Management and outcomes of heterotaxy syndrome associated with pulmonary atresia or pulmonary stenosis.

Background: Historic outcomes of patients with heterotaxy and pulmonary atresia or pulmonary stenosis (PA/PS) have been poor and in the current era are incompletely described. We reviewed our management of these patients and associated risk factors for death.

Methods: We retrospectively reviewed the records of all patients with heterotaxy and PA/PS treated in our institution from January 1, 2002, to August 31, 2012. Death data were also confirmed with the Social Security Death Index. The log-rank test was done to assess six risk factors for death.

Results: We identified 42 patients with heterotaxy and PA/PS. Median age at the first operation was 6.5 days, and median follow-up was 3.5 years. Death data were complete for all patients. Overall mortality was 19% (8 of 42). The 30-day, 1-year, and 5-year mortality estimation was 4.76%, 12.3%, and 19.1% respectively, as determined by the Kaplan-Meier method. The log-rank test showed total anomalous pulmonary venous return (TAPVR) (p<0.05) and obstructed TAPVR requiring an operation at less than 30 days (p=0.001) were significant risk factors for death.

Conclusions: In the current era, surgical treatment of heterotaxy and PA/PS can result in good outcomes. Associated TAPVR and obstructed TAPVR requiring neonatal correction were noted to be risk factors for death.