Rare type of intrapulmonary drainage of one lung by the other with total anomalous pulmonary venous return

A rare form of obstructed total anomalous pulmonary venous drainage is presented. A neonate with asplenia, pulmonary atresia and complex congenital heart disease also had drainage of the left lung forming a venous confluence that proceeded in an intrapulmonary fashion through the right lung. A hypoplastic pulmonary venous channel then emerged from the right upper lobe leading to the superior vena cava. Pulmonary venous obstruction was suspected on initial plain chest X-ray examination and on angiography, which revealed the pulmonary artery to be larger than anticipated. Administration of prostaglandin E1 may allow augmentation of pulmonary blood flow and angiographic definition of this association in infants with asplenia complex.