Elastic aortic properties in cystic fibrosis adults without cardiovascular risk factors: A case-control study

The presence of CFTR in smooth muscle and endothelial cells, systemic inflammation, and oxidative stress could explain vascular alterations in cystic fibrosis. Aortic elastic properties are determinants of left ventricular function by means of ventriculo-arterial coupling and indicators of cardiovascular risk.

The purpose of the present study was to compare clinically stable patients affected by cystic fibrosis without overt pulmonary hypertension with controls to evaluate aortic tissue Doppler elastic properties, such as distensibility, stiffness, and strain.

A total of 22 adults affected by cystic fibrosis, and 24 healthy volunteers matched for age and sex were enrolled. None had known cardiovascular risk factors, secondary diabetes, neither aortic stenosis nor regurgitation. All people underwent blood pressure measurement and transthoracic echocardiography.

Aortic diameter measured at Valsalva sinuses was significantly higher in patients with cystic fibrosis than healthy people, median 32.0 (interquartile range 29.8-35.0) vs 24.3 (22.2-30.0) mm; P < 0.001. Aortic distensibility was significantly lower among patients than controls, being 2.4 (1.3-3.3) vs 5.6 (3.4-8.3) per mm Hg (P < 0.001), while stiffness higher, 7.7 (6.0-14.8) vs 3.7 (2.9-6.7); P < 0.001. Finally, M-mode strain of ascending aorta was lower in patients, 4.1 (3.4-7.3)% than in controls, 13.4 (7.7-19.4)%; P < 0.001.

For the first time in humans, we demonstrated subclinical alterations in aortic elastic properties in young adults affected by cystic fibrosis without pulmonary hypertension or secondary diabetes. This phenomenon could influence left ventricular function earlier by means of ventriculo-arterial coupling and may be a tool to identify patients who benefit from a closer follow-up.