Anomalous Left Coronary Artery from the Pulmonary Artery with a Large Patent Ductus Arteriosus: Aversion of a Catastrophe

Abstract

We present an infant who had an anomalous left coronary artery arising from the pulmonary artery (ALCAPA) and a large patent ductus arteriosus (PDA), who was diagnosed before a potentially catastrophic closure of PDA. In the presence of normal left ventricular function and the absence of coronary artery collaterals, it is difficult to diagnose ALCAPA. A disproportionate degree of left ventricular dilation and severity of mitral valve regurgitation relative to the degree of PDA shunt, and echogenic papillary muscles on an echocardiogram should raise a suspicion of coronary artery anomalies. The infant underwent surgical ligation of PDA with translocation of coronary arteries and had an uneventful recovery.

Anomalous Origin of the Right Coronary Artery from the Pulmonary Artery in a Neonate with Turner Syndrome and Aortic Arch Hypoplasia

“…This result had been described in the presence of ALCAPA with PDA: robust blood flow through the ductus supports myocardial perfusion through the anomalous artery by elevating PA pressure. 7 Increased PA pressure may either support antegrade f low through the anomalous artery or limit retrograde f low or coronary steal if there is collateralization. When a PDA is closed percutaneously or surgically, PA pressure may decrease substantially and result in acute myocardial ischemia.…”

“…When a PDA is closed percutaneously or surgically, PA pressure may decrease substantially and result in acute myocardial ischemia. 7 In infants with unrecognized ALCAPA, PDA ligation has been associated with sudden myocardial ischemia, ventricular fibrillation and failure, and cardiac arrest. 8,9 To our knowledge, the beneficial physiologic effect of a PDA on myocardial perfusion in the presence of ARCAPA has not been described.…”

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery: A Review

“…Even though coronary heart disease is responsible for the majority of deaths associated with the heart, there are many other less prevalent, but just as deadly, heart conditions [1]. Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare heart anomaly [2][3][4][5][6][7][8][9][10][11][12]. ALCAPA is also known as Bland-White-Garland syndrome [2,3,8,10].…”

“…Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare heart anomaly [2][3][4][5][6][7][8][9][10][11][12]. ALCAPA is also known as Bland-White-Garland syndrome [2,3,8,10]. There are two types of ALCAPA: the infant-type and the adult-type."…”

“…2(p.1010) Patients who have an inefficient intercoronary anastomoses with left coronary artery (LCA) arising from the pulmonary artery (PA) have infant-type ALCAPA. The adult-type have extensive intercoronary systems supplying the left coronary system with the LCA arising from the PA [2][3]8,10]. Both types have the LCA originating in the PA where oxygen-poor blood is located [2][3]8,10].…”