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

Abstract

Introduction 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. Objectives 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. Methods 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. Results 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. Conclusion 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.

CFTR deficiency aggravates Ang II induced vasoconstriction and hypertension by regulating Ca𝟐+ influx and RhoA/Rock pathway in VSMCs

“…As a small outwardly rectifying chloride channel, the effect of CFTR on vasoconstriction should get more attention. In previous studies, aortic diameter measured at valsalva sinuses was significantly higher in patients with CF mutations [37]. CFTR inhibition enhanced pressure-induced myogenic vasoconstriction [15], and aortic rings from Cftr −/− mice showed a much stronger depolarization induced vasoconstriction than those from wild-type mice [16].…”

Aortic Elasticity Evaluated by Pulsed Tissue Doppler Imaging of the Ascending Aorta in Different Diseases: A Systematic Review

“…Aortic distensibility was lower and aortic stiffness was higher in patients with cystic fibrosis compared with healthy controls, even before development of pulmonary hypertension. 14 Similar changes were observed in patients with subclinical hypothyroidism, 15 sarcoidosis, 16 a1-antitrypsin deficiency, 17 erectile dysfunction, 18 obstructive sleep apnea, 19 chronic kidney disease, 20 metabolic syndrome, 21 hypertrophic cardiomyopathy, 22 and coronary artery disease (Table 1). 23 Aortic elastance was decreased in patients with a previous history of HELLP (hemolysis, elevated liver enzymes, and low platelet count) and preeclampsia which was associated with the gestational age.…”

ISPAD Clinical Practice Consensus Guidelines 2022: Management of cystic fibrosis‐related diabetes in children and adolescents

“…In a US cohort of 484 adults with CF, the prevalence of hypertension was 17% in normal weight CF adults increasing to 31% in overweight adults 84 . Studies of vascular distensibility suggest subtle changes, traditionally recognized as precursors to cardiovascular complications in non‐CF populations, may be present in CF 87,88 . Prior to the introduction of HEMT, cholesterol has been generally low in CF 89–92 .…”

“… 84 Studies of vascular distensibility suggest subtle changes, traditionally recognized as precursors to cardiovascular complications in non‐CF populations, may be present in CF. 87 , 88 Prior to the introduction of HEMT, cholesterol has been generally low in CF. 89 , 90 , 91 , 92 Although a recent study of 256 Canadian adults with CF found hyperlipidemia in a small percentage, it was not associated with CFRD or hyperglycemia.…”