Contemporary Management and Future Directions of Adults Living With a Fontan Circulation (JACC: Advances July 2026)-Article: Contemporary Management and Future Directions of Adults Living With a Fontan Circulation

Pdf Summary

This review summarizes contemporary care for adults living with a Fontan circulation, a population now reaching high long-term survival but increasingly experiencing Fontan circulatory failure (FCF). The authors emphasize that FCF is a continuum rather than a binary state and propose a practical grading system with “red flags” to help clinicians detect worsening function earlier and trigger reassessment.<br /><br />The review covers the major drivers and consequences of FCF: ventricular systolic and diastolic dysfunction, arrhythmias, pulmonary vascular remodeling, lymphatic complications, thromboembolism, and Fontan pathway obstruction. Management options include pharmacologic therapy, catheter-based interventions, surgery, ventricular assist devices, and heart or combined heart-liver transplantation. Evidence for many drug therapies is limited and often extrapolated from biventricular heart failure, so individualized care is essential.<br /><br />Arrhythmias are common and may require rhythm control, ablation, anticoagulation, pacing, or selected use of defibrillators. Pulmonary vasodilators show mixed results, with some benefit in exercise measures but unclear long-term outcomes. Lymphatic disease, including protein-losing enteropathy and plastic bronchitis, is increasingly recognized; magnetic resonance lymphangiography and targeted lymphatic embolization are emerging tools. Thromboprophylaxis is recommended for all Fontan patients, with stronger anticoagulation for high-risk individuals.<br /><br />The paper also stresses comprehensive care beyond hemodynamics, including nutrition, bone and endocrine health, iron deficiency, mental health, neurocognition, and sexual and reproductive health. Pregnancy is high risk and requires specialized counseling. Exercise training is highlighted as safe and beneficial, though underused.<br /><br />Finally, the authors call for future progress through multicenter registries, standardized outcomes, invasive exercise testing, advanced imaging, biomarker development, and learning-health systems to enable earlier detection and more personalized treatment.

Meta Tag

Concept Fontan Procedure

Concept Reduced Ventricular Function

Concept Circulatory function continuum

Concept Arrhythmia

Concept Pulmonary Vascular Remodeling

Keywords

Fontan circulation

Fontan circulatory failure

adult congenital heart disease

arrhythmias

pulmonary vasodilators

lymphatic complications

thromboembolism

thromboprophylaxis

heart transplantation

exercise training

Fontan Procedure

Reduced Ventricular Function

Circulatory function continuum

Arrhythmia

Pulmonary Vascular Remodeling