Three Cases of ALCAPA with Associated Anomalies

Baskar Ranjith Karthekeyan,Jebaraj Rethinasamy,Mahesh Vakamudi,Kamalakannan Sambandham,Siva Muthukumar,Periyasamy Thangavelu,Rajeshkumar Kodali,Sushma Nandipati

doi:10.4236/wjcs.2014.41001

Abstract

There are several potential issues that affect the treatment and diagnostic pattern of anomalous left coronary artery arising from the pulmonary artery. We report three cases of infants who presented with anomalous left coronary artery arising from the pulmonary artery with severe left ventricular dysfunction and severe mitral regurgitation along with associated anomalies. One patient had congenital lobar emphysema of the right midde lobe. Another patient had left main stem bronchus compression, collapse of basal segments of left lower lobe and panlobular emphysema in medial basal segment of right lower lobe. The third patient had cleft lip and palate. All patients underwent successful repair. The hemodynamic stability was compromised when the infant with congenital lobar emphysema had spontaneous pneumothorax after extubation and she needed an intercostal drainage. The infant with lung collapse had to be reintubated on the second day since she became hypoxic due to recollapse of the lung once the airway positive pressure was removed. She needed chest physiotherapy, vigorous endotracheal suctioning and inhaled bronchodilator therapy. The patient who had cleft palate succumbed to aspiration pneumonitis in the postoperative period. Follow-up of other two patients after three months showed very good improvement in left ventricular systolic function.

Highlights

Anomalous left coronary artery arising from the pulmonary artery is one of the few congenital heart anomalies in which myocardial function is profoundly compromised
The first clinical description of this anomaly was given by Bland, White, Garland in 1933 and anomalous left coronary artery arising from the pulmonary artery bears the eponym Bland-White-Garland syndrome [1]
We report three infants who presented with anomalous left coronary artery arising from the pulmonary artery with severe left ventricular dysfunction

Summary

Introduction

Anomalous left coronary artery arising from the pulmonary artery is one of the few congenital heart anomalies in which myocardial function is profoundly compromised. The first clinical description of this anomaly was given by Bland, White, Garland in 1933 and anomalous left coronary artery arising from the pulmonary artery bears the eponym Bland-White-Garland syndrome [1]. Myocardial ischaemia develops in the territory of the left coronary artery, interfering with left ventricular contractility and mitral valve function. The onset of the symptoms and the degree of myocardial insufficiency, or mitral valve regurgitation, depend on a balance of the rapidity of ductus arteriosus closure, maintenance of pulmonary hypertension and development of intercoro-. Nary collateral vessels from the right coronary artery to the anomalous left coronary artery arising from the pulmonary artery. In the adult type the collaterals are well developed, symptoms occur only in adulthood, or sometimes never appear [3]

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Discussion

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