Cardiovascular complications of cystic fibrosis

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.