The Fontan circulation underpins a venous-return–limited systemic circuit with broad organ and functional consequences. Research patterns converge on diastolic dysfunction, systemic venous return constraints, reduced exercise capacity, and hepatic remodeling across Fontan variants and conversions. Imaging-driven characterization of right ventricular function, relying on magnetic resonance imaging and echocardiography, informs diagnosis, prognosis, and management, while guidelines strive to harmonize right-heart assessment in adults with single-ventricle physiology.
Fontan physiology underpins a venous-return–limited systemic circulation with broad organ and functional consequences. Research patterns converge on diastolic dysfunction, systemic venous return constraints, and reduced exercise capacity, alongside hepatic remodeling and adaptation across Fontan variants and conversions.
Imaging-driven characterization of right ventricular function in CHD relies on complementary MRI and echocardiography, with validations across TOF and Fontan anatomies guiding diagnosis, prognosis, and management.
Imaging guidelines and appropriateness criteria for CHD imaging are coalescing into standard protocols for right-heart assessment in adults and single-ventricle physiology, aiming to harmonize acquisition, interpretation, and modality selection.
Therapeutic strategies for univentricular hearts include pharmacologic management of systemic ventricular dysfunction and mechanical support via ventricular assist devices in children, reflecting a continuum of care from medical to device-based therapies.
Biomarkers and functional correlates of Fontan/univentricular function are explored to detect early circuit dysfunction and relate structural features to exercise capacity, with candidates such as growth differentiation factor 15 (GDF-15) emerging as early indicators.
Influential works
Key work identified Fontan hemodynamics as the central determinant of exercise tolerance and long-term outcomes, connecting systemic venous return, pulmonary vascular resistance, and diastolic function with patient function and risk. Biomarkers such as Growth differentiation factor 15 emerged as early indicators of Fontan circuit dysfunction, catalyzing biomarker-guided surveillance. Pioneering pediatric ventricular assist device experiences and recognition of Fontan-related hepatic fibrosis shaped surveillance strategies and multidisciplinary care, while standardized right-heart imaging guidelines provided foundational tools for clinical practice and research.
Relation of Systemic Venous Return, Pulmonary Vascular Resistance, and Diastolic Dysfunction to Exercise Capacity in Patients With Single Ventricle Receiving Fontan Palliation demonstrated how Fontan hemodynamics—systemic venous return, pulmonary vascular resistance, and diastolic function—limit exercise capacity, guiding clinical assessment, risk stratification, and future hemodynamic optimization strategies.
Growth differentiation factor 15—an early marker of abnormal function of the Fontan circuit in patients with univentricular hearts identified GDF-15 as an early circulating biomarker of Fontan circuit dysfunction, enabling earlier detection and catalyzing biomarker-driven surveillance and mechanistic studies of Fontan failure.
The use of ventricular assist devices in pediatric patients with univentricular hearts provides early pediatric VAD experience, outlining feasibility, patient selection, and bridging strategies, setting the stage for subsequent development of pediatric mechanical supports and informing later Fontan-related support research.
Liver Disease After the Fontan Procedure documents the high risk of hepatic fibrosis and cirrhosis in long-term Fontan survivors, highlighting pathophysiology, clinical consequences, and the need for multidisciplinary surveillance, thus shaping long-term care guidelines for Fontan patients.
Guidelines for the Echocardiographic Assessment of the Right Heart in Adults: A Report from the American Society of Echocardiography provides comprehensive, standardized methods for evaluating right ventricular size and function—critical for single-ventricle patients—impacting both immediate clinical practice and long-term research.