Long-Term Outcomes of Total Anomalous Pulmonary Venous Drainage Repair in Neonates and Infants.

BACKGROUND

Outcomes of operations for total anomalous pulmonary venous drainage (TAPVD) have improved. However, operations in the neonatal period and the development of postoperative pulmonary venous obstruction are associated with a high mortality rate.

METHODS

A retrospective review was conducted for all neonates and infants (n = 214) who underwent operations for isolated TAPVD (1973 to 2014).

RESULTS

Median age was 18 days (1 day to 1 year). There were 17 (7.9%) early deaths. Risk factors for early death were prolonged cardiopulmonary bypass time (p = 0.005) and neonatal age at the operation (p = 0.048). Early mortality was 2.5% for infants (n = 81) and 11% for neonates (n = 133; p = 0.021) during the entire study period. Hospital deaths for neonates remained unchanged during the four eras of 1973 to 1988, 1989 to 1998, 1999 to 2008, and 2009 to 2014. Survival at 10 and 20 years was 88% ± 2.2% (95% confidence interval, 82% to 91%). Reoperation for postoperative pulmonary venous obstruction was required in 22 patients (10%). Risk factors for reoperation were prolonged cardiopulmonary bypass time (p = 0.015), lower operative weight (p = 0.003), and an episode of postoperative pulmonary hypertensive crisis (p = 0.005). Freedom from reoperation at 20 years was 86% ± 3.2% (95% confidence interval, 78% to 91%). All survivors were asymptomatic at a mean of 13 ± 9 years (range, 1 month to 42 years) after the operation.

CONCLUSIONS

Although isolated TAPVD repair in infants can be performed without death, the operation is associated with a high mortality rate in neonates that remained unchanged during the long study period. Survival beyond 1 year after the operation is associated with excellent long-term outcomes.