As 30-year survival after Fontan nears 90%, clinicians increasingly encounter Fontan circulatory failure (FCF). This panel introduces a pragmatic FCF continuum grading framework with red-flag triggers to prompt timely reassessment. Pathophysiology and management of key drivers and consequences of FCF are outlined, including ventricular dysfunction/heart failure, arrhythmias, pulmonary vascular remodeling, lymphatic dysfunction, thromboembolism, and pathway obstruction. Considerations for pharmacologic, catheter-based, surgical, and transplant strategies are outlined. Comprehensive care is summarized across nutrition, endocrine-metabolic and bone health, mental health and neurocognition, and sexual and reproductive health, including pregnancy risk. Future directions emphasize multicenter registries and learning-health systems, standardized outcome measures, invasive exercise hemodynamics, advanced imaging and biomarker programs. We highlight emerging therapies, such as image-guided lymphatic interventions, cavopulmonary assist devices, optimized heart±liver transplantation, and targeted/repurposed pharmacotherapy.
JACC: Advances Editor-in-Chief
Candice K. Silversides, MD, FACC
JACC: Advances CME Editor
Kenneth A. Ellenbogen, MD
Author
Jasmine Grewal, MD
Important Dates
Date of Release: July 22, 2026
Term of Approval/Date of CME/MOC Expiration: July 21, 2027