Cardiology

Y-Hassan, Shams; Jernberg, Tomas

doi: 10.1159/000329349pmid: 21757900

Bromocriptine-induced coronary spasm (BICS) causing myocardial infarction has been reported. Association between BICS and Takotsubo syndrome (TS) has not been described. We report on a 37-year-old woman presenting with a clinical picture of acute coronary syndrome 1 day after initiation of treatment with bromocriptine for ablactation 3 weeks after a full-term spontaneous vaginal delivery. Coronary angiography showed diffuse narrowing of a large diagonal branch. Left ventriculography showed widespread hypokinesia extending beyond the diagonal branch supply region. There was a slight elevation of myocardial infarction biomarkers that was disproportional to the degree of left ventricular dysfunction. Follow-up coronary angiography, intravascular ultrasound and left ventriculography showed normal coronary arteries including the diagonal branch and complete normalization of the left ventricular function. Cardiac magnetic resonance examination showed no signs of late myocardial gadolinium enhancement. The clinical picture and course of the disease was consistent with TS. Consequently, we describe for the first time a case of TS triggered by myocardial ischemia caused by BICS. Furthermore, our case and sufficient supporting data from the literature demonstrate that acute coronary syndrome is an important and frequent but up till now missed trigger factor for TS.

Khan, Waqas; Bustros, Thomas; Mitre, Cristina; Feit, Alan; Salciccioli, Louis; Kassotis, John

doi: 10.1159/000329050pmid: 21757901

Sinus bradycardia and associated isorhythmic AV dissociation may be seen during dobutamine stress echocardiography. This phenomenon is usually considered a benign peri-procedural event and is thought to be secondary to the Bezold-Jarisch reflex, with profound heart rate decrease secondary to a marked increased in vagal efferent discharge. To the best of our knowledge, dobutamine stress echocardiography unmasking clinically relevant sinus node dysfunction has never been reported. We report a case of a 50-year-old man presenting with chest pain and a history of syncope, who had worsening sinus bradycardia with isorhythmic AV dissociation during dobutamine stress echocardiography. An invasive electrophysiological study revealed marked sinus node dysfunction. The patient was referred for the insertion of a permanent pacemaker for sinus node dysfunction, chronotropic incompetence and symptomatic bradycardia. Dobutamine-associated bradycardia may not always be a benign peri-procedural physiologic effect, as in this case it may unmask clinically significant sinus node dysfunction.

Hsu, Po-Chao; Su, Ho-Ming; Lin, Tsung-Hsien; Huang, Jiann-Wei; Lai, Wen-Ter; Sheu, Sheng-Hsiung

doi: 10.1159/000329516pmid: 21757902

Functional coronary artery occlusion due to type A aortic dissection has rarely been reported in the literature. We report a case of aortic dissection presenting with anterior ST elevation. Coronary angiography revealed normal findings and chest computed tomography-confirmed type A aortic dissection. Emergent surgery was performed and dissection involving the right coronary artery orifice was found. An electrocardiogram after the operation revealed resolution of anterior ST elevation. The mechanism of initial anterior but not inferior ST elevation should be related to functional obstruction of the left main coronary artery by intimal flap and reciprocal ST-T change over inferior leads.

Yan, Shao-di; Chen, Mao; Li, Qiao; Liu, Xiao-jing; Peng, Yong; Chai, Hua; Xu, Yuan-ning; Wei, Jia-fu; Huang, De-jia

doi: 10.1159/000329048pmid: 21778720

Objectives: To assess the impact of different CYP2C19*2 polymorphisms on clinical outcomes and the effects of CYP2C19*2 polymorphism on predicting clinical outcomes in association with classic risk factors in patients with acute coronary syndromes (ACS). Methods: Between July 2008 and September 2009, 497 consecutive patients with ACS who were admitted to the West China Hospital of Sichuan University were enrolled and underwent CYP2C19*2 determination. The clinical outcomes were the composite of cardiovascular death, nonfatal myocardial infarction and nonfatal stroke. Results: Baseline characteristics were balanced between noncarrier, heterozygous and homozygous groups of the CYP2C19*2 variant. The clinical endpoint occurred more frequently in the homozygous group (HR 4.86, CI 1.62–14.56, p = 0.005). After multivariable analysis, the CYP2C19*2 genetic variant was an independent predictor of cardiovascular events (HR 5.96, CI 1.77–20.03, p = 0.0039) as well as GRACE score and Killip class. The combination of CYP2C19*2 with GRACE score and Killip class increases the potential to predict adverse outcomes. Conclusions: Homozygosity (A/A) for CYP2C19*2 mutant is an independent determinant of prognosis in patients with ACS. The combination of CYP2C19*2 polymorphism with classic risk factors may be a useful tool to predict the risk of cardiovascular events.

Tfelt-Hansen, Jacob; Winkel, Bo Gregers; Grunnet, Morten; Jespersen, Thomas

doi: 10.1159/000329047pmid: 21778721

Sudden infant death syndrome (SIDS) is always a devastating and unexpected occurrence. SIDS is the leading cause of death in the first 6 months after birth in the industrialized world. Since the discovery in 1998 of long QT syndrome as an underlying substrate for SIDS, around 10–20% of SIDS cases have been proposed as being caused by genetic variants in either ion channel or ion channel-associated proteins. Until now, 10 cardiac channelopathy susceptibility genes have been found to be implicated in the pathogenesis of SIDS. Four of the genes encode cardiac ion channel α-subunits, 3 genes encode ion channel β-subunits, and 3 genes encode other channel-interacting proteins. All 10 genes have been associated with primary electrical heart diseases. SIDS may hereby be the initial symptom of rare primary electric channelopathies such as long QT, short QT and Brugada syndrome, as well as catecholaminergic polymorphic ventricular tachycardia. In this review we describe the functional role of sodium, potassium and calcium channels in propagation, depolarization and repolarization in the context of the 4 arrhythmogenic diseases reported to be associated with SIDS. Lastly, the possibility of postmortem genetic testing and potential recommendations on how to deal with family members are discussed.

Ozkan, Mehmet; Aykan, Ahmet Cagri; Gursoy, Ozan; Tuncer, Mehmet Altug; Cakal, Beytullah; Yildiz, Mustafa; Kahveci, Gokhan

doi: 10.1159/000329843pmid: 21811071

Objectives: The aim of this study was to evaluate real-time 3-dimensional transesophageal echocardiography (RT-3D TEE) in the assessment of mitral paravalvular leakage curtaining surgery with a Dacron patch. Methods: RT-3D TEE was used before and after the operation. Results: Repairing a mitral paravalvular leak with Dacron patch curtaining was a useful, simple and new technique with promising feasibility and durability. Conclusions: In selected patients in whom the mitral paravalvular defect is relatively large, and may thus not be suitable for closing with primary suture, Dacron patch curtaining may be feasible for repair guided with RT-3D TEE; both are described for the first time.

Yang, Jeong Hoon; Jang, Hye Won; Park, Sung-Ji; Park, Seung Woo

doi: 10.1159/000329839pmid: 21811072

A 57-year-old woman was referred for cardiomegaly on a chest X-ray taken during thyroxine withdrawal for radioiodine therapy after total thyroidectomy. Baseline transthoracic echocardiography showed left ventricular (LV) wall motion abnormalities and an LV apical mural thrombus. Coronary angiography revealed normal epicardial coronary arteries. Despite anticoagulation treatment, the patient developed abrupt aphasia, agraphia and acalculia. The distal branch of the inferior segmental branch on the left middle cerebral artery was not well visualized on magnetic resonance angiography. Three days later, the patient had made a near full neurological recovery. After 4 weeks of anticoagulation therapy, the apical mural thrombus and wall motion abnormalities resolved. This is the first reported case in the medical literature of transient LV systolic dysfunction with thrombus and subsequent cardioembolic stroke in a patient with short-term overt hypothyroidism.

Sibellas, Franck; Girerd, Nicolas; Bejan-Angoulvant, Theodora; Rioufol, Gilles; Finet, Gérard; Chevalier, Philippe

doi: 10.1159/000329838pmid: 21822013

Coronary artery spasm is sometimes associated with life-threatening ventricular arrhythmias. Based on intravascular ultrasound findings, it appears that coronary artery spasm promotes negative arterial remodeling, suggesting that patients with coronary artery spasm might be at higher risk of accelerated coronary atherosclerosis. We report the cases of 3 patients with ventricular arrhythmia secondary to coronary artery spasm complicated by accelerated coronary atherosclerosis. Consequently, coronary disease progression should be considered in the case of angina pectoris recurrence in patients with coronary artery spasm, especially in those with coronary artery spasm complicated by ventricular arrhythmia.

Palmisano, Brian T.; Rottman, Jeffrey N.; Wells, Quinn S.; DiSalvo, Thomas G.; Hong, Charles C.

doi: 10.1159/000329834pmid: 21822014

Most sudden cardiac deaths in young athletes are caused by previously undetected inherited cardiac diseases. Here, we report a case of a young male athlete in whom a presumptive diagnosis of hypertrophic cardiomyopathy (HCM) was made following a near sudden cardiac death. Although his imaging studies initially suggested HCM, a detailed clinical and genetic evaluation of the patient and his asymptomatic father led to the diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVD) in both. DNA sequencing revealed that each individual was heterozygous for two rare variants in the PKP2 and DSC2 genes, both of which were previously shown to be associated with ARVD and to encode desmosomal proteins, i.e. the previously reported splicing variant c2489 + 1A>G in the PKP2 gene and the novel p.I109M variant in the DSC2 gene. Imaging and electrophysiologic studies further supported a diagnosis of ARVD in the father. This case highlights the importance of detailed clinical evaluation and genetic testing of family members when dealing with sudden cardiac death or unexplained cardiomyopathies in the young.

Saccucci, P.; Banci, M.; Cozzoli, E.; Neri, A.; Magrini, A.; Bottini, E.; Gloria-Bottini, F.

doi: 10.1159/000329919pmid: 21846984

Objectives: Recently, it has been shown that PTPN22 genetic polymorphism is associated with phenotypes related to the risk of atherosclerosis. In the present note, we have searched for a possible association of PTPN22 polymorphism with coronary artery disease (CAD). Methods: One hundred and thirty-four non-diabetic subjects admitted to hospital for CAD and 174 healthy subjects (blood donors) were studied. PTPN22 genotypes were determined by DNA analysis. Statistical analyses were performed by SPSS programs. Results: In CAD patients, the proportion of carriers of the *T allele of PTPN22 is significantly higher compared to healthy controls (OR 2.66; 95% CI 1.07–6.72). Conclusions: The present observation confirms the association of PTPN22 phenotype with atherosclerosis and suggests a role of immune mechanism in the pathogenesis of CAD.