Isolated partial anomalous pulmonary venous drainage associated with pulmonary vascular obstructive disease

Five patients with partial anomalous pulmonary venous drainage with intact atrial septum are described. In two patients, pulmonary arterial hypertension and pulmonary vascular disease developed. Both had one or more right pulmonary veins draining anomalously to the right superior vena cava. It is postulated that a combination of increased pulmonary blood flow and reflex pulmonary vascular changes contributes to the production of pulmonary vascular obstructive disease.