No abstract

Summary: Anomalous origin of the right coronary artery (ARCA) from the main pulmonary artery (MPA) is a rare congenital anomaly, with only 18 reported cases in the pediatric age group. More than half of these had associated cardiac anomalies that masked the presence of ARCA. Conversely, in many patients with ARCA as an isolated anomaly, the diagnosis has been missed during lifetime. The only patient with an isolated ARCA who was diagnosed in infancy presented with congestive heart failure. Asymptomatic infants with ARCA from the MPA have not been previously reported. Three additional cases, two infants and a child with ARCA from the MPA, are reported in this paper. The diagnostic dilemmas and the prognosis are discussed and management is recommended.Key words: anomalous, right coronary artery, diagnosis, management defect in 2; tetralogy of Fallot in 2; and ventricular septal defect, patent ductus arteriosus. and pulmonic stenosis each in 1 patient. Of the eight patients in whom ARCA was an isolated anomaly, one presented with congestive heart failure, four were asymptomatic but had heart murmurs considered significant, and three were diagnosed at autopsy. Four of the patients were diagnosed in infancy and three of these had additional cardiac anomalies."-l I The only patient with an isolated ARCA who was diagnosed in infancy presented with congestive heart failure.12 No patients with ARCA from the MPA who were asymptomatic were previously diagnosed in infancy. In this paper, three additional cases, two infants and one child with isolated ARCA from the MPA, are reported. All three were asymptomatic and were diagnosed by two-dimensional echocardiography and Doppler studies. Their management is discussed and a review of the literature is presented. Case No. I IntroductionAnomalous origin of the right coronary artery (ARCA) from the main pulmonary artery (MPA) is a rare congenital anomaly first reported by Brooks I as an incidental autopsy finding. Overall, 52 such cases have been reported in the literature,l-x but only 18 were reported in the pediatric age group (< 18 years). In this group, 10 patients had associated cardiac defects, including aortopulmonary window in 5; atrial septal A 9-month-old African American girl was referred for evaluation of a heart murmur. Birth and subsequent history were unremarkable, and she has been asymptomatic regarding her cardiovascular system. On physical examination, weight was 7.5 kg (5th percentile), height was 71 cm (25th percentile), heart sounds were normal, and there was a grade 11-III/VI continuous murmur at the left upper sternal border. The rest of the cardiovascular examination was normal. The electrocardiogram (ECG) showed a QRS axis of 80°, deep Q waves of 4-8 mm in leads 11,111, and aVF, but the width of the Q waves was only 1 mm. There was mild left ventricular hypertrophy by voltage criteria. The two-dimensional (2-D) echocardiogram showed a right coronary artery (RCA) in direct communication with the MPA (Fig. 1). The RCA was uniformly dilated and tortuous, and ...