Why the study?
The Fontan circulation imposes a lifelong hemodynamic burden and predisposes to cardiovascular dysfunction and multisystem morbidity, yet evidence-based guidance for assessing and managing Fontan-specific cardiac and vascular dysfunction remains limited.
This international consensus statement establishes structured, phenotype-guided clinical recommendations for assessing and managing cardiovascular and multisystem dysfunction in patients with a Fontan circulation.
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Phenotype-guided pathways may now direct Fontan care; leaves key gaps open for multicenter trials.
The Fontan operation enables long term survival for many individuals born with single ventricle heart disease, but the resulting circulation imposes a lifelong hemodynamic burden and predisposes to cardiovascular dysfunction and multisystem morbidity. Evidence based guidance for assessment and management of Fontan specific cardiac and vascular dysfunction, however, remains limited. The European Pediatric Pulmonary Vascular Disease Network (EPPVDN) convened an international, multidisciplinary executive writing group to review the available literature and develop consensus recommendations addressing cardiovascular dysfunction in children and young adults with a Fontan circulation. Recommendations were graded using established Class of Recommendation and Level of Evidence criteria adapted to the Fontan specific evidence base. The statement addresses cardiovascular assessment, pulmonary vascular disease and use of pulmonary vasodilator therapy, Fontan pathway obstruction, systemic venous and arterial abnormalities and collateral vessels, lymphatic dysfunction and intervention, myocardial dysfunction and heart failure therapy, and thromboembolic risk and antithrombotic therapy. The focus is on phenotype-guided management, hemodynamically informed treatment decisions, and medical, transcatheter, and surgical approaches tailored to Fontan circulation. Seven graded recommendation tables as well as a flowchart for considerations regarding pulmonary vasodilator therapy are provided to support clinical decision making. Fontan-specific evidence remains limited, so many recommendations necessarily represent expert opinion. This EPPVDN consensus statement, therefore, discusses knowledge gaps and priorities for collaborative research to improve outcomes for individuals living with a Fontan circulation.
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Opotowsky et al. (2026) studied Children and young adults with a Fontan circulation and cardiovascular dysfunction. Phenotype-guided management, pulmonary vasodilators, heart failure therapy, antithrombotic therapy, and transcatheter/surgical interventions was evaluated. The EPPVDN consensus statement provides 7 graded recommendation tables and a pulmonary vasodilator therapy flowchart for managing Fontan cardiovascular dysfunction.
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