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Related Experiment Video

Updated: Jan 18, 2026

An Immunological Model for Heterotopic Heart and Cardiac Muscle Cell Transplantation in Rats
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Fontan circulation and transplantation: Considerations for the complex candidate.

Drishti Tolani1, Sharon Chen2, Edo Bedzra3

  • 1Department of Pediatric Cardiology, Cleveland Clinic Children's, Cleveland, OH.

JHLT Open
|September 11, 2025
PubMed
Summary
This summary is machine-generated.

The Fontan operation improves survival for single ventricle heart disease but can lead to Fontan circulatory failure (FCF). Early recognition and heart transplant evaluation are crucial for managing FCF and optimizing long-term outcomes.

Keywords:
Congenital heart diseaseFontan failureFontan-associated liver diseaseHeart transplantationLymphatic complicationsMechanical circulatory supportSingle ventricleVentricular assist device

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Area of Science:

  • Cardiology
  • Pediatric Cardiology
  • Congenital Heart Disease

Background:

  • The Fontan operation significantly improves survival in single ventricle congenital heart disease.
  • Long-term durability of the Fontan physiology is limited, leading to Fontan circulatory failure (FCF).
  • FCF is a progressive condition causing multiorgan dysfunction and increased post-transplant risk if not identified early.

Purpose of the Study:

  • To review the pathophysiology of Fontan failure.
  • To outline consensus indications for heart transplant evaluation in Fontan patients.
  • To highlight essential pre-transplant strategies for optimizing outcomes.

Main Methods:

  • Literature review synthesizing current evidence on Fontan failure.
  • Analysis of consensus guidelines for transplant referral.
  • Discussion of pre-transplant management strategies.

Main Results:

  • Fontan failure is characterized by multiorgan dysfunction and lymphatic issues.
  • Delayed referrals for advanced therapies are common, with patients often presenting in advanced decline.
  • Advances in surgical techniques and mechanical support have improved post-transplant survival.

Conclusions:

  • Heart transplantation is the definitive treatment for advanced Fontan failure.
  • Timely referral, equitable access, and comprehensive pre-transplant care are critical.
  • Multidisciplinary coordination and early recognition are key to improving outcomes for this growing population.