ACARPA, a rare case of ALCAPA

Abstract

An anomalous circumflex coronary artery arising from the right pulmonary artery is very rare. Clinical presentation varies from absence of symptoms to arrhythmia and sudden cardiac death. Since prevalence is very low, no definite therapy has been delineated yet. A 19-year-old patient, previously treated for a vascular ring and mitral valve stenosis, was diagnosed with this anomaly through echocardiography revealing collateral coronary flow, with confirmation of this coronary anomaly on subsequent computed tomography examination. To alleviate the burden for arrhythmia in this young patient, he was successfully treated with surgical reimplantation followed by an uneventful recovery.

Anomalous origin of the circumflex coronary artery from the pulmonary artery: a case report

“…When no congenital heart disease is present, the presence of an anomalous LCX arising from the pulmonary artery is subsequently very rare [1,2]. We previously described an adult case discovered at routine echocardiography [3]. The precise incidence of anomalous coronary origin from the pulmonary artery is unknown.…”

“…In ACxAPA, the majority arises from the right pulmonary artery. In approximately 40% of the ACxAPA and ALADAPA patients, a concomitant cardiac lesion was found [2][3].…”

“…Presentation can range from an asymptomatic murmur, sometimes with discrete ECG changes, exertional angina, dyspnea to sudden cardiac death [1,2,3]. The symptoms and prognosis are determined by the amount of collateral circulation to provide adequate myocardial perfusion [1].…”

“…In neonates with ALCAPA and inadequate collateral circulation, symptoms can present after pulmonary resistance has decreased due to "coronary steal" with passive ow from the coronary artery to the pulmonary artery, resulting in 90% mortality if the anomalous coronary artery is not treated surgically [2]. If collateral arteries arising from the RCA supply adequate circulation and coronary steal is minimal, patients can remain asymptomatic, until the development of cardiac dysfunction or incidental diagnosis [2,3].…”

“…The treatment goal remains eliminating coronary steal and creating antegrade ow through the anomalous coronary artery in symptomatic patients. This can sometimes be performed by ligation of the LCX with or without aorta-coronary bypass, or reimplantation of the LCX in the aorta [1,2,3]. In the review by Guenther et al 72% of the patients (of which 35% asymptomatic) underwent a surgical intervention, with reimplantation of the anomalous coronary artery being the most common procedure (42.9%) [2].…”