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. 2025 Jun 24;85(24):2386-2398.
doi: 10.1016/j.jacc.2025.04.028.

Long-Term Survival and Patient-Reported Outcomes After Staged Reconstructive Surgery for Hypoplastic Left Heart Syndrome

J William Gaynor  1 , Marlene Mahle  2 , Richard F Ittenbach  3 , Michelle Kaplinski  4 , Kendall M Lawrence  5 , Mallory L Hunt  6 , Nancy B Burnham  2 , Stephanie Fuller  2 , Jonathan M Chen  2 , Katsuhide Maeda  2 , Constantine D Mavroudis  2 , Muhammad A K Nuri  2 , Matthew J O'Connor  7 , Kathryn M Dodds  7 , Monique M Gardner  8 , David J Goldberg  7 , Chitra Ravishankar  7 , Jack Rychik  7 , Yuli Y Kim  7 , Lyla Hampton  9 , Christopher E Mascio  10 , Thomas L Spray  2 , Susan C Nicolson  11
Affiliations

Long-Term Survival and Patient-Reported Outcomes After Staged Reconstructive Surgery for Hypoplastic Left Heart Syndrome

J William Gaynor et al. J Am Coll Cardiol. .

Abstract

Background: Survival for hypoplastic left heart syndrome (HLHS) and variants has improved over the past 4 decades; however, survival remains low compared with other forms of congenital heart disease. There is a paucity of data concerning long-term outcomes.

Objectives: This study aims to: 1) examine long-term survival and the impact of patient factors on survival for newborns with HLHS; and 2) examine functional and health outcomes, including quality-of-life (QOL) in adulthood.

Methods: The study cohort included patients with HLHS and variants undergoing the Norwood or hybrid procedure between January 1984 and December 2023. Data on patient characteristics and management were abstracted from medical records. Vital status was ascertained by direct subject and family contact, review of the medical record, and publicly available data. Functional outcomes and QOL in adults (≥18 years of age) were assessed by patient reports. The primary outcome was death or cardiac transplantation at last follow-up.

Results: In the study period, 2,012 neonates underwent staged reconstructive surgery for HLHS (Norwood, n = 1,921 and hybrid, n = 91). Transplant-free survival was 31.0% at 35 years. Transplant-free survival improved over time but is not different across recent eras. Most responders reported good to excellent general health.

Conclusions: In this cohort of newborns undergoing staged reconstructive surgery for HLHS, fewer than one-third are alive without a transplant at 35 years of age. Survival has not improved in recent years. However, there is a group of survivors who report good to excellent outcomes and QOL, consistent with a "high-performing" Fontan phenotype.

Keywords: Norwood procedure; adulthood; hybrid procedure; hypoplastic left heart syndrome; quality of life; survival.

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Conflict of interest statement

Funding Support and Author Disclosures This project was funded by a grant from Big Hearts to Little Hearts, the Mortimer J. Buckley Jr, MD, Endowed Chair in Cardiac Surgery; the Thomas L. Spray, MD, Endowed Chair in Pediatric Cardiothoracic Surgery; and the Daniel M. Tabas Endowed Chair in Pediatric Cardiothoracic Surgery at the Children’s Hospital of Philadelphia. The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

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