The failing Fontan: etiology, diagnosis and management

David J Goldberg1, Robert E Shaddy, Chitra Ravishankar

  • 1Division of Cardiology, The Children's Hospital of Philadelphia, 34th and Civic Center Blvd Philadelphia, PA 19104, USA. goldbergda@email.chop.edu

Insights

The Fontan operation helps children with single ventricle heart defects survive but creates abnormal circulation. Ongoing research focuses on medical therapies to manage long-term Fontan complications and reduce transplant needs.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease
  • Cardiac Surgery

Background:

  • The Fontan operation is a palliative procedure for single-ventricle congenital heart disease.
  • While improving survival, it results in a non-physiological circulation with long-term risks.
  • Fontan survivors face complications including ventricular dysfunction, plastic bronchitis, and protein-losing enteropathy.

Purpose of the Study:

  • To review the long-term implications of Fontan physiology.
  • To discuss emerging therapies and future directions for managing Fontan survivors.
  • To highlight the limitations of current Fontan circulation and the need for improved treatments.

Main Methods:

  • Review of existing literature on Fontan operation outcomes.
  • Analysis of current and emerging medical and surgical strategies.
  • Discussion of ongoing research into mechanical support and novel therapeutics.

Main Results:

  • Fontan circulation, while life-saving, leads to significant long-term morbidities.
  • Medical therapies are being developed to address specific Fontan-associated complications.
  • Mechanical circulatory support devices are in early investigation stages.

Conclusions:

  • Continued development of targeted medical therapies is crucial for improving outcomes in Fontan survivors.
  • These therapies may potentially decrease the need for cardiac transplantation.
  • Further research is needed for advanced mechanical solutions, but medical management remains a key focus.

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