Stenosis of pulmonary veins with ventricular septal defect. A cause of premature pulmonary hypertension in infancy.

Pulmonary vein stenosis is recognised as a rare, silent cause of pulmonary hypertension. ' The condition can occur with or without intracardiac anomalies. '3 The presence of lesions, such as ventricular septal defect, which cause pulmonary hypertension in their own right, can make suspicion of associated pulmonary venous obstruction difficult. In only one of the four reported cases4-7 of ventricular septal defect with pulmonary vein stenosis was the diagnosis made before operation. We report two further cases with unusual clinical presentation.