Copyright © Italian Federation of Cardiology. Unauthorized reproduction of this article is prohibited. A rare combination of coronary anomalies: what is the culprit? Vincent Roule a , Ziad Dahdouh a , Jean Goupil b , Katrien Blanchart a , Julien Wain-Hobson a and Gilles Grollier a The case of a woman with anomalous origin of the circumflex coronary artery that communicates with the left ventricle via a fistula, revealed by typical angina, is described and the several pathomechanisms involved are discussed. J Cardiovasc Med 2011, 12:883–884 Keywords: coronary anomalies, fistula, myocardial ischemia a Department of Cardiology and b Department of Radiology, University Hospital of Caen, Caen, France Correspondence to Vincent Roule, MD, Cardiology Department, University Hospital of Caen, Avenue Cote de Nacre, 14033 Caen, France Tel: +33 02 3106 3048; fax: +33 02 3106 4418; e-mail: v.roule@hotmail.fr Received 3 July 2011 Revised 30 August 2011 Accepted 1 September 2011 A 74-year-old woman with a history of hypertension and hypercholesterolemia presented recurrent constrictive chest pains on moderate exertion. Her clinical examin- ation was normal with a blood pressure of 135/85 mmHg. The ECG showed sinusal rhythm of 70 beats/min without ischemic changes. The transthoracic cardiac ultrasound was normal. The 6-h troponin I was raised to 0.7 ng/ml. Coronary angiography (Fig. 1) highlighted an anomalous rise of the circumflex artery originating from the right coronary artery shortly after its ostium, with marked angulation at its origin. The circumflex artery reached the atrioventricular groove and communicated with the left ventricular via a fistula. Otherwise, the other coronary arteries were smooth. Left ventriculography showed a normal ejection fraction with left ventricular hypertrophy. Multidetector computed tomography scan showed no interarterial course between the aorta and the pulmonary artery (Fig. 2). Due to the advanced age of our patient, a conservative approach was preferred and she was discharged on medical treatment including b-blockers, statins and aspirin. She remained event free at 3 months. During follow-up, we performed an ECG exercise test after withholding b-blockers, according to a standard Bruce protocol. The test, which was interrupted for muscular weakness at 90 W (90% of maximal heart rate), showed significant ST segment depression in the lateral leads. The exercise test was repeated 1 week later under b-blockers and was normal. As the patient was asymptomatic, we decided to continue the medical treatment. The incidence of congenital anomalies of the coronary arteries varies according to the studies from 0.3 to 8.3% due to the different definitions used. 1 The origin of the circumflex artery in the right coronary sinus is the most common anomaly of the origin of the coronary arteries. Coronary artery fistulas are rare and only a few cases of congenital fistula between left coronary artery and left ventricle have been described. 2 If most of the coronary anomalies seem to be only curiosities, a small number are associated with myocardial ischemia and sudden death. Several pathomechanisms could explain myocardial ischemia in our patient. First, the marked angulation of the anomalous rise of the circumflex at its origin, which has to bend over itself to reach its normal supply territory, could narrow the ostium to a slit and cause ischemia, 1 especially during exercise and with age because the aorta Images in cardiovascular medicine Fig. 1 Coronary angiogram showing anomalous rise of the circumflex coronary artery (Cx) originating from the right coronary artery (RCA) (a); smooth left descending coronary artery (b); fistula (arrow) between anomalous circumflex coronary artery and left ventricle (c); and origin of the anomalous circumflex coronary artery (zoom) showing a marked angulation (d). 1558-2027 ß 2011 Italian Federation of Cardiology DOI:10.2459/JCM.0b013e32834cadc4