Background: Survival after repair of total anomalous pulmonary venous return in patients with heterotaxy syndrome has consistently been reported in the literature to be far inferior to the results obtained in nonheterotaxy patients. The outcomes of heterotaxy patients at Texas Children's Hospital undergoing repair of total anomalous pulmonary venous return were examined and compared with those of nonheterotaxy patients.
Methods: Between July 7, 1995, and November 22, 2005, 122 consecutive patients underwent primary repair of total anomalous pulmonary venous return. Characteristics were as follows: mean age, 0.35 +/- 1.8 years; mean weight, 4.3 +/- 2.8 kg; single ventricle, 40 (33%); heterotaxy, 38 (31%); pulmonary atresia, 13 (11%). Concomitant procedures included pulmonary artery banding in 4 (3%), systemic-to-pulmonary shunt in 14 (12%), bidirectional Glenn in 11 (9%), and other in 16 (14%).
Results: With a mean follow-up of 2.6 +/- 2.5 years including all patients, overall 30-day and 5-year survival was 93% and 86%, respectively; for heterotaxy patients it was 84% and 79%; and for nonheterotaxy patients it was 96% and 89%. Heterotaxy and nonheterotaxy survival curves were not significantly different (p> 0.05). Overall, there were 12 (9.8%) patients who required reoperation for pulmonary vein stenosis, at a mean of 5.1 +/- 5.5 months postoperatively. Seven (58%) reoperations were in heterotaxy patients. Rate of pulmonary vein reoperation is statistically greater (p < 0.05) for heterotaxy patients than nonheterotaxy patients. At last follow-up, all patients were in New York Heart Association class II or less. Using Cox proportional hazards models, pulmonary atresia and need for systemic-to-pulmonary shunt were risk factors for death (not heterotaxy). Single ventricle, operation after July 2000, and need for systemic-to-pulmonary shunt were risk factors for pulmonary vein reoperation.
Conclusions: At midterm follow-up, heterotaxy patients undergoing total anomalous pulmonary venous return repair have a higher pulmonary vein reoperative rate but a comparable overall survival to nonheterotaxy patients.