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Published on: May 2, 2025
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.
Abstract:
The Fontan procedure refers to any operation that results in the flow of systemic venous blood to the lungs without passing through a ventricle. It is performed to treat several complex congenital heart abnormalities including tricuspid atresia, pulmonary atresia with intact ventricular septum, hypoplastic left heart syndrome, and double-inlet ventricle. The original Fontan procedure included direct anastomosis of the right atrium to the main pulmonary artery; however, multiple modifications have been employed. Creation of Fontan circulation is palliative in nature, with good results in patients with ideal hemodynamics and substantial morbidity and mortality in those with poor hemodynamics. Complications of Fontan circulation include exercise intolerance, ventricular failure, right atrium dilatation and arrhythmia, systemic and hepatic venous hypertension, portal hypertension, coagulopathy, pulmonary arteriovenous malformation, venovenous shunts, and lymphatic dysfunction (eg, ascites, edema, effusion, protein-losing enteropathy, and plastic bronchitis). Magnetic resonance imaging is best for postoperative evaluation of patients who underwent the Fontan procedure, and cardiac transplantation remains the only definitive treatment for those with failing Fontan circulation.
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