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The Fontan circulation: Arrhythmia is just one price we have to pay

Jack Rychik1,2

  • 1Fontan FORWARD Program, Children's Hospital of Philadelphia, Philadelphia, PA, USA.

Annals of Pediatric Cardiology
|July 25, 2026
PubMed

Insights

The Fontan circulation improves survival for single-ventricle heart disease patients. While extracardiac Fontan may lower arrhythmia risk, long-term benefits and other complications should guide pathway choice.

Area of Science:

  • Cardiology
  • Pediatric Cardiology
  • Congenital Heart Disease

Background:

  • The Fontan circulation enables survival for patients with single-ventricle congenital heart disease into adulthood.
  • Despite improved survival, Fontan physiology presents long-term complications, notably arrhythmia.
  • Arrhythmia remains a significant concern in the Fontan population.

Purpose of the Study:

  • To discuss findings comparing arrhythmia outcomes after extracardiac vs. intracardiac Fontan completion.
  • To contextualize arrhythmia risk within the broader Fontan physiology.
  • To emphasize comprehensive long-term outcome assessment for Fontan pathway selection.

Main Methods:

  • Systematic review and meta-analysis of arrhythmia outcomes.
  • Comparative analysis of extracardiac and intracardiac Fontan completion.
  • Discussion within the context of Fontan physiology and associated complications.

Main Results:

  • Extracardiac Fontan appears associated with a lower risk of arrhythmia compared to intracardiac Fontan.
  • The clinical significance of specific rhythm disturbances and their impact on overall outcomes requires careful interpretation.
  • Other Fontan-associated issues like exercise limitation, hepatic problems, and quality of life are critical considerations.

Conclusions:

  • Fontan pathway choice should prioritize overall long-term benefits over arrhythmia risk alone.
  • Further robust longitudinal data and collaborative registries are essential for informed decision-making.
  • A holistic approach considering all complications is necessary for optimal Fontan patient management.

Keywords:
ArrhythmiaFontan circulationlong-term outcomessingle-ventricle physiology

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