Pathophysiology of endothelial dysfunction in Fontan circulation: from bench to bedside and back again

IF 9.2 1区医学 Q1 PERIPHERAL VASCULAR DISEASE

Angiogenesis Pub Date : 2025-08-21 DOI:10.1007/s10456-025-09996-2

Amalia Baroutidou, Artemios G. Karagiannidis, Theodoros Dimitroulas, Vasileios Kamperidis, Antonios Ziakas, Konstantinos Dimopoulos, George Giannakoulas

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Abstract

The Fontan procedure is a definitive surgical approach for complex cardiac malformations, redirecting systemic venous blood into the pulmonary circulation through a staged repair that separates systemic and pulmonary venous returns in the absence of a subpulmonary ventricle. The ensuing unique hemodynamic conditions compromise the endothelial function both in the pulmonary and the systemic circulation. The underlying pathophysiological mechanisms, although distinct within each vascular bed, are interrelated and may collectively contribute to progressive end-organ dysfunction, ultimately accounting for the significant morbidity burden in Fontan patients. This review provides an overview of the current knowledge on the pathophysiology of pulmonary and systemic vasculopathy in Fontan circulation, with particular emphasis on the interplay between endothelial dysfunction and adverse clinical outcomes. Remaining gaps in knowledge and directions of future research are also discussed.

Graphical abstract

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方丹循环内皮功能障碍的病理生理：从工作台到床边再回来

Fontan手术是治疗复杂心脏畸形的一种确定的手术方法，通过分阶段修复将全身静脉血重新引导到肺循环中，在没有肺下心室的情况下分离全身静脉和肺静脉回流。随之而来的独特的血流动力学条件损害了肺循环和体循环的内皮功能。潜在的病理生理机制，虽然在每个血管床中都是不同的，但却是相互关联的，并可能共同导致终末器官功能障碍的进行性，最终导致Fontan患者的显著发病率负担。这篇综述综述了当前Fontan循环中肺和全身血管病变的病理生理学知识，特别强调了内皮功能障碍和不良临床结果之间的相互作用。讨论了知识上的空白和未来的研究方向。图形抽象

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来源期刊

Angiogenesis PERIPHERAL VASCULAR DISEASE-

CiteScore

21.90

自引率

8.20%

发文量

审稿时长

6-12 weeks

期刊介绍： Angiogenesis, a renowned international journal, seeks to publish high-quality original articles and reviews on the cellular and molecular mechanisms governing angiogenesis in both normal and pathological conditions. By serving as a primary platform for swift communication within the field of angiogenesis research, this multidisciplinary journal showcases pioneering experimental studies utilizing molecular techniques, in vitro methods, animal models, and clinical investigations into angiogenic diseases. Furthermore, Angiogenesis sheds light on cutting-edge therapeutic strategies for promoting or inhibiting angiogenesis, while also highlighting fresh markers and techniques for disease diagnosis and prognosis.