285: Adult-type anomalous origin of coronary artery from pulmonary artery: Echocardiographic and Doppler diagnosis and outcomes

Abstract

Adult-type abnormal origin of coronary artery from pulmonary artery (ACAPA) is uncommon compared to infant-form which usually induces extended myocardial necrosis and severe heart failure. The adult-type form results in mild and rare symptoms, but it can cause myocardial ischemia and sudden cardiac death at childhood and adult age. The objective of this study was to describe the mode of presentation, cardiovascular imaging methods for diagnosis and outcomes of patients diagnosed with adult-type ACAPA. The Patient Congenital Heart Disease database was reviewed to identify all patients diagnosed with ACAPA and/or who had surgical repair beyond the first year of life. From March 1976 to July 2011, 13 patients were identified with adult-type ACAPA, at the age of 6 months to 64 years: 9 with left coronary artery from pulmonary artery (ALCAPA) and 4 with right coronary artery from pulmonary artery (ARCAPA). Cardiac symptoms initiated investigation in 9 patients and a murmur in 4. Nine were diagnosed by transthoracic echocardiography, 4 by coronary artery angiography and 1 by cardiac computed tomography. Myocardial ischemia was detected preoperatively in 6 patients with ALCAPA (66.7%) and 1 with ARCAPA (25%). Ten patients underwent surgical implantation of the ACAPA into the ascending aorta. All were asymptomatic after repair except one who required a second surgery at 7.9 years follow-up. Adult-type ACAPA can be assessed by non invasive cardiovascular imaging. Direct aortic implantation is a reliable and effective to establish dual coronary artery circulation and prevent risks due to myocardial ischemia.