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Endothelial dysfunction in single ventricle physiology and the Fontan circulation – What lies ahead

Lookup NU author(s): Dr Louise Coats

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This work is licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0).


Abstract

© 2025 The Author(s)Endothelial dysfunction is characterized by a vasoconstricted, pro-coagulative, and pro-inflammatory phenotype and is known to play a role in several chronic non-communicable diseases. Several biophysical and biochemical markers have been developed to assess endothelial function clinically. Its relevance in individuals born with single-ventricle congenital heart disease (SV-CHD) is increasingly recognized. Endothelial dysfunction has been observed in all stages of palliation in SV-CHD patients. Several mechanisms possibly contribute, including genetic factors, hypoxia, loss of pulsatility of blood flow, neurohormonal and sympathetic overactivation, and oxidative stress. Clinically, it possibly contributes to impaired pulmonary flow, exercise limitation, thromboembolisms, liver dysfunction, and adverse pregnancy outcomes. Based on this information, several therapeutic targets have been proposed such as early surgical and exercise interventions, pulmonary vasodilators, and other common pharmacological agents. However, much remains unknown and future studies must unravel the relationship of endothelial dysfunction with this complex patient group, ultimately improving their clinical care.


Publication metadata

Author(s): Agarwal R, Coats LE, Zaidi AN

Publication type: Review

Publication status: Published

Journal: International Journal of Cardiology Congenital Heart Disease

Year: 2025

Volume: 20

Print publication date: 01/06/2025

Online publication date: 28/04/2025

Acceptance date: 26/04/2025

ISSN (electronic): 2666-6685

Publisher: Elsevier B.V.

URL: https://doi.org/10.1016/j.ijcchd.2025.100589

DOI: 10.1016/j.ijcchd.2025.100589

Data Access Statement: No data was generated for this review article.


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