Contemporary Management and Future Directions of Adults Living With a Fontan Circulation
Abstract
As 30-year survival after Fontan nears 90%, clinicians increasingly encounter Fontan circulatory failure. This panel introduces a pragmatic Fontan circulation function grading framework with red-flag triggers to prompt timely reassessment. Pathophysiology and management of key drivers and consequences of Fontan circulatory failure 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.
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