Cardiovascular complications in cystic fibrosis: A review of the literature.

Cystic fibrosis is a genetic disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, leading to dysfunction of the CFTR protein. CFTR dysfunction leads to disease in the respiratory and gastrointestinal systems. Disorders of the cardiovascular system in individuals with CF are usually attributed to secondary effects from progressive lung disease. However, CFTR has been localized to vascular endothelium and smooth muscle, suggesting that CFTR dysfunction may directly impact cardiovascular function. As treatments for CF improve and life-expectancy increases, the risk of vascular disease may increase in prevalence related to primary and secondary CFTR dysfunction, chronic systemic inflammation, nutritional health and hyperglycemia in individuals with CF related diabetes. Here we review the available literature on CF and the cardiovascular system, examining the secondary effects and evidence for direct CFTR dysfunction in the heart, aorta, pulmonary vessels, and vasculature, as well as future directions and treatment options.
Competing Interests: Declaration of Competing Interest Dr. Taylor–Cousar reports grants and personal fees from Gilead, grants from N30, grants and personal fees from Vertex, grants and personal fees from Proteostasis, grants from Bayer, personal fees from Novartis, personal fees from Genentech, personal fees from Protalix, personal fees from Santhera, personal fees from 4DMT, personal fees from Polarean Imaging, personal fees from Insmed, personal fees from Abbvie, grants and personal fees from Celtaxys outside the submitted work; and Service on the CF TDN Clinical Research Executive Committee, Clinical Research Advisory Board, and as Chair of the Women's Health Research Working Group as well as service as the Chair of the ATS Clinical Problems Assembly Program Committee. Dr. Zemanick reports personal fees from Cystic Fibrosis Foundation, site clinical trial support from Vertex Pharmaceuticals and Savara Pharmaceuticals Inc., and material agreements and consulting contracts with Calithera Biosciences and Concert Pharmaceuticals outside the scope of the submitted work.
(Copyright © 2021 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.)