The Fontan procedure: anatomy, complications, and manifestations of failure

Tyler B Fredenburg1, Tiffanie R Johnson, Mervyn D Cohen

  • 1Department of Radiology and Imaging Sciences, Riley Hospital for Children, Indianapolis, IN 46202, USA.

Insights

The Fontan procedure reroutes blood flow to the lungs without a ventricle, offering palliative treatment for complex congenital heart defects. While effective in some, it carries risks like heart failure and organ complications, necessitating ongoing monitoring.

Area of Science:

  • Cardiovascular Surgery
  • Pediatric Cardiology
  • Congenital Heart Disease

Background:

  • The Fontan procedure is a surgical intervention for complex congenital heart defects, directing systemic venous blood to the pulmonary circulation without ventricular assistance.
  • It addresses conditions such as tricuspid atresia, pulmonary atresia with intact ventricular septum, hypoplastic left heart syndrome, and double-inlet ventricle.

Purpose of the Study:

  • To provide a comprehensive overview of the Fontan procedure, including its modifications, outcomes, and associated complications.
  • To highlight the palliative nature of Fontan circulation and its variable success based on patient hemodynamics.

Main Methods:

  • Review of existing literature and surgical techniques related to the Fontan procedure.
  • Analysis of complications and long-term sequelae associated with Fontan circulation.
  • Discussion of diagnostic modalities for postoperative evaluation, particularly magnetic resonance imaging.

Main Results:

  • Fontan circulation is palliative, with outcomes heavily dependent on preoperative hemodynamics.
  • Significant morbidity and mortality are observed in patients with suboptimal hemodynamics.
  • Common complications include exercise intolerance, ventricular dysfunction, arrhythmias, venous hypertension, and lymphatic abnormalities.

Conclusions:

  • The Fontan procedure is a critical palliative option for specific congenital heart diseases.
  • Long-term management requires vigilant monitoring for a spectrum of potential complications.
  • Cardiac transplantation is the definitive treatment for Fontan circulation failure.

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