Association of patient-level characteristics with long-term outcomes after Fontan palliation: Rationale, design, and baseline characteristics of the Pediatric Cardiac Care Consortium Fontan cohort study

Yanxu Yang; Tzu-Chun Chu; Divya Suthar; Asaad G Beshish; Matthew Oster; Alvaro Alonso; Yijian Huang; Gourav Modanwal; Lazaros Kochilas; Jessica H Knight

doi:10.1016/j.ahj.2024.04.012

Association of patient-level characteristics with long-term outcomes after Fontan palliation: Rationale, design, and baseline characteristics of the Pediatric Cardiac Care Consortium Fontan cohort study

Am Heart J. 2024 Apr 25:S0002-8703(24)00101-7. doi: 10.1016/j.ahj.2024.04.012. Online ahead of print.

Authors

Affiliations

¹ Department of Pediatrics, Emory University School of Medicine, Atlanta, GA.
² Department of Epidemiology and Biostatistics, University of Georgia College of Public Health, Athens, GA.
³ Department of Pediatrics, Emory University School of Medicine, Atlanta, GA; Children's Healthcare of Atlanta Cardiology, Atlanta, GA.
⁴ Department of Epidemiology, Rollins School of Public Health, Emory University. Atlanta, GA.
⁵ Department of Biostatistics and Bioinformatics, Rollins School of Public Health of Emory University, Atlanta, GA.
⁶ Wallace H. Coulter Department of Biomedical Engineering, Georgia Institute of Technology, and Emory University School of Medicine, Atlanta, GA.
⁷ Department of Pediatrics, Emory University School of Medicine, Atlanta, GA; Children's Healthcare of Atlanta Cardiology, Atlanta, GA. Electronic address: Lazaros.Kochilas@emory.edu.

PMID: 38677504
DOI: 10.1016/j.ahj.2024.04.012

Abstract

Background: The Fontan operation is used to palliate single ventricle congenital heart defects (CHD) but poses significant morbidity and mortality risks. We present the design, planned analyses and rationale for a long-term Fontan cohort study aiming to examine the association of patient characteristics at the time of Fontan with post-Fontan morbidity and mortality.

Methods and results: We used the Pediatric Cardiac Care Consortium (PCCC), a US-based, multicenter registry of pediatric cardiac surgeries to identify patients who underwent the Fontan procedure for single ventricle CHD between 1 and 21 years of age. The primary outcomes are in-hospital Fontan failure (death or takedown) and post-discharge mortality through 2022. A total of 1461 (males 62.1%) patients met eligibility criteria and were included in the analytical cohort. The median age at Fontan evaluation was 3.1 years (IQR: 2.4-4.3). While 95 patients experienced in-hospital Fontan failure (78 deaths and 17 Fontan takedown), 1366 (93.5%) survived to discharge with Fontan physiology and formed the long-term analysis cohort. Over a median follow-up of 21.2 years (IQR: 18.4-24.5) 184 post-discharge deaths occurred. Thirty-year post Fontan survival was 75.0% (95% CI: 72.3-77.8%) for all Fontan types with higher rates for current techniques such as lateral tunnel and extracardiac conduit 77.1% (95% CI: 73.5-80.8).

Conclusion: The PCCC Fontan study aims to identify predictors for post-Fontan morbidity and mortality, enabling risk- stratification and informing surveillance practices. Additionally, the study may guide therapeutic interventions aiming to optimize hemodynamics and enhance Fontan longevity for individual patients.