Transposition of the Pulmonary Veins

Abstract

The seriousness of incomplete transposition of the pulmonary veins has probably been underestimated in the past.Data on 11 patients is presented, including 10 with associated atrial septal defects. Common symptoms were dyspnea, fatigability, and frequent respiratory infections. Examination revealed no cyanosis, widely split second sound, soft systolic murmur and middiastolic murmur at the apex. One third demonstrated a presystolic murmur of medium pitch. The electrocardiographic and radiographic features were characteristic of atrial septal defects. There were no specific vascular shadows in any of our patients. Catheterization data were also characteristic of atrial septal defects. There was an inverse relationship between the right atrial pressure and systemic index. Anomalous pulmonary veins were entered during the right heart catheterization in 6 of the 10 patients catheterized.Seven patients have had atrioseptopexy to close the septal defect and to direct the blood from the anomalously draining veins into the newly enlarged left atrium. An eighth patient had repair and shift of the atrial septum by means of open heart surgery through a pump oxygenator. This approach will probably prove optimal for future operations.