• J. Thorac. Cardiovasc. Surg. · Jul 2024

    Long-Term Impact of Anatomic Subtype in Hypoplastic Left Heart Syndrome after Fontan Completion.

    • Jiyong Moon, Timothy Lancaster, Vikram Sood, Ming-Sing Si, Richard G Ohye, and Jennifer C Romano.
    • Section of Section of Pediatric Cardiac Surgery, Department of Cardiac Surgery, University of Michigan Health System, C.S. Mott Children's Hospital, Ann Arbor, Mich. Electronic address: moonji@med.umich.edu.
    • J. Thorac. Cardiovasc. Surg. 2024 Jul 1; 168 (1): 193201.e3193-201.e3.

    BackgroundAortic atresia (AA)/mitral stenosis (MS) is a well-known risk factor for survival after Norwood; however, the effect of anatomical subtypes in those who survive surgical palliation is unknown.MethodsWe performed a retrospective single-center study of patients with classic hypoplastic left heart syndrome (HLHS) who underwent Fontan at our center between August 1989 and July 2017. Clinical outcomes, as well as ventricular and atrioventricular-valve (AVV) function, were determined for each patient, and the effects of HLHS subtype were estimated using multivariable statistical analyses.ResultsWe included 418 patients with HLHS (AA/mitral atresia [MA] 153, AA/MS 100, aortic stenosis [AS]/MS 154, and AS/MA 11). The median follow-up period was 8.6 (interquartile range, 2.9-15.8) years. Overall transplant-free survival, cumulative incidence of AVV failure, and ventricular failure, which were defined by moderate dysfunction or greater or the necessity of surgical interventions, were 70.1%, 35.9%, and 17.9% at 20 years, respectively. Of the 3 major subtypes, AA/MS was associated with lower survival rate (AA/MA 74.6% vs AS/MS 79.1% vs AA/MS 56.1% at 17 years, P = .04). The subanalysis between AA/MA and AA/MS revealed AA/MS tended to have a greater rate of ventricular failure without a significant difference of AVV failure (AA/MA 11.2% vs AA/MS 26.2% at 17 years, P = .053).ConclusionsThe survival risk of the anatomic subtype AA/MS persisted long term after Fontan completion and was likely due to a greater rate of single ventricle rather than AVV failure. These findings suggest that the abnormal pressure overload condition of the hypoplastic left ventricle created by AA/MS has a detrimental effect on single right ventricle function.Copyright © 2023 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

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