Cardiovascular complications of cystic fibrosis

Damian G. Downey*, J. Stuart Elborn

*Corresponding author for this work

Research output: Chapter in Book/Report/Conference proceedingChapter (peer-reviewed)peer-review

2 Citations (Scopus)


Cystic fibrosis (CF) is an autosomal-recessive disease caused by mutations in the gene that encodes the CF transmembrane conductance regulator (CFTR) protein. This results in progressive lung damage, respiratory failure and premature death. Recent development of therapies to modulate CFTR has resulted in significant improvements in lung function. As people with CF (PWCF) live longer, there will be a rise in nonpulmonary complications. CVD is the leading cause of death worldwide, with increasing recognition that inflammatory respiratory tract conditions have been linked to a higher risk of CVD. Although right heart changes are established consequences of severe lung disease, left ventricular dysfunction and arterial stiffness have been identified in young PWCF. CFTR loss has also been implicated in impaired endothelial cell function and systemic inflammation, which can lead to CVD. Further research is required for PWCF with increasing risk factors for CVD; this should inform screening recommendations and risk-reduction strategies in the era of CFTR modulation.
Original languageEnglish
Title of host publicationCardiovascular complications of respiratory disorders
EditorsMiguel Ángel Martínez-García, Jean-Louis Pépin, Mario Cazzola
PublisherEuropean Respiratory Society
Number of pages10
ISBN (Electronic)9781849841191
Publication statusPublished - 01 Jun 2020

Publication series

NameERS Monograph
PublisherEuropean Respiratory Society
ISSN (Print)2312-508X

Bibliographical note

Publisher Copyright:
© ERS 2020.

Copyright 2020 Elsevier B.V., All rights reserved.

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine


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