Pulmonary vein atresia with severe contralateral pulmonary vein stenosis in a child.

Unilateral pulmonary vein atresia is a very rare congenital anomaly associated with high morbidity and mortality. Most cases present in infancy or childhood with recurrent respiratory infections or hemoptysis. Further, the diagnosis can often be difficult to make. We present an infant who died due to unilateral right-sided pulmonary vein atresia in conjunction with severe contralateral pulmonary vein stenosis who was diagnosed with the assistance of cardiac computed tomography scanning.