Cardiol Ther (2018) 7:119–123 https://doi.org/10.1007/s40119-018-0103-4 CASE REPORT Single Coronary Artery Anomaly: A Case Report and Review of Literature . . . . Ayman Elbadawi Basarat Baig Islam Y. Elgendy Erfan Alotaki . . . Ahmed H. Mohamed Kirolos Barssoum David Fries Muhammad Khan Rami N. Khouzam Received: November 21, 2017 / Published online: February 6, 2018 The Author(s) 2018. This article is an open access publication showed normal sinus rhythm, with intermittent ABSTRACT episodes of sinus bradycardia, and non-speciﬁc T-wave changes. Trans-thoracic echocardio- Introduction: Single coronary artery is a rare gram showed normal left ventricular function anomaly, which is usually associated with other and no segmental wall-motion abnormalities. cardiac congenital abnormalities. Selective coronary angiography showed a nor- Case Report: A 56-year-old female presented mal left main coronary artery arising from left with unstable angina. The patient reported coronary cusp. The left main branched to a complaints of typical chest pain on exertion few normal left anterior descending artery and to months prior to presentation, which progressed the left circumﬂex artery; a large vessel which to become at rest. The pain was associated pal- supplied also the territory of the right coronary pitations and dizziness. Past medical history was artery (RCA) through its terminal extension. signiﬁcant for hypertension and hyperlipi- Aortography showed absence of RCA with no demia. Vital signs were stable. Physical exami- other vessels arising from the right or non- nation was non-remarkable. Electrocardiogram coronary cusps. The patient was managed con- servatively and discharged home with resolu- Enhanced content To view enhanced content for this tion of symptoms. article go to https://doi.org/10.6084/m9.ﬁgshare. Conclusions: We report a rare case of isolated single coronary artery with absent RCA. The A. Elbadawi B. Baig E. Alotaki A. H. Mohamed patient presented with unstable angina, and K. Barssoum was managed conservatively. Cardiologists Internal Medicine Department, Rochester General should be aware of this rare condition, which Hospital, Rochester, NY, USA carries a potential risk of sudden cardiac death. I. Y. Elgendy (&) Division of Cardiovascular Medicine, University of Keywords: Absent right coronary artery; Florida, Gainesville, FL, USA e-mail: email@example.com Congenital heart disease; Single coronary artery anomaly D. Fries M. Khan Sands Constellation Heart Institute, Rochester General Hospital, Rochester, NY, USA BACKGROUND R. N. Khouzam Department of Medicine, Division of Cardiology, Single coronary artery (SCA) is a rare congenital University of Tennessee Health Science Center, anomaly where only one coronary artery arises Memphis, TN, USA 120 Cardiol Ther (2018) 7:119–123 from a single coronary ostium to supply the angiography (Fig. 1a) showed a normal left entire heart [1, 2]. It is usually associated with main coronary artery arising from the left other cardiac abnormalities. SCA has variable coronary cusp, branching to a normal left presentation, ranging from mild non-speciﬁc anterior descending artery (LAD) and to a left symptoms up to sudden cardiac death . circumﬂex artery (LCX). The latter was a large vessel that also supplied the territory of the right coronary artery (RCA) through its terminal CASE REPORT extension. All vessels were free of atherosclero- sis and had normal ﬂow. Aortography (Fig. 1b) A 56-year-old woman presented to our hospital showed absence of RCA with no other vessels with unstable angina. The patient complained of arising from right or non-coronary cusps. The progressive substernal chest pain on exertion that patient was managed conservatively with started a few months prior to presentation, and emphasis on aggressive control of risk factors. then progressed to become more frequent and at Informed consent was obtained from the rest. She also had associated palpitations and patient for reporting this rare case. dizziness. Her past medical history was signiﬁcant for hypertension and hyperlipidemia. On admis- sion, vital signs showed a heart rate of 80 bpm, DISCUSSION blood pressureof130/90 mmHg, and temperature of 36.6 C. Physical examination was otherwise We report a rare case of isolated SCA with unremarkable. Electrocardiogram showed normal absent RCA, with LCX supplying the territory of sinus rhythm, with non-speciﬁc T-wave changes. the RCA. SCA is a congenital anatomic abnor- Trans-thoracic echocardiogram showed normal mality in which only one coronary artery orig- left ventricular function and no segmental wall- inates from a single coronary ostium in the motion abnormalities. Blood work showed nega- aortic sinus. It has an incidence ranging from tive cardiac troponin as well as normal complete 0.024 to 1% per various reports [1, 3, 4]. Con- blood count and metabolic proﬁle. genital cardiac structural deformities associated We decided to proceed with a coronary with SCA include pulmonary artery atresia, angiogram given her clinical presentation with tetralogy of Fallot, and patent truncus arteriosus unstable angina. Selective coronary . Incidence of RCA arising from the left Fig. 1 a Coronary angiography showing left circumﬂex artery supplying RCA region. b Aortography showing absent RCA. RCA right coronary artery Cardiol Ther (2018) 7:119–123 121 coronary vasculature ranges from 0.1 to 0.9% dilatation of the great outﬂow tracts during [5, 6], with most of the published cases report- exercise, with secondary compression of the ing RCA originating from the proximal or aberrant coronary artery along their varied middle portion of the LAD. course . Other features, like acute take-off Lipton et al.  originally proposed the angle of the anomalous vessel, slit-like oriﬁce, angiographic classiﬁcation of SCA, which was and proximal intramural course, are character- later modiﬁed by Yamanaka et al. (Table 1). istics that can predispose to anginal symptoms This classiﬁcation takes into account the origin and sudden death during exertion [5, 8]. of the ostium from the sinus of Valsalva, Basso et al.  identiﬁed 27 cases of sudden anatomical course of the vessel, and the course cardiac death in young adults with congenital of the transverse trunk. Alphabets R or L are coronary anomalies in a retrospective review. It used to identify the ostial origin of the vessel, was observed that the patients with coronary roman numerals I, II, or III are used to represent arteries originating from contralateral sinuses the anatomical distribution of the vessel, and were more likely to die during exertional activ- letters A, B, P, S, and C are used to delineate the ity. This is theorized to be secondary to com- course of the vessel with respect to the pul- pression of the anomalous vessel between the monary artery and the aorta . Per this system, aorta and the pulmonary artery during exercise our patient would be classiﬁed as LIA. because of the dilation of the latter two vessels Most patients are asymptomatic at the time [11, 12]. Taylor et al.  looked at the records of of diagnosis, and cases of SCA are usually found 242 deceased patients with isolated congenital incidentally on coronary angiography. Many coronary anomalies and found that one-third of patients might have atypical chest pain or non- the patients suffered sudden cardiac death, and speciﬁc symptoms, with absence of obstructive half of these were exercise-related deaths. coronary artery disease and negative workup for Patients younger than 30 years of age were sig- ischemia . Others may present with features niﬁcantly more likely to suffer from sudden of typical chest pain, sudden death especially cardiac death compared to older patients. during exercise, syncope, palpitations, ventric- Younger patients were also more likely to die ular tachycardia, and myocardial infarction . suddenly during physical exertion. Certain anomaly classiﬁcations usually have a Coronary angiography remains the gold benign clinical course, such as RI, LI types. standard for diagnosis and classiﬁcation. Meanwhile, the aberrant coronary artery cour- Echocardiography is useful mainly to delineate ses between the outﬂow tracts such as R/LIIB or other structural abnormalities accompanying RIII, which makes them more prone to serious the SCA. Computed tomography angiography clinical complications . The underlying offers a less-invasive imaging modality despite pathophysiology could be related to the requiring administration of contrast media. Table 1 Lipton’s classiﬁcation of single coronary artery Originating from Description Originating from right cusp left cusp RI Solitary vessel arising from either the left or right coronary cusp, following the LI course of either a normal right or left coronary artery RIIA Divided into 3 types depending on the relationship of the aberrant vessel to the LIIA great vessels. Type A courses anterior to the pulmonary trunk. Type B travels RIIIB LIIB between the aorta and pulmonary trunk. Type P travels posterior to the aorta RIIP LIIP RIII Absent left coronary artery with the left anterior descending and circumﬂex arteries arising from the common trunk originating from right coronary cusp 122 Cardiol Ther (2018) 7:119–123 Cardiovascular magnetic resonance (CMR) is a management by CABG in two symptomatic suitable alternative investigation for assessing patients with SCA complicated by stenotic coronary anatomy. Advantages extended by lesions along the vessel course . Pulmonary CMR include lack of exposure to ionizing radi- artery translocation is usually reserved for ations, no need to use iodinated or ionized patients with coronary arteries arising from the intravenous contrast media, no restrictions contralateral coronary sinus without stenotic or imposed by body habitus, simultaneous evalu- slit-like lesions along the vessel . ation of multiple parameters of cardiovascular Due to the lack of stenotic lesions, acute anatomy, perfusion and function, along with angle cut-off and slit-like lesions, our patient the ability to achieve high spatial and temporal was managed medically with risk factor control. resolutions to better assess structural abnor- malities . CONCLUSIONS Treatment options include conservative medical management, percutaneous coronary We report a rare case of SCA, a rare congenital intervention (PCI) with stent placement, and abnormality. Patients are usually asymptomatic, surgical correction. Most asymptomatic patients however, may present with typical chest pain, do not require invasive therapy and should be ventricular ﬁbrillation, or myocardial infarction. managed with strict control of risk factors. Cardiologists should be aware of this rare condi- Given the variety of anatomical presentations, a tion, which has been associated with sudden multidisciplinary approach involving cardio- cardiac death in young individuals. Management thoracic surgery and interventional cardiology usually involves a multi-disciplinary approach can be considered to determine the best course with interventionists and surgeons aiming for an of action in symptomatic patients. Presenta- individualized plan based on presentation and tions with unstable angina, similar to our case, anatomy of each case. have been previously described in cases of SCA. While some of those patients improved with medical treatment, others had obstructive coronary disease requiring intervention. ACKNOWLEDGEMENTS PCI in cases of SCA poses certain technical challenges. The abnormal origin and course in cases of SCA lead to difﬁculties in cannulation Funding. No funding or sponsorship was of the coronary ostium as well as difﬁculties in received for this study or publication of this article. providing optimal catheter support during PCI . Awareness of those variations is important Authorship. All named authors meet the for catheter-based treatment. Rudan et al.  International Committee of Medical Journal successfully performed PCI with stent place- Editors (ICMJE) criteria for authorship for this ment in a case of SCA where RCA originated as article, take responsibility for the integrity of extension of left coronary vasculature. Kafkas the work as a whole, and have given their et al.  reported successful stent placement in approval for this version to be published. an L1 type SCA. Other case reports also demonstrated successful management of similar Disclosures. Ayman Elbadawi, Basarat Baig, lesions by PCI [14, 16]. Islam Y. Elgendy, Erfan Alotaki, Ahmed H. Surgical options include osteoplasty, coro- Mohamed, Kirolos Barssoum, David Fries, nary artery bypass grafting (CABG) of the Muhammad Khan, and Rami N. Khouzam have anomalous artery, re-implantation of the no conﬂicts of interest to declare. anomalous artery to the aorta, and pulmonary artery translocation [5, 17]. Rinaldi et al.  Compliance with Ethics Guidelines. In- reported the management of eight cases of formed consent was obtained from the patient anomalous RCA plus slit ostium with ostial for reporting this rare case. widening. Others reported successful Cardiol Ther (2018) 7:119–123 123 9. Evrengul H, Ozcan EE, Turhan H, Ozturk A. Single Data Availability. The datasets during and/ coronary artery originating from the right sinus of or analyzed during the current study are avail- Valsalva and hypoplastic left anterior descending able from the corresponding author on reason- artery: an extremely rare combination of congenital able request. coronary artery anomalies. Exp Clin Cardiol. 2012;17:243. Open Access. This article is distributed 10. Basso C, Maron BJ, Corrado D, Thiene G. Clinical under the terms of the Creative Commons proﬁle of congenital coronary artery anomalies Attribution-NonCommercial 4.0 International with origin from the wrong aortic sinus leading to License (http://creativecommons.org/licenses/ sudden death in young competitive athletes. J Am Coll Cardiol. 2000;35:1493–501. by-nc/4.0/), which permits any noncommer- cial use, distribution, and reproduction in any 11. Yurtdas M, Gulen O. 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Published: Feb 6, 2018
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