Timing and Outcome of Superior Cavopulmonary Anastomosis in Patients with Heterotaxy Syndrome

Background: Patients with heterotaxy syndrome often have complex congenital heart disease requiring palliation involving a superior cavopulmonary anastomosis (SCPA). Due to the potential complexity of cardiac and extracardiac anomalies in this population, it is unknown whether SCPA can be performed in a similar manner between patients with and without heterotaxy syndrome.

Methods: Single-center retrospective chart review of patients who underwent SCPA from January 1, 2008 to October 31, 2019. The diagnosis of heterotaxy syndrome was established from imaging studies performed prior to intervention. Original cardiac catheterization reports, operative reports, and discharge summaries were used for data collection. Study data were collected and managed using REDCap electronic data capture tools and analysis was conducted in R (R Core Team, 2020). Data are presented as mean +/- SD and two-tailed unpaired t-tests were used for comparison between heterotaxy and non-heterotaxy patients with significance level of P < 0.05.

Results: Complete data was available on 23 heterotaxy patients and 113 non-heterotaxy patients who underwent SCPA during the study period. Pre-SCPA catheterization was performed at age 6.81 +/- 3.54 months in heterotaxy patients and 6.20 +/- 3.29 months in non-heterotaxy patients (P = 0.67). Pulmonary vascular resistance (indexed) at pre-SCPA catheterization was 2.06 +/- 1.34 WU*m^2 in heterotaxy patients and 2.03 +/- 0.82 WU*m^2 in non-heterotaxy patients (P = 0.58). SCPA was performed at age 8.42 +/- 3.41 months in heterotaxy patients and 7.84 +/- 4.32 months in non-heterotaxy patients (P = 0.54). There was one death in a heterotaxy patient and two deaths in non-heterotaxy patients prior to discharge.

Conclusion: Despite potentially higher complexity due to cardiac and extracardiac anomalies, SCPA can be performed safely in patients with heterotaxy syndrome at age ranges and pulmonary vascular resistance levels similar to non-heterotaxy patients requiring SCPA. These data can inform the management of this patient population.