Highlights
- •Xe ventilation MRI and spirometry are sensitive to changes post ACT.
- •Large structural and associated functional defects are mostly unchanged by ACT.
- •Xe ventilation defects improved most in areas without obvious structural defects.
Abstract
Background
Airway clearance therapy (ACT) with a high-frequency chest wall oscillation (HFCWO)
vest is a common but time-consuming treatment. Its benefit to quality of life for
cystic fibrosis (CF) patients is well established but has been questioned recently
as new highly-effective modulator therapies begin to change the treatment landscape.
129Xe ventilation MRI has been shown to be very sensitive to lung obstruction in mild
CF disease, making it an ideal tool to identify and quantify subtle, regional changes.
Methods
20 CF patients (ages 20.7 ± 5.1 years) refrained from performing ACT before arriving
for a single-day visit. Multiple-breath washout (MBW), spirometry, Xe MRI, and ultrashort
echo-time (UTE) MRI were obtained twice—before and after patients performed ACT using
their prescribed HFCWO vests (average 4.7 ± 0.5 h). UTE MRIs were scored for structural
abnormalities, and standard functional metrics were obtained from MBW, spirometry,
and Xe MRI—FEV1,pp, LCI2.5, and VDPN4, respectively.
Results
Spirometry and Xe MRI detected significant improvements in lung function post-ACT.
15/20 patients showed improvements from a baseline median of 92% FEV1,pp. Similarly, 16/20 patients showed improvements in Xe MRI from a baseline median of
15.2% VDPN4. Average individual changes were +2.6% in FEV1,pp and -1.3% in VDPN4, but without spatial correlations to easily-identifiable causative structural defects
(e.g. mucus plugs or bronchiectasis) on UTE MRI.
Conclusions
Lung function improved after a single instance of HFCWO-vest ACT and was detectable
by spirometry and Xe MRI. The only common structural abnormalities were mucus plugs,
which corresponded to ventilation defects, but ventilation defects were often present
without visible abnormalities.
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Article info
Publication history
Published online: February 03, 2023
Accepted:
January 30,
2023
Received in revised form:
January 10,
2023
Received:
August 25,
2022
Publication stage
In Press Corrected ProofIdentification
Copyright
© 2023 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.
