Coexistence of partial anomalous pulmonary venous drainage and pulmonary atresia with ventricular septal defect: a report of two cases

Coexistence of partial anomalous pulmonary venous drainage (PVD) and pulmonary atresia with ventricular septal defect (PA-VSD) is very rare with the exception of heterotaxy syndromes. We presented two cases of PA-VSD in which partial anomalous PVD was detected during pulmonary artery angiography. One was a six-month-old girl who underwent cardiac catheterization to evaluate the chance of unifocalization. There were true pulmonary arteries and three major aortopulmonary collaterals. It was noted that the right upper pulmonary vein was in direct continuity with the right atrium. The second case was a 12-year-old girl who was previously diagnosed as having PA-VSD and absence of true pulmonary arteries, for which she had undergone two subsequent aortopulmonary shunt operations four years before. During cardiac catheterization, confluent but hypoplastic true pulmonary arteries and multiple minor aortopulmonary collaterals were detected. The right and left upper pulmonary veins were draining to the right atrium. It should be known that, albeit very rare, partial anomalous PVD may be present in PA-VSD cases, requiring careful evaluation of pulmonary venous drainage during echocardiographic and angiographic studies.