Comparison of phase-resolved functional lung (PREFUL) and hyperpolarized ¹²⁹Xe MRI for longitudinal monitoring of lung function in pediatric cystic fibrosis following elexacaftor/tezacaftor/ivacaftor.

Authors

Samal Munidasa, Faiyza Alam, Brandon Zanette, Daniel Li, Wallace Wee, Sharon Braganza, Jason Woods, Felix Ratjen, Giles Santyr

Published in

Pediatric radiology. Sep 12, 2025. Epub Sep 12, 2025.

Abstract

Phase-resolved functional lung (PREFUL) MRI offers a more accessible alternative to hyperpolarized ¹²⁹Xe MRI (Xe-MRI) for monitoring treatment response in pediatric cystic fibrosis (CF), but longitudinal comparisons are limited.
To assess longitudinal lung function changes following elexacaftor/tezacaftor/ivacaftor (ETI) treatment initiation in CF children using PREFUL MRI, in comparison with Xe-MRI and pulmonary function tests (PFTs).
PREFUL MRI, Xe-MRI, and PFTs were performed in 14 CF patients (median [IQR] age 15 [14-16.5] years old) at baseline and 1 month, 6 months, 12 months, and 24 months following initiation of ETI treatment. Ventilation and defect percentage (VDP) was derived from PREFUL MRI (regional ventilation VDP, VDP_RVent; regional-flow volume loop cross-correlation VDP, VDP_CC; and the combination of VDP_RVent and VDP_CC, VDP_combined) and Xe-MRI (VDP_Xe) maps. Perfusion defect percentage (QDP) was derived from normalized perfusion maps and, with VDP_combined, determined the percentage of healthy ventilation-perfusion matching (VQM). Significance of 1-month treatment changes was determined using the Wilcoxon-signed rank test and was correlated between metrics using Spearman ranked correlation.
All PREFUL measures significantly improved (P < 0.01) 1-month post-treatment in agreement with changes in Xe-MRI VDP and PFTs (P < 0.03). The absolute change in VDP_CC and VDP_combined significantly correlated with VDP_Xe (r ≥ 0.62, P < 0.02), unlike VDP_RVent (P = 0.35). The change in QDP did not correlate with any metric (P > 0.10). PREFUL MRI and Xe-MRI measures showed minimal changes 1 to 24 months post-treatment (median changes = -2.3% to 1.4%), in agreement with PFTs.
PREFUL MRI detects longitudinal treatment-related changes in pulmonary ventilation and perfusion in CF children post ETI.

PMID:
40938435
Bibliographic data and abstract were imported from PubMed on 12 Sep 2025.

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