A large coronary-pulmonary artery fistula in a cyanotic patient leading to severe biventricular dysfunction and heart failure

In cases of pulmonary atresia (PA) with ventricular septal defect (VSD), the commonest source of pulmonary blood flow is via major aortoplumonary collaterals. Collaterals may also arise from the coronary arteries and the reported prevalence of such coronary-pulmonary artery fistulas (CPAF) in PA with VSD is 10%. However gross congestive heart failure (CHF) and ventricular dysfunction is extremely rare in such cases. We report a 17-year-old male with PA with VSD and a large CPAF from the anterior right aortic sinus connecting to the left pulmonary artery, who presented with severe CHF. The left anterior descending and the right coronary artery both arose from the proximal part of the CPAF, possibly leading to extensive coronary steal and biventricular dysfunction.