Hemodynamic and Clinical Implications of Impaired Pulmonary Vascular Reserve in the Fontan Circulation.

ABSTRACT:

Background

Pulmonary vascular disease, pulmonary endothelial dysfunction, liver fibrosis, renal disease, and exercise intolerance are common in adults with Fontan physiology. Although the pathophysiologic mechanisms linking these phenomena have been studied, certain aspects are not well understood.

Objectives

This study hypothesized that impaired pulmonary vascular reserve (VR) plays a central role linking these abnormalities, and that patients with abnormal pulmonary VR with exercise, compared with patients with normal VR, would display poorer pulmonary endothelial function, greater liver stiffness, more renal dysfunction, and poorer exercise capacity.

Methods

Symptomatic adults with the Fontan palliation (n = 29) underwent invasive cardiopulmonary exercise testing,

SUBMITTER: Egbe AC 

PROVIDER: S-EPMC7720935 | biostudies-literature | 2020 Dec

REPOSITORIES: biostudies-literature