Congenital heart disease

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 JPublished Retrospective cohort study

Study context

Population
Patients who underwent the Fontan procedure for single ventricle congenital heart defects (CHD) between 1 and 21 years of age, in the Pediatric Cardiac Care Consortium (PCCC) registry1,366 in long-term cohort
Participants
1,461 patients
Outcome
In-hospital Fontan failure (death or takedown) and post-discharge mortality
Follow-up
Through 2022
Methodological detailsrationale, design and baseline characteristics, multicentre, US registry-based, Median age at Fontan evaluation 3.1 years (IQR 2.4-4.3); males 62.1%
  • Rationale, design and baseline characteristics
  • Multicentre
  • US registry-based
  • Median age at Fontan evaluation 3.1 years (IQR 2.4-4.3); males 62.1%

Main finding

As reported in the abstract

In-hospital Fontan failure and post-discharge mortality
In-hospital Fontan failure, n
95
In-hospital deaths, n
78
Fontan takedown, n
17
Survived to discharge with Fontan physiology
1366 (93.5%)
Post-discharge deaths, n (median follow-up 21.2 years, IQR 18.4-24.5)
184

Observational data: associations, not effects.

Further findings

30-year post-Fontan survival

All Fontan types
75.0% (95% CI: 72.3%-77.8%)
Lateral tunnel and extracardiac conduit
77.1% (95% CI: 73.5-80.8)

Original publication

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. Published 26 April 2024. doi:10.1016/j.ahj.2024.04.012

Summarised from the published abstract; every number is checked against it

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