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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.
Insights
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.
Abstract:
Single-ventricle physiology encompasses a heterogeneous spectrum of congenital cardiac malformations in which a single dominant ventricle supports both pulmonary and systemic circulation. Advances in staged surgical palliation culminating in the Fontan procedure have transformed survival, allowing most patients to reach adulthood. Consequently, general cardiologists increasingly encounter adults with Fontan circulation and its long-term cardiac and extracardiac complications. Although life-saving, Fontan circulation is a palliative physiology characterized by passive pulmonary blood flow, limited preload reserve, and chronic systemic venous hypertension. Over time, patients are at risk for progressive complications including arrhythmias, thromboembolism, ventricular dysfunction, Fontan pathway obstruction, lymphatic disorders including protein-losing enteropathy, hepatic disease, and reduced exercise capacity. This review provides general cardiologists with a practical, physiology-based overview of single-ventricle anatomy, the surgical pathway to Fontan circulation, common late complications, and principles of lifelong surveillance. Early recognition of deterioration and timely referral to specialized adult congenital heart disease centers are essential to improving outcomes in this growing patient population.
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