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

• Published in: Journal of the American College of Cardiology Vol. 85; no. 24; pp. 2386 - 2398
• Main Authors: Gaynor, J. William, Mahle, Marlene, Ittenbach, Richard F., Kaplinski, Michelle, Lawrence, Kendall M., Hunt, Mallory L., Burnham, Nancy B., Fuller, Stephanie, Chen, Jonathan M., Maeda, Katsuhide, Mavroudis, Constantine D., Nuri, Muhammad A.K., O’Connor, Matthew J., Dodds, Kathryn M., Gardner, Monique M., Goldberg, David J., Ravishankar, Chitra, Rychik, Jack, Kim, Yuli Y., Hampton, Lyla, Mascio, Christopher E., Spray, Thomas L., Nicolson, Susan C.
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 24.06.2025
• Subjects: Adolescent; Adult; adulthood; Cardiovascular; Female; Follow-Up Studies; Humans; hybrid procedure; hypoplastic left heart syndrome; Hypoplastic Left Heart Syndrome - mortality; Hypoplastic Left Heart Syndrome - surgery; Infant, Newborn; Male; Norwood procedure; Norwood Procedures - methods; Patient Reported Outcome Measures; Plastic Surgery Procedures - methods; Quality of Life; Retrospective Studies; survival; Survival Rate - trends; Young Adult; MBTTS; hypoplastic left heart syndrome; survival; PLE; adulthood; HLHS; QOL; Norwood procedure; PENN; PB; RVPA; AV; CHOP; quality of life; WHOQOL-BREF; CHD; hybrid procedure; University of Pennsylvania; congenital heart disease; The Children’s Hospital of Philadelphia; World Health Organization Quality of Life Assessment; protein-losing enteropathy; atrioventricular; plastic bronchitis; modified Blalock-Taussig-Thomas shunt; right ventricle to pulmonary artery conduit
• ISSN: 0735-1097; 1558-3597; 1558-3597
• DOI: 10.1016/j.jacc.2025.04.028

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. 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. 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. 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. 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. [Display omitted]