SESSION TYPE: Cardiovascular Case Report Posters
PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM
INTRODUCTION: First described in 1866 by Brooks, anomalous origin of the coronary arteries from the pulmonary artery is a rare congenital anomaly that tends to occur in isolation. It comprises 0.4% of all congenital heart diseases. Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) has an estimated incidence of 1 in 300,000. It is also known as Bland-White-Garland syndrome since they first described it clinically in 1933. We present a rare case of the 1st presentation of ALCAPA in adulthood with ventricular fibrillation and cardiac arrest.
CASE PRESENTATION: A 30-year old man with a past medical history of polysubstance abuse and hepatitis C was found down. He received defibrillation for ventricular fibrillation as part of cardiopulmonary resuscitation in the field. On arrival, the patient was alert and with an unrevealing physical examination. Initial lab work showed mildly elevated cardiac troponins. EKG showed left bundle branch block, left axis deviation and signs of left atrial enlargement. Urine drug screen was negative and chest radiograph was unremarkable. Echocardiography showed reduced left ventricular ejection fraction (LVEF) along with regional wall motion abnormalities in the distribution of the left anterior descending and left circumflex coronary arteries. Coronary angiography revealed global hypokinesis of the left ventricle with a LVEF at 35% and anomalous origin of the left main coronary artery from the pulmonary artery with otherwise normal coronaries. The patient underwent surgical correction with aortic reimplantation of the left coronary artery as well as ICD implantation for secondary prevention.
DISCUSSION: ALCAPA is a rare congenital anomaly with 85% of cases presenting in the first few months of life; usually with severe left sided heart failure and mitral insufficiency. Delayed presentations of ALCAPA into late adulthood have been reported and are ascribed to good collateral flow from the right coronary system. In adulthood, ALCAPA can present with symptoms of heart failure, mitral valve insufficiency, angina or arrhythmias. Arrhythmias in ALCAPA either arise from scar tissue after previous myocardial infarctions as with our patient or are triggered from an acute ischemic event due to coronary steal phenomenon.
CONCLUSIONS: ALCAPA as a rare cause of arrhythmias and left sided heart failure should be considered in adults in the absence of atherosclerotic coronary artery disease. Diagnosis with CT angiography is equivalent if not superior to coronary angiography. Recognition of this rare entity is important since surgical treatment generally results in an excellent prognosis.
1) Kristensen, T., Kofoed, K.F., Helqvist, S., Helvind, M. & Sondergaard, L. Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) presenting with ventricular fibrillation in an adult: a case report. J Cardiothorac Surg 3, 33 (2008).
DISCLOSURE: The following authors have nothing to disclose: Mona Selej
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