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Single Ventricle Fontan: The Basics for General Cardiologists
Berardo Sarubbi1, Flavia Fusco1, Michela Palma1
1Adult Congenital Heart Disease Unit, Monaldi Hospital, Naples, Italy.
Methodist Debakey Cardiovascular Journal
|July 6, 2026
Summary
Adults with single-ventricle physiology and Fontan circulation face lifelong risks of cardiac and extracardiac complications. Early recognition and specialized care are crucial for managing these complex Fontan-associated conditions.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Pediatric Cardiology
Background:
- Single-ventricle physiology presents complex congenital heart defects.
- Staged surgical palliation, including the Fontan procedure, has improved survival to adulthood.
- General cardiologists now frequently manage adults with Fontan circulation.
Purpose of the Study:
- To provide a physiology-based overview of single-ventricle anatomy and the Fontan pathway.
- To review common long-term cardiac and extracardiac complications of Fontan circulation.
- To outline principles for lifelong surveillance in adults with Fontan physiology.
Main Methods:
- Review of current literature on single-ventricle physiology and Fontan circulation.
- Physiology-based approach to understanding Fontan-associated complications.
- Synthesis of guidelines for surveillance and management.
Main Results:
- Fontan circulation is palliative, leading to chronic venous hypertension and limited reserve.
- Common complications include arrhythmias, thromboembolism, ventricular dysfunction, and organ-specific diseases.
- Lifelong surveillance is essential for early detection of deterioration.
Conclusions:
- Adults with Fontan circulation require specialized lifelong care.
- Timely referral to adult congenital heart disease centers improves outcomes.
- Understanding Fontan physiology is key to managing long-term risks.
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