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.
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* From the Departments of Surgery, Pediatrics and Pathology, University of Minnesota, Minneapolis, and the Department of Pathology, The Charles T. Miller Hospital, St. Paul, Minn. This study was supported by Research Grant 5 ROl HE-05694 and Research Training Grant 5Tl HE 5570 from the National Heart Institute.