Abstract
Background: Hypoplastic left heart syndrome (HLHS) is a severe congenital heart defect characterized by the underdevelopment of the left-sided heart structures, necessitating staged univentricular palliation to repurpose the right ventricle as the systemic pumping chamber. While the Fontan procedure offers a well-established method to manage single-ventricle physiology and improve survival, the potential of fetal aortic valvuloplasty (FAV) to prevent the progression of critical aortic stenosis to HLHS and achieve biventricular circulation postnatally remains significant. However, the long-term outcomes of both interventions are still uncertain. Therefore this study aims to evaluate the survival and reverse progression outcome of fetal aortic valvuloplasty and extra-cardiac conduit or lateral tunnel Fontan procedure in evolving and evolved hypoplastic left heart syndrome Methods: The literature search was conducted using PubMed, ScienceDirect, and Google Scholar databases up to May 2024. The analyses in this study were performed using R statistical software v.3.3. Results: This analysis reviewed 25 prospective and retrospective cohort studies discussing the outcomes of the FAV procedure in evolving HLHS fetuses and the Fontan procedure in pediatric HLHS patients. Among evolving HLHS fetuses, the FAV procedure had a mortality rate of 0.18 (95% CI 0.07-0.29) and a procedural success rate of 0.81 (95% CI 0.78-0.85). In pediatric patients with evolved HLHS undergoing the Fontan procedure, the mortality rate was 0.13 (95% CI 0.00-0.25), and the procedural success rate was 0.93 (95% CI 0.85-1.00). Conclusion: The Fontan procedure has shown better results than FAV, likely due to interstage procedural interventions and more established techniques for reducing morbidity and mortality in HLHS patients.
Published Version
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