ArticleEuropean radiology2024
Effect of CFTR modulator therapy with elexacaftor/tezacaftor/ivacaftor on pulmonary ventilation derived by 3D phase-resolved functional lung MRI in cystic fibrosis patients.
Article in European radiology, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers, 1 of them a synthesis that pooled it.
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Who cites it
14 citing papers in PubMed, 1 synthesis or guideline pooled it, 21 citations in OpenAlex.
- Non-contrast enhanced functional lung MRI in children: systematic review.Frontiers in pediatrics · 2025Pooled it
- Automated Ultrashort Echo Time (UTE) MRI Low Signal Volume Analysis in Stable Pediatric Cystic Fibrosis and After Elexacaftor/Tezacaftor/Ivacaftor Therapy.Journal of magnetic resonance imaging : JMRI · 2026Article
- Characteristics of pulmonary perfusion and ventilation in healthy adults: a prospective observational study with phase-resolved functional lung magnetic resonance imaging.BMC medical imaging · 2026Observational
- Comparison of Signal- and Volume-Based Ventilation-Weighted Assessment Using 3D FLORET UTE MRI in Patients With Various Pulmonary Disease.Magnetic resonance in medicine · 2026Article
- Structural and Functional Pulmonary MRI to Predict Pulmonary Exacerbations in Cystic Fibrosis.Chest · 2026Article
- Comparison of phase-resolved functional lung (PREFUL) and hyperpolarizedPediatric radiology · 2025Article
- [Pulmonary imaging of cystic fibrosis with a focus on children and adolescents].Radiologie (Heidelberg, Germany) · 2025Review
- Evaluation of pulmonary perfusion and ventilation in suspected chronic thromboembolic pulmonary hypertension via phase-resolved functional lung magnetic resonance imaging: correlations with hemodynamics and cardiopulmonary function.Quantitative imaging in medicine and surgery · 2025Article
- Progress of personalized medicine of cystic fibrosis in the times of efficient CFTR modulators.Molecular and cellular pediatrics · 2025Review
- Global trends and developments in pulmonary magnetic resonance imaging research: a bibliometric analysis of the past decade.Quantitative imaging in medicine and surgery · 2025Article
- Real-world improvement in ultra-low-dose thoracic computed tomography scores, systemic inflammatory markers and patient-reported outcome measures after elexacaftor/tezacaftor/ivacaftor treatment.ERJ open research · 2025Article
- Comparison of 3D UTE free-breathing lung MRI with hyperpolarizedMagnetic resonance in medicine · 2025Article
- A Need for Clarification Regarding Current State-of-the-Art Techniques for Characterizing Lung Parenchymal Aeration?Radiology. Cardiothoracic imaging · 2024Article
- Contrast agent-free functional magnetic resonance imaging with matrix pencil decomposition to quantify abnormalities in lung perfusion and ventilation in patients with cystic fibrosis.Frontiers in medicine · 2024Article
Corrections and comments
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Authors and funding
11 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectivesTo investigate whether 3D phase-resolved functional lung (PREFUL)-MRI parameters are suitable to measure response to elexacaftor/tezacaftor/ivacaftor (ETI) therapy and their association with clinical outcomes in cystic fibrosis (CF) patients.
methodsTwenty-three patients with CF (mean age: 21; age range: 14-46) underwent MRI examination at baseline and 8-16 weeks after initiation of ETI. Morphological and 3D PREFUL scans assessed pulmonary ventilation. Morphological images were evaluated using a semi-quantitative scoring system, and 3D PREFUL scans were evaluated by ventilation defect percentage (VDP) values derived from regional ventilation (RVent) and cross-correlation maps. Improved ventilation volume (IVV) normalized to body surface area (BSA) between baseline and post-treatment visit was computed. Forced expiratory volume in 1 second (FEV
resultsAfter ETI therapy, all 3D PREFUL ventilation markers (all p < 0.0056) improved significantly, except for the mean RVent parameter. The BSA normalized IVV
conclusions3D PREFUL MRI is a very promising tool to monitor CFTR modulator-induced regional dynamic ventilation changes in CF patients. CLINICAL RELEVANCE STATEMENT: 3D PREFUL MRI is sensitive to monitor CFTR modulator-induced regional ventilation changes in CF patients. Improved ventilation volume correlates with the relative change of mucus plugging, suggesting that reduced endobronchial mucus is predominantly responsible for regional ventilation improvement. KEY POINTS: • 3D PREFUL MRI-derived ventilation maps show significantly reduced ventilation defects in CF patients after ETI therapy. • Significant post-treatment correlations of 3D PREFUL ventilation measures especially with LCI, FEV
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