–all three coronary artery spontaneous dissections . J Cardiol 2009 ; 53 : 458 – 462 . Google Scholar Crossref Search ADS PubMed 144 Goel K , Tweet M , Olson TM , Maleszewski JJ , Gulati R , Hayes SN. Familial spontaneous coronary artery dissection: evidence for genetic susceptibility . JAMA Intern Med 2015 ; 175 : 821 – 826 . Google Scholar Crossref Search ADS PubMed 145 Al-Hussaini A , Adlam D. Spontaneous coronary artery dissection . Heart 2017 ; 146 Luong C , Starovoytov A , Heydari M , Sedlak T , Aymong E , Saw J. Clinical presentation of patients with spontaneous coronary artery dissection . Catheter Cardiovasc Interv 2017 ; 147 Tweet MS , Gulati R , Williamson EE , Vrtiska TJ , Hayes SN. Multimodality imaging for spontaneous coronary artery dissection in women . JACC Cardiovasc Imaging 2016 ; 9 : 436 – 450 . Google Scholar Crossref Search ADS PubMed 148 Rogers JH , Lasala JM. Coronary artery dissection and perforation complicating percutaneous coronary intervention . J Invasive Cardiol 2004 ; 16 : 493 – 499 . Google Scholar PubMed 149 Saw J. Coronary angiogram classification of spontaneous coronary artery dissection . Catheter Cardiovasc Interv 2014 ; 84 : 1115 – 1122 . Google Scholar Crossref Search ADS PubMed 150 Saw J , Mancini GB , Humphries K , Fung A , Boone R , Starovoytov A , Aymong E. Angiographic appearance of spontaneous coronary artery dissection with intramural hematoma proven on intracoronary imaging . Catheter Cardiovasc Interv 2016 ; 87 : E54 – E61 . Google Scholar Crossref Search ADS PubMed 151 De-Giorgio F , Grassi VM , Abbate A , d'Aloja E , Arena V. Causation or coincidence? A case of sudden death due to spontaneous coronary artery dissection in presence of myocardial bridging . Int J Cardiol 2012 ; 159 : e32 – e34 . Google Scholar Crossref Search ADS PubMed 152 Burke AP , Kutys R , Fowler D , Virmani R. Multiple spontaneous coronary artery dissections in association with anomalous origin of right coronary and intramural coronary artery dysplasia . Cardiovasc Pathol 2004 ; 13 : 173 – 175 . Google Scholar Crossref Search ADS PubMed 153 Degrauwe S , Zuffi A , Muller O , Schiele F , Eeckhout E , Iglesias JF. Very late multiple recurrent spontaneous coronary artery dissection in a young woman with recidivating acute myocardial infarction . Int J Cardiol 2016 ; 223 : 168 – 170 . Google Scholar Crossref Search ADS PubMed 154 Motreff P , Souteyrand G , Dauphin C , Eschalier R , Cassagnes J , Lusson JR. Management of spontaneous coronary artery dissection: review of the literature and discussion based on a series of 12 young women with acute coronary syndrome . Cardiology 2010 ; 115 : 10 – 18 . Google Scholar Crossref Search ADS PubMed 155 Sanchez-Recalde A , Guzman G , Armada E , Moreno R. Multiple spontaneous coronary artery dissection associated with a left main coronary artery lesion treated by stenting. Late multiple stent fractures detected by multislice CT . Rev Esp Cardiol 2009 ; 62 : 225 – 226 . Google Scholar Crossref Search ADS PubMed 156 Sharma AM , Herrera B , Aronow HD. Simultaneous spontaneous coronary and vertebral artery dissection in a postpartum woman . J Invasive Cardiol 2010 ; 22 : E229 – E232 . Google Scholar PubMed 157 Prakash R , Starovoytov A , Heydari M , Mancini GB , Saw J. Catheter-induced iatrogenic coronary artery dissection in patients with spontaneous coronary artery dissection . JACC Cardiovasc Interv 2016 ; 9 : 1851 – 1853 . Google Scholar Crossref Search ADS PubMed 158 Arnold JR , West NE , van Gaal WJ , Karamitsos TD , Banning AP. The role of intravascular ultrasound in the management of spontaneous coronary artery dissection . Cardiovasc Ultrasound 2008 ; 6 : 24. Google Scholar Crossref Search ADS PubMed 159 Poon K , Bell B , Raffel OC , Walters DL , Jang IK. Spontaneous coronary artery dissection: utility of intravascular ultrasound and optical coherence tomography during percutaneous coronary intervention . Circ Cardiovasc Interv 2011 ; 4 : e5 – e7 . Google Scholar Crossref Search ADS PubMed 160 Lempereur M , Fung A , Saw J. Stent mal-apposition with resorption of intramural hematoma with spontaneous coronary artery dissection . Cardiovasc Diagn Ther 2015 ; 5 : 323 – 329 . Google Scholar PubMed 161 Haraki T , Uemura R , Masuda S , Lee T. Progressed multivessel spontaneous coronary artery dissection that naturally healed in a male patient with non-st segment elevation myocardial infarction . Case Rep Cardiol 2016 ; 2016 : 4109496. Google Scholar PubMed 162 Auer J , Punzengruber C , Berent R , Weber T , Lamm G , Hartl P , Eber B. Spontaneous coronary artery dissection involving the left main stem: assessment by intravascular ultrasound . Heart 2004 ; 90 : e39. Google Scholar Crossref Search ADS PubMed 163 Danzi GB , Sesana M , Capuano C , Baglini R. Intravascular ultrasound assessment of a spontaneous coronary artery dissection causing acute myocardial infarction . Ital Heart J 2004 ; 5 : 796 – 797 . Google Scholar PubMed 164 Kearney P , Erbel R , Ge J , Zamorano J , Koch L , Gorge G , Meyer J. Assessment of spontaneous coronary artery dissection by intravascular ultrasound in a patient with unstable angina . Cathet Cardiovasc Diagn 1994 ; 32 : 58 – 61 . Google Scholar Crossref Search ADS PubMed 165 Maehara A , Mintz GS , Castagna MT , Pichard AD , Satler LF , Waksman R , Suddath WO , Kent KM , Weissman NJ. Intravascular ultrasound assessment of spontaneous coronary artery dissection . Am J Cardiol 2002 ; 89 : 466 – 468 . Google Scholar Crossref Search ADS PubMed 166 Alfonso F , Canales E , Aleong G. Spontaneous coronary artery dissection: diagnosis by optical coherence tomography . Eur Heart J 2009 ; 30 : 385. Google Scholar Crossref Search ADS PubMed 167 Satogami K , Ino Y , Kubo T , Shiono Y , Nishiguchi T , Matsuo Y , Orii M , Yamano T , Yamaguchi T , Hirata K , Tanaka A , Akasaka T. Successful stenting with optical frequency domain imaging guidance for spontaneous coronary artery dissection . JACC Cardiovasc Interv 2015 ; 8 : e83 – e85 . Google Scholar Crossref Search ADS PubMed 168 Bento AP , Fernandes RG , Neves DC , Patricio LM , de Aguiar JE. Spontaneous coronary dissection: “Live Flash” optical coherence tomography guided angioplasty . Case Rep Cardiol 2016 ; 2016 : 5643819. Google Scholar PubMed 169 Mori H , Kutys R , Romero M , Virmani R , Finn AV. Stenting of spontaneous coronary artery dissection from a pathological point of view . Circ Cardiovasc Interv 2016 ; 9 : e004549. Google Scholar Crossref Search ADS PubMed 170 Combaret N , Souteyrand G , Amonchot A , Coupez E , Motreff P. Contribution of guidance by optical coherence tomography (OCT) in rescue management of spontaneous coronary artery dissection . Eur Heart J Cardiovasc Imaging 2013 ; 14 : 714. Google Scholar Crossref Search ADS PubMed 171 Cade J , Mintz GS , Silva RMF , Caixeta A. Spontaneous coronary artery dissection and healing documented by optical coherence tomography . Einstein (Sao Paulo) 2016 ; 14 : 435 – 436 . Google Scholar Crossref Search ADS PubMed 172 Kwon TG , Gulati R , Matsuzawa Y , Aoki T , Guddeti RR , Herrmann J , Lennon RJ , Ritman EL , Lerman LO , Lerman A. Proliferation of coronary adventitial vasa vasorum in patients with spontaneous coronary artery dissection . JACC Cardiovasc Imaging 2016 ; 9 : 891 – 892 . Google Scholar Crossref Search ADS PubMed 173 Eleid MF , Tweet MS , Young PM , Williamson E , Hayes SN , Gulati R. Spontaneous coronary artery dissection: challenges of coronary computed tomography angiography . Eur Heart J Acute Cardiovasc Care 2017 ;2048872616687098. 174 Torres-Ayala SC , Maldonado J , Bolton JS , Bhalla S. Coronary computed tomography angiography of spontaneous coronary artery dissection: a case report and review of the literature . Am J Case Rep 2015 ; 16 : 130 – 135 . Google Scholar Crossref Search ADS PubMed 175 Alzand BS , Vanneste L , Fonck D , Van Mieghem C. Spontaneous coronary artery dissection undissolved using cardiac computed tomography . Int J Cardiol 2016 ; 222 : 1040 – 1041 . Google Scholar Crossref Search ADS PubMed 176 Conrotto F , D'Ascenzo F , Cerrato E , Fernandez-Ortiz A , Gonzalo N , Macaya F , Tamburino C , Barbanti M , van Lavieren M , Piek JJ , Applegate RJ , Latib A , Spinnler MT , Marzullo R , Iannaccone M , Pavani M , Crimi G , Fattori R , Chinaglia A , Presbitero P , Varbella F , Gaita F , Escaned J. Safety and efficacy of drug eluting stents in patients with spontaneous coronary artery dissection . Int J Cardiol 2017 ; 238 : 105 – 109 . Google Scholar Crossref Search ADS PubMed 177 Martins JL , Afreixo V , Santos L , Costa M , Santos J , Goncalves L. Medical treatment or revascularisation as the best approach for spontaneous coronary artery dissection: a systematic review and meta-analysis . Eur Heart J Acute Cardiovasc Care 2017 ;2048872617706502. 178 Arrivi A , Milici C , Bock C , Placanica A , Boschetti E , Dominici M. Idiopathic, serial coronary vessels dissection in a young woman with psychological stress: a case report and review of the literature . Case Rep Vasc Med 2012 ; 2012 : 1. Google Scholar Crossref Search ADS 179 Dashwood AM , Saw J , Dhillon P , Murdoch D. Use of a three-stent technique for a case of spontaneous coronary artery dissection . Can J Cardiol 2017 ; 33 : 830.e13 – 830.e15 . Google Scholar Crossref Search ADS 180 Walsh SJ , Jokhi PP , Saw J. Successful percutaneous management of coronary dissection and extensive intramural haematoma associated with ST elevation MI . Acute Card Care 2008 ; 10 : 231 – 233 . Google Scholar Crossref Search ADS PubMed 181 Alfonso F , Bastante T , Garcia-Guimaraes M , Pozo E , Cuesta J , Rivero F , Benedicto A , Antuna P , Alvarado T , Gulati R , Saw J. Spontaneous coronary artery dissection: new insights into diagnosis and treatment . Coron Artery Dis 2016 ; 27 : 696 – 706 . Google Scholar Crossref Search ADS PubMed 182 Ito T , Shintani Y , Ichihashi T , Fujita H , Ohte N. Non-atherosclerotic spontaneous coronary artery dissection revascularized by intravascular ultrasonography-guided fenestration with cutting balloon angioplasty . Cardiovasc Interv Ther 2017 ; 32 : 241 – 243 . Google Scholar Crossref Search ADS PubMed 183 Alkhouli M , Cole M , Ling FS. Coronary artery fenestration prior to stenting in spontaneous coronary artery dissection . Catheter Cardiovasc Interv 2016 ; 88 : E23 – E27 . Google Scholar Crossref Search ADS PubMed 184 Motreff P , Barber-Chamoux N , Combaret N , Souteyrand G. Coronary artery fenestration guided by optical coherence tomograhy before stenting: new interventional option in rescue management of compressive spontaneous intramural hematoma . Circ Cardiovasc Interv 2015 ; 8 : e002266. Google Scholar Crossref Search ADS PubMed 185 Yumoto K , Sasaki H , Aoki H , Kato K. Successful treatment of spontaneous coronary artery dissection with cutting balloon angioplasty as evaluated with optical coherence tomography . JACC Cardiovasc Interv 2014 ; 7 : 817 – 819 . Google Scholar Crossref Search ADS PubMed 186 Watt J , Egred M , Khurana A , Bagnall AJ , Zaman AG. 1-year follow-up optical frequency domain imaging of multiple bioresorbable vascular scaffolds for the treatment of spontaneous coronary artery dissection . JACC Cardiovasc Interv 2016 ; 9 : 389 – 391 . Google Scholar Crossref Search ADS PubMed 187 Sengottuvelu G , Rajendran R. Full polymer jacketing for long-segment spontaneous coronary artery dissection using bioresorbable vascular scaffolds . JACC Cardiovasc Interv 2014 ; 7 : 820 – 821 . Google Scholar Crossref Search ADS PubMed 188 Panoulas VF , Ielasi A. Bioresorbable scaffolds and drug-eluting balloons for the management of spontaneous coronary artery dissections . J Thorac Dis 2016 ; 8 : E1328 – E1330 . Google Scholar Crossref Search ADS PubMed 189 Serruys PW , Chevalier B , Sotomi Y , Cequier A , Carrié D , Piek JJ , Van Boven AJ , Dominici M , Dudek D , McClean D , Helqvist S , Haude M , Reith S , de Sousa Almeida M , Campo G , Iñiguez A , Sabaté M , Windecker S , Onuma Y. Comparison of an everolimus-eluting bioresorbable scaffold with an everolimus-eluting metallic stent for the treatment of coronary artery stenosis (ABSORB II): a 3 year, randomised, controlled, single-blind, multicentre clinical trial . Lancet 2016 ; 388 : 2479 – 2491 . Google Scholar Crossref Search ADS PubMed 190 Wehman B , Lehr EJ , Mukherjee R , Grigore A , Griffith B , Bonatti J. Robotic totally endoscopic coronary artery bypass grafting for spontaneous coronary artery dissection . Int J Med Robot 2012 ; 8 : 166 – 168 . Google Scholar Crossref Search ADS PubMed 191 Abu-Laban RB , Migneault D , Grant MR , Dhingra V , Fung A , Cook RC , Sweet D. Extracorporeal membrane oxygenation after protracted ventricular fibrillation cardiac arrest: case report and discussion . CJEM 2015 ; 17 : 210 – 216 . Google Scholar Crossref Search ADS PubMed 192 Aliyary S , Mariani MA , Verhorst PM , Hartmann M , Stoel MG , von Birgelen C. Staged therapeutic approach in spontaneous coronary dissection . Ann Thorac Surg 2007 ; 83 : 1879 – 1881 . Google Scholar Crossref Search ADS PubMed 193 Farhat F , Sassard T , Maghiar A , Jegaden O. Primary spontaneous coronary artery dissection complicated by iatrogenous aortic dissection: from David procedure to full arterial coronary revascularization . Interact Cardiovasc Thorac Surg 2006 ; 5 : 149 – 152 . Google Scholar Crossref Search ADS PubMed 194 Jacob JC , Kiernan FJ , Patel N , Rock J , Hammond J Jr. , Wencker D , Lasala AF. SCAD: a rare case of cardiac arrest in a young female . Conn Med 2011 ; 75 : 147 – 152 . Google Scholar PubMed 195 Jorge-Pérez P , García-González MJ , Ávalos-Pinto RM , G-Cosio-Carmena MD , Renes-Carreño E , Delgado JF , Yanes-Bowden G , Ferrer-Hita JJ. Spontaneous coronary dissection and cardiogenic shock requiring mechanical circulatory support in a non-transplant center . Int J Cardiol 2016 ; 221 : 629 – 630 . Google Scholar Crossref Search ADS PubMed 196 Julia I , Tauron M , Munoz-Guijosa C. Postpartum acute coronary syndrome due to intramural hematoma and coronary artery dissection . Thorac Cardiovasc Surg 2012 ; 61 : 085 – 087 . Google Scholar Crossref Search ADS 197 Paez M , Buisan F , Herrero E. Spontaneous dissection of the left coronary artery trunk during the postpartum period treated with revascularization surgery, ventricular assistance and a successful heart transplant . Acta Anaesthesiol Scand 2007 ; 51 : 960 – 961 . Google Scholar Crossref Search ADS PubMed 198 Rahman S , Abdul-Waheed M , Helmy T , Huffman LC , Koshal V , Guitron J , Merrill WH , Lewis DF , Dunlap S , Shizukuda Y , Weintraub NL , Meyer C , Cilingiroglu M. Spontaneous left main coronary artery dissection complicated by pseudoaneurysm formation in pregnancy: role of CT coronary angiography . J Cardiothorac Surg 2009 ; 4 : 15. Google Scholar Crossref Search ADS PubMed 199 Weinberg L , Ong M , Tan CO , McDonnell NJ , Lo C , Chiam E. Spontaneous coronary artery dissection in pregnancy requiring emergency caesarean delivery followed by coronary artery bypass grafting . Anaesth Intensive Care 2013 ; 41 : 251 – 255 . Google Scholar PubMed 200 Bashir M , Mustafa H , Singh H , Bonser R. Cardiac transplantation for spontaneous coronary artery dissection . Interact Cardiovasc Thorac Surg 2013 ; 16 : 91 – 92 . Google Scholar Crossref Search ADS PubMed 201 Evans R. Post-partum spontaneous coronary artery dissection and the use of veno-arterial extra-corporeal membrane oxygenation . Nurs Crit Care 2014 ; 19 : 304 – 309 . Google Scholar Crossref Search ADS PubMed 202 Knapp KE , Weis RA , Cubillo EI , Chapital AB , Ramakrishna H. Spontaneous, postpartum coronary artery dissection and cardiogenic shock with extracorporeal membrane oxygenation assisted recovery in a 30-year-old patient . Case Rep Cardiol 2016 ; 2016 : 1. Google Scholar Crossref Search ADS 203 Martins RP , Leurent G , Corbineau H , Fouquet O , Seconda S , Baruteau AE , Moreau O , Le Breton H , Bedossa M. Coronary angiography of pregnancy-associated coronary artery dissection: a high-risk procedure . Cardiovasc Revasc Med 2010 ; 11 : 182 – 185 . Google Scholar Crossref Search ADS PubMed 204 Patane F , Boffini M , Sansone F , Campanella A , Rinaldi M. ECMO as a bridge to transplantation in biventricular dysfunction due to primary spontaneous coronary artery dissection . Transpl Int 2009 ; 22 : 500 – 502 . Google Scholar Crossref Search ADS PubMed 205 Ponikowski P , Voors AA , Anker SD , Bueno H , Cleland JG , Coats AJ , Falk V , Gonzalez-Juanatey JR , Harjola VP , Jankowska EA , Jessup M , Linde C , Nihoyannopoulos P , Parissis JT , Pieske B , Riley JP , Rosano GM , Ruilope LM , Ruschitzka F , Rutten FH , van der Meer P. Authors/Task Force M . 2016 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure: the Task Force for the diagnosis and treatment of acute and chronic heart failure of the European Society of Cardiology (ESC) Developed with the special contribution of the Heart Failure Association (HFA) of the ESC . Eur Heart J 2016 ; 37 : 2129 – 2200 . Google Scholar Crossref Search ADS PubMed 206 Roffi M , Patrono C , Collet JP , Mueller C , Valgimigli M , Andreotti F , Bax JJ , Borger MA , Brotons C , Chew DP , Gencer B , Hasenfuss G , Kjeldsen K , Lancellotti P , Landmesser U , Mehilli J , Mukherjee D , Storey RF , Windecker S , Baumgartner H , Gaemperli O , Achenbach S , Agewall S , Badimon L , Baigent C , Bueno H , Bugiardini R , Carerj S , Casselman F , Cuisset T , Erol C , Fitzsimons D , Halle M , Hamm C , Hildick-Smith D , Huber K , Iliodromitis E , James S , Lewis BS , Lip GY , Piepoli MF , Richter D , Rosemann T , Sechtem U , Steg PG , Vrints C , Luis Zamorano J. Management of Acute Coronary Syndromes in Patients Presenting without Persistent STSEotESoC . 2015 ESC Guidelines for the management of acute coronary syndromes in patients presenting without persistent ST-segment elevation: task Force for the Management of Acute Coronary Syndromes in Patients Presenting without Persistent ST-Segment Elevation of the European Society of Cardiology (ESC) . Eur Heart J 2016 ; 37 : 267 – 315 . Google Scholar Crossref Search ADS PubMed 207 Ibanez B , James S , Agewall S , Antunes MJ , Bucciarelli-Ducci C , Bueno H , Caforio ALP , Crea F , Goudevenos JA , Halvorsen S , Hindricks G , Kastrati A , Lenzen MJ , Prescott E , Roffi M , Valgimigli M , Varenhorst C , Vranckx P , Widimsky P. 2017 ESC Guidelines for the management of acute myocardial infarction in patients presenting with ST-segment elevation . Rev Esp Cardiol (Engl Ed) 2017 ; 70 : 1082 . Google Scholar Crossref Search ADS PubMed 208 Priori SG , Blomström-Lundqvist C , Mazzanti A , Blom N , Borggrefe M , Camm J , Elliott PM , Fitzsimons D , Hatala R , Hindricks G , Kirchhof P , Kjeldsen K , Kuck K-H , Hernandez-Madrid A , Nikolaou N , Norekvål TM , Spaulding C , Van Veldhuisen DJ. 2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: the Task Force for the Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death of the European Society of Cardiology (ESC). Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC) . Eur Heart J 2015 ; 36 : 2793 – 2867 . Google Scholar Crossref Search ADS PubMed 209 Jovic Z , Obradovic S , Djenic N , Mladenovic Z , Djuric P , Spasic M , Tavicovski D. Does thrombolytic therapy harm or help in ST elevation myocardial infarction (STEMI) caused by the spontaneous coronary dissection? Vojnosanit Pregl 2015 ; 72 : 536 – 540 . Google Scholar Crossref Search ADS PubMed 210 Andreou AY , Georgiou PA , Georgiou GM. Spontaneous coronary artery dissection: report of two unsuspected cases initially treated with thrombolysis . Exp Clin Cardiol 2009 ; 14 : e89 – e92 . Google Scholar PubMed 211 Shamloo BK , Chintala RS , Nasur A , Ghazvini M , Shariat P , Diggs JA , Singh SN. Spontaneous coronary artery dissection: aggressive vs. conservative therapy . J Invasive Cardiol 2010 ; 22 : 222 – 228 . Google Scholar PubMed 212 Zupan I , Noc M , Trinkaus D , Popovic M. Double vessel extension of spontaneous left main coronary artery dissection in young women treated with thrombolytics . Catheter Cardiovasc Interv 2001 ; 52 : 226 – 230 . Google Scholar Crossref Search ADS PubMed 213 Goh AC , Lundstrom RJ. Spontaneous coronary artery dissection with cardiac tamponade . Tex Heart Inst J 2015 ; 42 : 479 – 482 . Google Scholar Crossref Search ADS PubMed 214 Maas AH , Euler M , Bongers MY , Rolden HJ , Grutters JP , Ulrich L , Schenck-Gustafsson K. Practice points in gynecardiology: abnormal uterine bleeding in premenopausal women taking oral anticoagulant or antiplatelet therapy . Maturitas 2015 ; 82 : 355 – 359 . Google Scholar Crossref Search ADS PubMed 215 Tweet MS , Olin JW. Insights into spontaneous coronary artery dissection: can recurrence be prevented? J Am Coll Cardiol 2017 ; 70 : 1159 – 1161 . Google Scholar Crossref Search ADS PubMed 216 Colletti PM , Lee KH , Elkayam U. Cardiovascular imaging of the pregnant patient . AJR Am J Roentgenol 2013 ; 200 : 515 – 521 . Google Scholar Crossref Search ADS PubMed 217 Tweet MS , Hayes SN , Gulati R , Rose CH , Best PJ. Pregnancy after spontaneous coronary artery dissection: a case series . Ann Intern Med 2015 ; 162 : 598 – 600 . Google Scholar Crossref Search ADS PubMed 218 Guo LQ , Wasfy MM , Hedgire S , Kalra M , Wood M , Prabhakar AM , Ghoshhajra BB. Multimodality imaging of spontaneous coronary artery dissection: case studies of the Massachusetts General Hospital . Coron Artery Dis 2016 ; 27 : 70 – 71 . Google Scholar Crossref Search ADS PubMed 219 Bhatt DD , Kachru R , Gupta S , Kaul U. Recurrent chest pain after treatment of spontaneous coronary artery dissection: an enigma . Indian Heart J 2015 ; 67 : S18 – S20 . Google Scholar Crossref Search ADS PubMed 220 Chou AY , Prakash R , Rajala J , Birnie T , Isserow S , Taylor CM , Ignaszewski A , Chan S , Starovoytov A , Saw J. The first dedicated cardiac rehabilitation program for patients with spontaneous coronary artery dissection: description and initial results . Can J Cardiol 2016 ; 32 : 554 – 560 . Google Scholar Crossref Search ADS PubMed 221 Krittanawong C , Tweet MS , Hayes SE , Bowman MJ , Gulati R , Squires RW , Hayes SN. Usefulness of cardiac rehabilitation after spontaneous coronary artery dissection . Am J Cardiol 2016 ; 117 : 1604 – 1609 . Google Scholar Crossref Search ADS PubMed 222 Liang JJ , Tweet MS , Hayes SE , Gulati R , Hayes SN. Prevalence and predictors of depression and anxiety among survivors of myocardial infarction due to spontaneous coronary artery dissection . J Cardiopulm Rehabil Prev 2014 ; 34 : 138 – 142 . Google Scholar Crossref Search ADS PubMed Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2018. For permissions, please email: journals.permissions@oup.com. This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/open_access/funder_policies/chorus/standard_publication_model)

Journal

European Heart JournalOxford University Press

Published: Sep 21, 2018

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