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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.
Yang, Yanxu; Chu, Tzu-Chun; Suthar, Divya; Beshish, Asaad G; Oster, Matthew E; Alonso, Alvaro; Huang, Yijian; Modanwal, Gourav; Kochilas, Lazaros K; Knight, Jessica H.
Afiliación
  • Yang Y; Department of Pediatrics, Emory University School of Medicine, Atlanta, GA.
  • Chu TC; Department of Epidemiology and Biostatistics, University of Georgia College of Public Health, Athens, GA.
  • Suthar D; Department of Pediatrics, Emory University School of Medicine, Atlanta, GA; Children's Healthcare of Atlanta Cardiology, Atlanta, GA.
  • Beshish AG; Department of Pediatrics, Emory University School of Medicine, Atlanta, GA; Children's Healthcare of Atlanta Cardiology, Atlanta, GA.
  • Oster ME; Department of Pediatrics, Emory University School of Medicine, Atlanta, GA; Children's Healthcare of Atlanta Cardiology, Atlanta, GA.
  • Alonso A; Department of Epidemiology, Rollins School of Public Health, Emory University, Atlanta, GA.
  • Huang Y; Department of Biostatistics and Bioinformatics, Rollins School of Public Health of Emory University, Atlanta, GA.
  • Modanwal G; Wallace H. Coulter Department of Biomedical Engineering, Georgia Institute of Technology, and Emory University School of Medicine, Atlanta, GA.
  • Kochilas LK; Department of Pediatrics, Emory University School of Medicine, Atlanta, GA; Children's Healthcare of Atlanta Cardiology, Atlanta, GA. Electronic address: Lazaros.Kochilas@emory.edu.
  • Knight JH; Department of Epidemiology and Biostatistics, University of Georgia College of Public Health, Athens, GA.
Am Heart J ; 273: 111-120, 2024 07.
Article en En | MEDLINE | ID: mdl-38677504
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.
Asunto(s)

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Sistema de Registros / Procedimiento de Fontan / Cardiopatías Congénitas Límite: Adolescent / Adult / Child / Child, preschool / Female / Humans / Infant / Male País/Región como asunto: America do norte Idioma: En Revista: Am Heart J Año: 2024 Tipo del documento: Article Pais de publicación: Estados Unidos

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Sistema de Registros / Procedimiento de Fontan / Cardiopatías Congénitas Límite: Adolescent / Adult / Child / Child, preschool / Female / Humans / Infant / Male País/Región como asunto: America do norte Idioma: En Revista: Am Heart J Año: 2024 Tipo del documento: Article Pais de publicación: Estados Unidos