Improved outcomes after modified Stage-I palliation for hypoplastic left heart syndrome

Objective:To evaluate short- and long-term outcomes after modified Stage-I (mStage-I) palliation for hypoplastic left heart syndrome (HLHS).MethodsThis retrospective single-centre study analyzed 128 patients with HLHS treated between 1993 and 2023. Patients were divided into two groups: Era-I (n=36), from 1993 to 2001, who underwent the classic Norwood procedure, and Era-II (n=92), from 2002 to 2023, who underwent the mStage-I palliation. Postoperative outcomes, including the need for postoperative extracorporeal membrane oxygenation (ECMO), peritoneal dialysis (PD), recoarctation rate, and overall survival, were compared between eras.ResultsMedian follow-up was 9.7 years, with 100% completeness for survival data, and ranged from 0.9 to 30.9 years. Ninety-day survival improved from 69.4% in Era-I to 95.7% in Era-II (P<.001). Transplant-free survival improved from 47% to 84% at 5 years (P<.001) and from 42% to 71% at 20 years (P<.001). Only one patient in Era-II required postoperative ECMO. The use of PD decreased from 86.1% to 22.8%, and recoarctation rate decreased from 13.9% to 7.6%.ConclusionsOn the foundation of important developments in perioperative care across the study period, the introduction of modified Stage-I palliation may have contributed to improved survival and reduced postoperative morbidity, and may represent an alternative to centralization of care.