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Ann Thorac Surg 1987;43:207-211
© 1987 The Society of Thoracic Surgeons

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Articles

Surgical Management of Infants with Complex Cardiac Anomalies Associated with Reduced Pulmonary Blood Flow and Total Anomalous Pulmonary Venous Drainage

From the Divisions of Cardiovascular-Thoracic Surgery and Cardiology, the Children's Memorial Hospital, and the Departments of Surgery and Pediatrics, Northwestern University Medical School, Chicago, IL

Accepted for publication April 21, 1986.

^* Address reprint requests to Dr. DeLeon, Division of Cardiovascular-Thoracic Surgery, The Children's Memorial Hospital, 2300 Children's Plaza, Chicago, IL 60614

Eight infants with complex cardiac anomalies and pulmonary stenosis or atresia were noted to have obstructed total anomalous pulmonary venous drainage (TAPVD) either at the initial cardiac catheterization (Group 1; n = 2) or after creation of systemic-pulmonary artery shunts (Group 2; n = 6). The 2 patients in Group 1 underwent early repair of TAPVD (1 at 7 days, the other at 1 months of age) before any subsequent operation and are now doing well at 18 months of age. The 6 patients in Group 2 underwent repeat cardiac catheterization because of persistent severe cyanosis with faint or absent continuous murmur and were found to have patent shunts and obstructed TAPVD (1 mild, 5 severe). One patient who underwent repair of TAPVD at 2 months of age survived and is well at 2 years of age, whereas 4 patients who underwent repair at an average age of 6 months (age range, 3–16 months) subsequently died. The sixth patient, who did not undergo repair, remained severely cyanotic with hypoplastic pulmonary arteries in spite of repeated shunts. We feel that increased awareness of the possible association of TAPVD and reduced pulmonary blood flow in infants with complex cardiac defects, in combination with echocardiography, oxygen saturation studies, and angiography with prostaglandin E₁ challenge, should lead to early diagnosis, avoidance of unnecessary systemic-pulmonary artery shunts, and increased survival rates in these infants.

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