RESPIRATORY INSUFFICIENCY IN INFANCY DUE TO CONGENITAL HYPOPLASIA OF PULMONARY VEINS

An infant with progressive respiratory insufficiency beginning at 4 we eks of age presented with the characteristic radiological signs of int erstitial lung disease. An open lung biopsy, however, showed no inters titial inflammation, but rather a marked dilatation of pulmonary capil laries suggesting obstructed drainage of pulmonary veins. Subsequent e chocardiography and angiography revealed generalised pulmonary vein st enosis and -hypoplasia. Attempts to dilate the pulmonary veins by impl anting stents into two pulmonary veins led to radiologic improvement o f lung oedema, but this was not accompanied by any clinical improvemen t. The patient died of respiratory insufficiency at 4 months of age. C ongenital pulmonary vein stenosis, although rare, should be included i n the differential diagnosis of infants presenting with progressive re spiratory insufficiency. Unfortunately, therapeutic options for this s evere disease are as yet unsatisfying.