Single Ventricle Fontan: The Basics for General Cardiologists

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.