Early and long-term outcomes following cardiac surgery for patients with Heterotaxy syndrome.

Objective Heterotaxy syndrome (HS) is a complex multisystem abnormality historically associated with high morbidity and mortality. We sought to evaluate the early and long-term outcomes following cardiac surgery in HS. Methods Single-Center retrospective review of HS patients undergoing single ventricle palliation (SV), primary or staged biventricular repair (BiV) from 1998-2018. Patients were stratified by SV vs. BiV, and by the severity of atrioventricular valve regurgitation (AVVR). Demographics, anatomic characteristics, early and late outcomes, including length of stay, mortality, surgical and/or catheter reinterventions, were analyzed. Results Among 250 patients, 150 (60%) underwent BiV repair. In-hospital mortality was 7.6% (n=19). Median follow-up was 5.2 (range 0 to 16) years. Among survivors to discharge, mortality was 19% (n=44), and reintervention was 52% (n= 120). Patients with moderate/severe AVVR were older (32 vs 16 months, p=.02), more likely to experience adverse events during their index surgical admission (72% vs 46%, p<.001) and had longer in-hospital LOS (20 vs 12 days, p=.009). Among patients with moderate to severe AVVR, SV palliation is associated with a greater risk of unplanned reintervention compared to patients undergoing BiV repair (HR = 2.13; CI:1.10 – 4.12; p=0.025). Conclusions There was no significant difference in early or late outcomes in SVP vs. BiV repair strategies in heterotaxy. In the subgroup of patients with moderate/severe AVVR, patients who underwent SVP were 2.5 times more likely to need a late reintervention compared to those undergoing BiV repair.