Anomalous left coronary artery from pulmonary artery: Case series and brief review

Abstract

Anomalous origin of left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary anomaly. In this study, we present all the ALCAPA patients which were admitted at our institution during April 2007-December 2010. Retrospective review of these patients regarding their clinical presentation and the use of diagnostic modalities will be presented in this series. There were total of five patients, three male and 2 female, with age range of 2 -12 months. The most common symptoms at presentation were tachypnea (4/5) and poor feeding with irritability (3/5). Electrocardiogram was abnormal in 2/5 cases and chest X ray revealed cardiomegaly with pulmonary congestion in 4/5 patients. Echocardiogram showed mitral valve regurgitation in 5/5 cases (3 with moderate and 2 with mild to moderate), Left ventricular dilatation/dysfunction in 4/5 patients, echogenic left ventricular papillary muscles in 4/5 patients and prominent right coronary with strong suspecision of ALCAPA in 4/5 patients. Coronary angiography was performed in 4/5 cases to confirm the diagnosis. We conclude that by thorough clinical assessment along with ECG and CXR, the diagnosis of ALCAPA can be strongly suspected. Echocardiogram can almost always make the diagnosis of ALCAPA and coronary angiography can confirm the diagnosis in rare atypical cases.

The role of multimodal imaging in the diagnosis of an asymptomatic patient with congenital anomaly

“…Electrocardiography may raise the suspicion of this anomaly in a young adult, showing ischemic changes (most frequently negative T waves in DI and aVL) [4], which were also present in our patient.…”

Effective myocardial perfusion and concomitant haemodynamic status determine the clinical diversity of anomalous left coronary artery from the pulmonary artery

“…First, regardless of the severity of clinical presentation, all patients presented with cardiomegaly on chest radiograph as well as variable degree of left ventricular dilation, as was reported previously. 4,10,15,16 Cardiomegaly primarily represents left ventricular dilatation due to direct myocardial ischemic injury, compensatory response to left ventricular systolic dysfunction, and/or increased volume overload due to left-to-right shunt via collateral vessels and mitral regurgitation. Second, all patients except one (patient #12) initially presented with clinical evidence of myocardial ischemic injury regardless of the severity.…”

Anomalous Left Coronary Artery from Pulmonary Artery: An Important Cause of Ischemic Mitral Regurgitation in Children

“…Three embryological theories have been put forth to explain this anomaly: the first proposes an abnormal disposition of the aortopulmonary septum; the second one suggests persistence of the left coronary buds on the pulmonary artery and involution of the aortic buds permitting anomalous development of that coronary from the wrong artery; the third one relates to a septation defect of the conotruncus tube [4-5]. The anomaly rarely involves the right coronary artery, left anterior descending artery, or both coronary arteries, the latter being incompatible with life [6]. Although reported to be fatal in infancy, children can survive past infancy into teenage and adulthood without symptoms.…”

“…The fourth stage is of normal pulmonary arterial pressure and vascular resistance with the left coronary circulation supplied by the right coronary artery and a left-to-right shunt ensues into the pulmonary artery. This causes subclinical myocardial ischemia leading to mitral valve regurgitation and sudden cardiac death due to arrhythmia [5-6]. The first two stages explain the pediatric type and the last two are found in the adult type of presentations.…”