Monitoring cardiovascular disease risk in children with cystic fibrosis using arterial stiffness: A new perspective

Abstract

Introduction: Early diagnosis with newborn screening programs and prolonged life expectancy with new treatment strategies have made cardiovascular disease (CVD) one of the important issues in cystic fibrosis (CF). In the early stages of CVD, it is difficult to recognize and follow-up increased arterial stiffness with conventional methods. Different measurement methods are needed. Therefore, in this study, we aimed to use arterial stiffness measurements in the follow-up of children with CF.

Materials and methods: This is a follow-up study examining the changes in arterial stiffness in children with CF by repeating hemodynamic measurements [augmentation index (AIx) and pulse wave velocity (PWV)]. We repeated hemodynamic measurements and CF-related CVD risk factors (Atherosclerosis risk factors: Fasting blood sugar, lipid profiles, and HbA1c) and systemic inflammation markers [C-reactive protein (CRP) and immunoglobulin G and pulmonary function tests] in children undergoing routine annual complication evaluation and examined changes during follow-up.

Result: Hemodynamic measurements could be repeated in 37 of 52 patients due to inclusion criteria. Mean age of the study group was 12 ± 4.5 years and 48.6% were female. There was a statistically significant increase in high density lipoprotein, HbA1c, and CRP and a decrease in low density lipoprotein and FEV1 at the follow-up. Heart rate, central blood pressure, augmented pressure, and PWV were similar. AIx, peripheral systolic blood pressure (SBP), and mean arterial pressure were increased significantly (p< 0.05). The increase in AIx was greater than expected for age and greater in female patients and in those with low body mass index, moderate-severe disease, and high CRP levels. Also, the change in AIx was positively correlated with changes in peripheral SBP and CRP.

Conclusions: This is the first study to evaluate the use of PWV and AIx in the follow-up of children with CF and showed that arterial stiffness measured with AIx increased at follow-up. The use of markers of arterial stiffness in CF from childhood onwards may enable early detection and monitoring of CVD risk and future prevention.