Anomalous origin of the right coronary artery from the main pulmonary artery: Diagnosis and management

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 ...

Hallazgo casual de origen anómalo de la arteria coronaria derecha desde la arteria pulmonar en un lactante

“…11 Además, como en el caso de nuestro paciente, las arterias coronarias dilatadas y el flujo retrógrado desde la arteria coronaria derecha hacia la arteria pulmonar son otras manifestaciones ecocardiográficas típicas de esta afección. 12,13 Podría resultar difícil realizar estudios de diagnóstico por imágenes de las arterias coronarias en algunos pacientes debido a las ventanas acústicas deficientes. La tomografía computarizada multicorte y la resonancia magnética cardíaca son modalidades de diagnóstico por imágenes no invasivas con una elevada precisión diagnóstica para detectar anomalías anatómicas en las arterias y podrían ser otras opciones para el diagnóstico.…”

An incidentally detected anomalous origin of the right coronary artery from the pulmonary artery in an infant

“…10 Certain echocardiographic findings, such as intracoronary collaterals within the ventricular septum visualized by color flow Doppler, has been reported to be highly suggestive of ARCA-PA. 11 In addition, like our patient, dilated coronary arteries and a retrograde flow from the right coronary artery into the pulmonary artery is another characteristic echocardiographic finding for this disease. 12,13 Coronary artery imaging with echocardiography may be difficult in some patients due to poor acoustic windows. Multislice CT and cardiovascular MRI which are noninvasive imaging modalities with high diagnostic accuracy for coronary artery anatomic anomalies may be another options for diagnosis.…”

Anomalous Origin of the Right Coronary Artery from the Pulmonary Artery Diagnosed as an Incidental Finding

“…However, other congenital anomalies such as ventricular septal defect, atrial septal defect, double outlet right ventricle, patent ductus arteriosus, coarctation of the aorta, pulmonary stenosis, and aortic stenosis have been also reported. 2,5 Certain echocardiographic findings, such as intercoronary collaterals within the ventricular septum visualized by color flow Doppler, has been reported to be highly suggestive of this syndrome. 6 In addition, like our patient, dilated coronary arteries and a retrograde flow from the RCA into the pulmonary artery is another characteristic echocardiographic finding for this disease.…”

“…6 In addition, like our patient, dilated coronary arteries and a retrograde flow from the RCA into the pulmonary artery is another characteristic echocardiographic finding for this disease. 4,5 The diagnosis of ARCAPA is usually achieved by an invasive coronary angiogram, showing a dilated and tortuous RCA with collateral filling from the left coronary system. Silent myocardial ischemia might be the only sign in asymptomatic patients, 4 being responsible of the potential risk of sudden death; thus, scintigraphy would take place to rule out this concern.…”

“…7,8 As stated before, although the diagnosis can be established noninvasively, cardiac catheterization is still suggested to assess the degree of the shunt. 3,5 Therapeutic recommendations for ARCAPA patients are controversial. Several authors claim the potentially fatal outcome and therefore suggest reimplantation of the RCA into the aortic root soon after the diagnosis.…”