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

To evaluate short- and long-term outcomes after modified Stage I palliation for hypoplastic left heart syndrome.

This retrospective single-center study analyzed 128 patients with hypoplastic left heart syndrome treated between 1993 and 2023. Patients were divided into 2 groups: Era I (n = 36), from 1993 to 2001, included patients who underwent the classic Norwood procedure, and Era II (n = 92), from 2002 to 2023, included patients who underwent modified Stage I palliation. Postoperative outcomes, including the need for postoperative extracorporeal membrane oxygenation, peritoneal dialysis, recoarctation rate, and overall survival, were compared between eras.

Median 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 1 patient in Era II required postoperative extracorporeal membrane oxygenation. The use of peritoneal dialysis decreased from 86.1% to 22.8%, and recoarctation rate decreased from 13.9% to 7.6%.

On 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.