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Non-Invasive Assessment of Endothelial Dysfunction and Arterial Stiffness in Fontan Circulation

Baroutidou A, Pagkopoulou E, Kamperidis V, Ziakas A, Dimitroulas T, Giannakoulas G

European journal of clinical investigation, 2026

Abstract

Background: The Fontan procedure constitutes the definitive surgical strategy for complex congenital heart disease, establishing a circulation in which systemic venous blood is directed to the pulmonary arteries through a staged reconstruction that separates systemic and pulmonary venous return in the absence of a subpulmonary ventricle. This uniquely altered haemodynamic state exerts deleterious effects on endothelial function, thereby promoting progressive end-organ dysfunction and contributing substantially to the long-term morbidity observed in patients with Fontan circulation. ΜETHODS/RESULTS: Over recent decades, clinical research has employed a range of functional methods to assess macro- and microvascular function, as well as arterial stiffness. This review summarizes the available functional non-invasive techniques for evaluating vascular function in the Fontan circulation and discusses the existing evidence linking these vascular parameters to cardiac dysfunction, associated comorbidities, and clinical outcomes in this population.

Conclusions: Functional non-invasive vascular assessment may provide valuable information for monitoring disease progression and risk stratification in Fontan circulation; however, further longitudinal studies are required to clarify its prognostic significance and potential clinical utility.

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