Serafin Y. DeLeon, Samuel S. Gidding, Michel N. Ilbawi, Farouk S. Idriss, Alexander J. Muster, Roger B. Cole, Milton H. Paul
Northwestern University Medical School. Children’s Memorial Hospital.
United States
Annals of Thoracic Surgery
Ann Thorac Surg 1987; 43: 207-211
DOI: 10.1016/s0003-4975(10)60398-0
Abstract
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 1/2 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 1/2 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.
Category
Stenosis or Obstruction of Pulmonary Veins Following Surgical Repair of Anomalous Pulmonary Venous Connections
Surgical Interventions for Pulmonary Venous Obstruction After the Onset of Disease
Year of Publication: 1987
Age Focus: Pediatric
Article Type: Case Reports or Retrospective Observations in Small Groups of Patients (≤10 patients)
Article Access: Free PDF File or Full Text Article Available Through PubMed or DOI: Yes