Heart Failure in Adults who had the Fontan Procedure: Natural History, Evaluation, and Management

Ari Cedars1, Susan Joseph, Philip Ludbrook

  • 1, 660 S. Euclid Ave, Campus Box 8086, Saint Louis, MO, 63110, USA, acedars@dom.wustl.edu.

Insights

Fontan palliations create an artificial circulation, but patients often develop heart failure over time. This review covers the natural history, evaluation, and management of heart failure in Fontan circulation survivors.

Area of Science:

  • Cardiology
  • Pediatric Cardiology
  • Adult Congenital Heart Disease

Background:

  • Fontan-type palliations are surgical procedures for single-ventricle physiology.
  • These operations create a series circulation, altering normal pulmonary and systemic blood flow.
  • The Fontan circulation, while life-sustaining, can lead to long-term complications.

Purpose of the Study:

  • To review the current understanding of heart failure in patients with Fontan circulation.
  • To summarize the natural history, evaluation, and management strategies for Fontan-associated heart failure.
  • To increase awareness among cardiologists and adult congenital heart disease specialists regarding Fontan sequelae.

Main Methods:

  • This is a review article.
  • It synthesizes existing literature on Fontan circulation and heart failure.
  • Data on natural history, evaluation, and management are summarized.

Main Results:

  • Fontan circulation supports patients for decades.
  • Progressive heart failure is a common long-term sequela.
  • Specific data on Fontan heart failure are limited but growing.

Conclusions:

  • Heart failure is an inevitable complication in many Fontan survivors.
  • Familiarity with Fontan circulation's long-term effects is crucial for adult congenital heart disease specialists.
  • Further research is needed to optimize management of heart failure in this population.
Abstract

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