Abstract 12907: Treatment of Anomalous Left Coronary Artery From the Pulmonary Artery in a 60-year-old Patient With Primary Re-implantation

Abstract

Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect accounting for 0.26% - 0.46% of congenital anomalies. Most (85%) cases are diagnosed in the first month of life with only 18-25% of patients reaching adulthood. Patients diagnosed in adulthood typically have collaterals from the right coronary artery (RCA) which provide blood flow to the left coronary artery (LCA) system. They can present with heart failure, mitral insufficiency, arrhythmias, and/or sudden cardiac death. An otherwise healthy 60 y/o female presented to an outside hospital after a witnessed cardiac arrest when leaving the gym. Bystander CPR was started, and she received 2 shocks from an automated external defibrillator for ventricular tachycardia/ventricular fibrillation (VT/VF) with return of spontaneous circulation and taken to the local ED. A CTPE showed no acute PE and a possible ALCAPA. She was transferred to our institution for further assessment by adult congenital cardiology. TTE was performed on arrival which showed a global cardiomyopathy with color flow in the myocardium consistent with extensive collateralization. CT coronary artery was performed which confirmed ALCAPA anatomy without significant CAD. The case was discussed with CT surgery, interventional cardiology and adult congenital cardiology. The decision was made for a right and left heart catheterization and cardiac MRI to plan for possible surgery. Angiography showed a large RCA with extensive collaterals filling the left coronary system and no significant disease. RHC showed a normal cardiac output with a large left to right shunt and moderate pulmonary hypertension. MRI showed a mild diffuse subendocardial ischemia with high viability of the entire left ventricle and a pulmonary to systemic flow ratio (Qp:Qs) of 2.1. The decision was made to go ahead with primary re-implantation of the LCA to the aorta, the preferred method. Ligation of the anomalous coronary artery and coronary artery bypass grafting can also be used. She was taken for surgery and tolerated the procedure well. Follow up echo is pending, but she has had no further VT/VF episodes. This case demonstrates successful re-implantation of an ALCAPA in a symptomatic adult.