Anomalous origin o f the left c o r o n a r y artery from the p u l m o n a r y artery is a rare congenital heart defect accounting for less than 0.5% o f patients with COngenital heart disease [3]. Early diagnosis is important for optimal medical and surgical management. Traditionally, the triad of congestive heart failure, significant cardiomegaly without a murmur, and electrocardiographic evidence o f ischemia or infarction has strongly suggested the diagnosis of rted in part by Research Fellowship Award 1 F32 HL-05675-01~ and by research grant HL-12738-092 from the National Heart, Lung, and Blood Institute, United States Public Health Service.Address reprint requests to: Ehud Krongrad, MD, Department of Pediatrics, Columbia Presbyterian Medical Center, 3975 Broadway (BHA 1-102), New York, NY 10032, USA
Materials and MethodsClinical Data. Observations were made in four infants who were referred for cardiac evaluation to Babies Hospital, Columbia Presbyterian Medical Center. The initial clinical and pertinent cardiac catheterization data are given in Table 1. All the patients were in severe congestive heart failure. Only one infant had an apical murmur of mitral insufficiency. Chest x-ray films showed striking cardiomegaly and pulmonary vascular congestion.The presenting electrocardiogram was abnormal in all four patients. Common features were deep Q-waves in leads 1 and AVL and signs of left ventricular ischemia or infarction. In addition, there was evidence of left ventricular conduction delay with a QRS duration of 0.08 to 0.09 sec. and a slurred or notched upstroke of the R wave in V5 and V6 in each case. The electrocardiogram from patient 2 is representative (Fig. 1). Echocardiograms were performed at the time of initial evaluation.Cardiac catheterizations were also performed, and the diagnosis of anomalous left coronary artery arising from the pulmonary artery was confirmed in each patient. Left ventricular function was uniformly impaired with high left ventricular end-diastolic pressures and markedly decreased ejection fractions. A left-to-0172-0643/79/0001-0023 $01.20