Exercise capacity and ventilation inhomogeneity in cystic fibrosis: A cross‐sectional study

Abstract

Background Lung clearance index (LCI2.5) is a marker of overall lung ventilation inhomogeneity and has proven to be able to detect early peripheral damage in subjects with cystic fibrosis (CF), with greater sensitivity than conventional spirometry. Combining its sensitivity with the output of an incremental exercise testing, we hypothesized that any sign of ventilation inhomogeneity in subjects without severe airflow obstruction and with a normal exercise tolerance could be relevant for the CF team, tracking early lung disease and potential exercise limiting factors. Methods Patients with CF in clinical stable conditions were recruited between 2015 and 2017. Available spirometry, nitrogen multiple‐breath washout test and symptoms‐limited exercise testing performed as parts of patients' annual routine evaluation were considered for this cross‐sectional study. To describe the relationship between exercise intensity and ventilation inhomogeneity, a linear regression analysis was performed using backward elimination based on Akaike information criteria. Results Seventy‐seven patients (38 females) were included. Sacin and LCI2.5 were significantly higher in patients with an overall reduced exercise tolerance. Peak work developed during exercise was associated with body mass index (b = 5.25; 95% confidence interval [CI] = 1.53‐8.98), forced expiratory volume in 1 second (FEV1; b = 3.71; 95% CI = 1.96‐5.46), Pseudomonas aeruginosa chronic infection (b = −8.84; 95% CI = −15.84 to −1.84) but not with LCI2.5. Conclusion Exercise capacity and airflow obstruction are associated in this Italian CF cohort. Considering the greater discriminatory power of LCI2.5 over FEV1 and peak work, the Godfrey protocol without gas analysis cannot provide detailed information about lung function or efficiency. However, this incremental protocol without gas exchange measures can still provide the CF team with information about exercise tolerance and disability.

Pediatric Pulmonology Year in Review 2020: Physiology

“…Gambazza, et al evaluated the association of LCI with exercise capacity in patients with CF [8]. LCI was significantly higher in patients with exercise intolerance, but it was also higher in some patients with normal exercise tolerance and normal to moderate airflow obstruction.…”

Pediatric pulmonology year in review 2020: Physiology

“…Gambazza et al 16 evaluated the association of LCI with exercise capacity in patients with CF. LCI was significantly higher in patients with exercise intolerance, but it was also higher in some patients with normal exercise tolerance and normal to moderate airflow obstruction.…”

Time Free From Hospitalization in Children and Adolescents With Cystic Fibrosis: Findings From FEV1, Lung Clearance Index and Peak Work Rate

“…Peak work capacity (Wpeak) elicited during continuous incremental cycle ergometry was shown to be a valid metric of cardiorespiratory fitness in children with CF ( 12 ) and predictive of survival ( 11 ). Furthermore, it is associated with nutritional status, airflow obstruction, and the presence of chronic Pseudomonas aeruginosa infection in adult patients ( 13 ). Altogether, exercise tests combined with respiratory function measures offer a comprehensive clinical evaluation of lung disease in children with CF.…”