Outcomes of heart transplantation in children with heterotaxy syndrome.

Abstract

End-stage congenital heart disease in children with heterotaxy syndrome might necessitate heart transplantation. Heart transplantation in heterotaxy patients can be associated with several technical (e.g. redo, systemic/pulmonary-venous/situs anomalies, pulmonary artery reconstruction), and extra-cardiac (e.g. ciliary dyskinesia, infections, gastrointestinal) challenges. We aim to examine if heterotaxy syndrome is associated with increased early or late transplantation risks. The United Network for Organ Sharing transplantation database was merged with the Paediatric Health Information System administrative database to identify children with heterotaxy who received heart transplantation. Characteristics and outcomes were compared between children with heterotaxy and contemporaneous non-heterotaxy congenital and non-congenital cardiomyopathy control groups. After merger, our cohort of 1122 patients was divided to three groups: group heterotaxy (n = 143), group non-heterotaxy congenital (n = 428) and group cardiomyopathy (n = 551). There were differences in the characteristics between the 3 groups, with heterotaxy being comparable to non-heterotaxy congenital group. The waitlist duration was longer for heterotaxy than non-heterotaxy congenital and cardiomyopathy groups (91 vs 63 vs 56 days, p < 0.001). Early post-transplant complications were similar for all groups except for operative mortality that was 1% for cardiomyopathy and 4% for heterotaxy and non-heterotaxy congenital, p < 0.001. Post-transplant stay was shorter for cardiomyopathy (57) compared to non-heterotaxy congenital (99) and heterotaxy (89) days, p < 0.001. While rejection prior to discharge was comparable between heterotaxy and CHD groups, it was higher at 1-year for heterotaxy (22%), than non-heterotaxy congenital (19%) and cardiomyopathy (13%), p < 0.001. Survival at 5 years was superior for cardiomyopathy (87%) compared to heterotaxy (69%) and non-heterotaxy congenital (78%), p < 0.001. For the heterotaxy group, no risk factors affecting survival were identified on multivariable analysis. Despite complexity, heart transplantation in selected children with heterotaxy is associated with good mid-term outcomes. Despite early results that are comparable to other patients with congenital heart disease, the increasing rejection rate at one year and the relatively accelerated attrition at mid-term warrant further follow-up. Due to database limitations in defining morphologic and surgical details, further work is warranted to delineate anatomic and surgical variables that could affect survival.