Familial pulmonary veno-occlusive disease: a case report

Abstract

A case of pulmonary veno-occlusive disease in a male infant who died at the age of 3 months is presented. Both intra- and extrapulmonary veins were involved. Two years earlier a brother had died of the same disease at the age of 8 weeks, but, in that case, the disease was restricted to the intrapulmonary veins. It is suggested that the disease may have been caused by a viral infection, the mother acting as a carrier. The simultaneous occurrence of intra- and extraparenchymal pulmonary vein occlusion indicates that some instances of isolated extraparenchymal pulmonary vein atresia or obstruction may also have been examples of pulmonary veno-occlusive disease.