Anomalies of the pulmonary veins^*

Eighty pathologic specimens with anomalies of pulmonary veins were reviewed for the type of lesions encountered. These were classified on an anatomic basis into five groups, characterized by (1) stenotic lesions; (2) accessory veins; and (3) anomalous connection of pulmonary veins (a) either partial or total, to a systemic vein or right atrium (the most common condition found), (b) to a systemic vein while connection to the arterial atrium was also present and (c) to a pulmonary artery (arteriovenous fistula). Combinations may occur, since pulmonary veins involved in anomalous connection may also be stenotic. The latter combination effects an adverse course on the patient with total anomalous pulmonary venous connection. The clinical manifestations of these malformations are dependent on the anatomic structure and in general result in one of three clinical pictures: (1) pulmonary venous obstruction, (2) bidirectional shunt and (3) cyanosis without abnormal cardiac signs.