Widespread endotheliopathy in adults with cyanotic congenital heart disease

Abstract

Endothelial function is impaired in the systemic arteries and retinal vessels in adults with cyanotic congenital heart disease, suggesting a widespread endotheliopathy. Diminished numbers of endothelial progenitor cells might potentially contribute to these observations.

Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association

“…many diagnoses carry clinical manifestations of arterial vascular disease, the subclinical assessment of the pathophysiology is revealing unique abnormalities in many of the seemingly heterogeneous congenital diagnoses. [396][397][398] To date, CoA is the best-studied vascular phenotype and presents the panoply of challenges in vascular assessment and management. Individuals with CoA have endothelial dysfunction that persists despite successful repair in many individuals, including those without hypertension.…”

Arterial stiffness and arterial function in adult cyanotic patients with congenital heart disease

“…mediates vascular tone, hemostasis, and angiogenesis [9,10]. The majority of [10][11][12][13][14], although not all [15], studies in the literature to date, show that endothelium function in cyanotic CHD patients assessed by flow-mediated distensibility is impaired. It is postulated to be related to disturbed production of endothelialderived nitric oxide, an important regulator of smooth muscle cell relaxation, and has a pathognomonic relation with increased arterial wall stiffness.…”

Evaluation of systemic microvascular reactivity in adults with congenital heart disease

“…The results available in the literature on the microvascular endothelial function of ACHD are conflicting. Cordina et al evaluated the vascular reactivity of individuals with cyanotic congenital heart disease and reported the presence of endothelial dysfunction when patients were evaluated for flow‐mediated dilatation but not for peripheral arterial tonometry (PAT), suggesting the presence of endothelial dysfunction in conductance vessels but not in the microcirculation . Lambert et al used the PAT technique and observed microvascular endothelial dysfunction in patients with Fontan circulation .…”

“…TA B L E 4 Microvascular data and arterial blood pressure of healthy volunteers (CONTROL, n = 29) and adult patients with congenital heart disease (ACHD, n = 31 for ACh and PORH data; n = 24 for SNP data) patients were evaluated for flow-mediated dilatation but not for peripheral arterial tonometry (PAT), suggesting the presence of endothelial dysfunction in conductance vessels but not in the microcirculation. 24 Lambert et al used the PAT technique and observed microvascular endothelial dysfunction in patients with Fontan circulation. 26 Oechslin et al employed venous occlusion plethysmography and observed the occurrence of microvascular endothelial dysfunction in cyanotic congenital cardiopathies.…”

“…21 Among the pathophysiological changes in HF, endothelial dysfunction is highlighted and is present even in the early stages. 22 However, the presence of endothelial dysfunction in patients with congenital heart disease is controversial because some studies have demonstrated the occurrence of endothelial dysfunction in these patients, [23][24][25][26][27] whereas other studies have not confirmed this condition. [28][29][30][31] Research in individuals with HF has shown that these individuals present compromised systemic endothelial function of the cutaneous microcirculation.…”