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Ann Thorac Surg 2008;85:1077-1079. doi:10.1016/j.athoracsur.2007.09.052
© 2008 The Society of Thoracic Surgeons

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Case Reports

Sudden Death Related to an Anomalous Origin of the Right Coronary Artery

Florence Boissier, MDa, Nathalie Coolen, MDb, Patrick Nataf, MDa, Didier Tchetche, MDb,*

a Department of Cardiothoracic Surgery, Hôpital Bichat-Claude Bernard, Assistance Publique—Hôpitaux de Paris and Université Denis Diderot-Paris 7, Paris, France
b Department of Cardiology, Hôpital Bichat-Claude Bernard, Assistance Publique—Hôpitaux de Paris and Université Denis Diderot-Paris 7, Paris, France

Accepted for publication September 25, 2007.

* Address correspondence to Dr Tchetche, Département de Cardiologie, Hôpital Bichat-Claude Bernard, 46 rue Henri Huchard, Paris, 75018, France (Email: didiertchetche{at}yahoo.fr).


    Abstract
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 Abstract
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Nonatherosclerotic anomalies of the coronary arteries are not rare. They account for a third of sudden cardiac deaths in young patients. We report a case of resuscitated cardiac arrest due to an abnormal origin of the right coronary artery. Subsequently, coronary bypass grafting was carried out successfully. After resuscitated sudden death, there is no consensus on the diagnosis or therapeutic management of patients with abnormal coronary arteries.


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Each year, approximately 50,000 new cases of sudden cardiac death (SCD) occur in France and 300,000 in the United States [1]. Atherosclerotic disease and cardiac arrhythmia are the most frequent causes of SCD, but nonatherosclerotic anomalies of the coronary arteries can explain a third of SCDs in young patients. We report a case of SCD due to an abnormal origin of the right coronary artery (RCA), and we discuss the diagnosis and treatment of such an anomaly.

A 35-year-old man incurred a cardiac arrest while running. The patient did not have any cardiovascular risk factors, but he did have the antecedents of unexplored chest pains and syncope on exertion. An emergency medical team performed an immediate cardiopulmonary resuscitation including defibrillation for ventricular tachycardia. The patient was admitted to the hospital where an electrocardiogram postresuscitation showed a sinus rhythm with right bundle branch block, transient ST segment elevation in DII-DIII-aVf as well as bouts of nonsustained ventricular tachycardia (Fig 1). An emergency coronary angiography was also performed, which revealed Thrombolysis in Myocardial Infarction 3 flow in the coronary arteries, but the RCA originated from the left sinus of Valsalva with a tapered proximal segment (Fig 2). Percutaneous transluminal coronary angioplasty was not attempted because ST elevation had quickly disappeared and no obvious thrombus was discernible. In-hospital peak level of troponin Ic was 18 ng/mL (upper limit, < 0.15 ng/mL). The patient was weaned from mechanical ventilation on day 2 of his hospitalization; the patient did not have any recurring myocardial ischemia. A transthoracic echocardiography showed a normal morphology of the ventricles without segmental wall motion abnormality. Multi-slice computed tomography on day 7 of his hospitalization confirmed the abnormal origin of the RCA and its initial compression between the ascending aorta and the pulmonary artery (Fig 3). After medico-surgical discussion, the RCA and the left main coronary artery ostia were considered to be too close to attempt a percutaneous transluminal coronary angioplasty. Thus, a coronary artery bypass graft of the RCA using the right internal mammary artery was performed on day 16. The surgical examination of the RCA showed atheroma in its first segment. There was no postoperative complication, and the patient was discharged from the hospital on day 25 with beta-blockers. Seven months later, he remained symptom free and was released to work. Multi-slice computed tomography in month 7 confirmed the permeability of his right internal mammary artery and RCA.


Figure 1
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Fig 1. (A) Initial electrocardiogram, incomplete right bundle branch block with Q waves and transient ST elevation in leads DII-DIII-aVf. (B) Discharge electrocardiogram.

 

Figure 2
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Fig 2. Coronary angiography showing left anterior oblique 45° view. Right coronary artery (RCA) originates from the left sinus of Valsalva. Arrow enhances systolic compression of proximal RCA.

 

Figure 3
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Fig 3. Multislice computed tomography. (A) Two dimensional cross section. (B) Three dimensional reconstruction of coronary arteries origin. Left and right coronary arteries originate from the left sinus of Valsalva. The right coronary artery systolic compression appears between the aorta and the pulmonary artery trunk. (Ao = aorta; LAD = left anterior descending artery; LCX = left circumflex artery; PA = pulmonary artery; RCA = right coronary artery.)

 

    Comment
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 Abstract
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 Comment
 References
 
Half of SCD of young patients are related to structural disease. One third of these cases are nonatherosclerotic anomalies of coronary arteries [2, 3]. The incidence of such anomalies, seen on angiography, is approximately 0.8% to 1% in retrospective studies [4]. A single coronary artery (less than 0.05%), left anterior descending coronary artery or left main coronary artery originating in the right sinus of Valsalva (5% and 25%, respectively), and the RCA originating in the left sinus of Valsalva (10%) can be involved in SCD, whereas an abnormal origin of the left circumflex artery (55%) is seldom found in SCD. The incidence of an abnormal origin of the RCA seems higher among males [5]. It can be responsible for SCD and myocardial ischemia, arrhythmia, and syncope. Several mechanisms of ischemia in case of abnormal origin of the RCA were suggested (ie, acute angulation with the aorta, split-like ostium, ostial ridge, compression between the left and right cusps of the aortic valve, or compression in the intramural part of the aorta). It may be associated with accelerated development of atherosclerotic disease [4]. In the present clinical observation, the hypothetical mechanism for SCD was assumed to be compression between the large vessels, causing ischemia by the expansion of the large vessels during effort. As this anomaly was mainly revealed by autopsies, diagnoses before mortal events are paramount. Retrospective studies noted that electrocardiograms, stress tests, and transthoracic echocardiography were often normal and could not rule out an abnormal origin of coronary arteries [3]. Therefore, angiography and multi-slice computed tomography are recommended when a suspicion persists in patients with resuscitated cardiac arrest. Multi-slice computed tomography shows the course of the abnormal coronary artery, helps to clarify the mechanism of ischemia, and helps in choosing the best surgical approach. The optimal treatment of abnormal coronary arteries is not well defined. Medical care with beta-blockers can be selected when the risk of SCD is supposed to be low. Percutaneous transluminal coronary angioplasty can be carried out as an alternative to surgery, particularly in evolving myocardial infarction. Mild re-stenosis can occur after percutaneous transluminal coronary angioplasty and long-term results are not known [6]. In our case, because the ostia of the left main coronary artery and the RCA were considered to be too close in the left sinus of Valsalva, coronary artery bypass graft was preferred. The long-term results of a coronary artery bypass graft must be evaluated to determine the risks of occlusion of the native coronary arteries due to atherosclerosis or the competing flow. Thus, one proposed the ligature of the ostium of the RCA in combination with a coronary artery bypass graft. Other techniques were described, according to the underlying mechanism (ie, fixation into the correct sinus from outside the aorta, transaortic modification of the origin, and proximal portion of the RCA, in case of slit-like orifice [7]).

In conclusion, we report an observation of resuscitated sudden death due to an abnormal RCA originating from the left sinus of Valsalva. A RCA compression between the great vessels occurred on exertion. The diagnosis was made by coronary angiography and multi-slice computed tomography. Surgical therapy with a coronary artery bypass graft was decided. There is currently no consensus for the management of this anomaly after a sudden death or an ischemic event, or when it is found incidentally. So far, screening tests have not proved to be efficient to detect this anomaly before sudden death.


    References
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 Abstract
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 Comment
 References
 

  1. Myerburg RJ, Kessler KM, Castellanos A. Sudden cardiac death: epidemiology, transient risk, and intervention assessment Ann Intern Med 1993;119:1187-1197.[Abstract/Free Full Text]
  2. Eckart RE, Scoville SL, Campbell CL, et al. Sudden death in young adults: a 25-year review of autopsies in military recruits Ann Intern Med 2004;141:829-834.[Abstract/Free Full Text]
  3. Basso C, Maron BJ, Corrado D, Thiene G. Clinical profile of congenital coronary artery abnormalities with origin from the wrong aortic sinus leading to sudden death in young competitive athletes J Am Coll Cardiol 2000;35:1493-1501.[Abstract/Free Full Text]
  4. Jim MH, Siu CW, Ho HH, Miu R, Lee SW. Anomalous origin of the right coronary artery from the left coronary sinus is associated with early development of coronary artery disease J Invasive Cardiol 2004;16:466-468.[Medline]
  5. Kimbiris D, Iskandrian AS, Segal BL, Bemis CE. Anomalous aortic origin of coronary arteries Circulation 1978;58:606-615.[Free Full Text]
  6. Hariharan R, Kacere RD, Angelini P. Can stent-angioplasty be a valid alternative to surgery when revascularization is indicated for anomalous origination of a coronary artery from the opposite sinus? Tex Heart Inst J 2002;29:308-313.[Medline]
  7. Garcia-Rinaldi R, Sosa J, Olmeda S, Cruz H, Carballido J, Quintana C. Surgical treatment of right coronary arteries with anomalous origin and slit ostium Ann Thorac Surg 2004;77:1525-1529.[Abstract/Free Full Text]



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