Long-Term Follow-Up in Patients with Modified Fontan: Assessing Outcomes, Complications, and Management Strategies for Single-Ventricle Survivors

The modified Fontan procedure, a pivotal surgical intervention for patients with single-ventricle congenital heart defects, has drastically improved survival rates and quality of life for individuals who would otherwise face limited options. Despite these advancements, patients with modified Fontan physiology experience a unique set of hemodynamic challenges that require vigilant long-term follow-up. The Fontan circulation, characterized by passive venous return to the pulmonary arteries without a ventricular pump, predisposes patients to a variety of late complications, including Fontan-associated liver disease (FALD), arrhythmias, protein-losing enteropathy (PLE), thromboembolic events, and systemic venous hypertension. This review aims to evaluate the outcomes and long-term complications associated with modified Fontan patients, emphasizing the importance of early detection, multidisciplinary care, and individualized management strategies. The article explores current evidence on surveillance protocols, pharmacological treatments, and surgical interventions, as well as emerging therapeutic options like Fontan conversion, catheter-based therapies, and heart transplantation. This comprehensive analysis seeks to provide healthcare providers with a roadmap for optimizing care and improving the long-term prognosis of these patients.