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

Cutting GR (2014) Cystic fibrosis genetics: from molecular understanding to clinical application. Nat Rev Genet 16:45–56

Article  PubMed  PubMed Central  Google Scholar 

Harman K, Dobra R, Davies JC (2017) Disease-modifying drug therapy in cystic fibrosis. Paediatr Respir Rev 26:7–9

PubMed  Google Scholar 

Griese M, Costa S, Linnemann RW et al (2021) Safety and efficacy of elexacaftor/tezacaftor/ivacaftor for 24 weeks or longer in people with cystic fibrosis and one or more F508del alleles: interim results of an open-label phase 3 clinical trial. Am J Respir Crit Care Med 203:381–385

Article  PubMed  PubMed Central  Google Scholar 

Barry PJ, Mall MA, Álvarez A et al (2021) Triple therapy for cystic fibrosis Phe508del-gating and -residual function genotypes. N Engl J Med 385:815–825

Article  PubMed  PubMed Central  CAS  Google Scholar 

Wielpütz MO, Stahl M, Triphan SMF et al (2024) Longitudinal magnetic resonance imaging of changes in lung morphology and perfusion in children with cystic fibrosis from infancy through adolescence. Published online, Ann Am Thorac Soc. https://doi.org/10.1513/AnnalsATS.202404-396OC

Book  Google Scholar 

Graeber SY, Renz DM, Stahl M et al (2022) Effects of elexacaftor/tezacaftor/ivacaftor therapy on lung clearance index and magnetic resonance imaging in patients with cystic fibrosis and one or two F508del alleles. Am J Respir Crit Care Med 206:311–320

Article  PubMed  CAS  Google Scholar 

Santyr G, Kanhere N, Morgado F et al (2018) Hyperpolarized gas magnetic resonance imaging of pediatric cystic fibrosis lung disease. Acad Radiol 26:344–354

Article  PubMed  Google Scholar 

Thomen RP, Walkup LL, Roach DJ et al (2016) Hyperpolarized 129Xe for investigation of mild cystic fibrosis lung disease in pediatric patients. J Cyst Fibros 16:275–282

Article  PubMed  PubMed Central  Google Scholar 

Rayment JH, Couch MJ, McDonald N et al (2019) Hyperpolarised 129Xe magnetic resonance imaging to monitor treatment response in children with cystic fibrosis. Eur Respir J 53(5):1802188. https://doi.org/10.1183/13993003.02188-2018

Article  PubMed  CAS  Google Scholar 

Walkup L, Roach D, Zanette B et al (2022) Hyperpolarized xenon gas ventilation magnetic resonance imaging detects lung ventilation improvement in children with cystic fibrosis treated with elexacaftor/tezacaftor/ivacFaftor in a multisite study. J Cyst Fibros 21:S97

Article  Google Scholar 

Bauman G, Puderbach M, Deimling M et al (2009) Non-contrast-enhanced perfusion and ventilation assessment of the human lung by means of Fourier decomposition in proton MRI. Magn Reson Med 62:656–664

Article  PubMed  Google Scholar 

Fischer A, Weick S, Ritter CO et al (2014) Self-gated non-contrast-enhanced functional lung imaging (SENCEFUL) using a quasi-random fast low-angle shot (FLASH) sequence and proton MRI. NMR Biomed 27:907–917

Article  PubMed  Google Scholar 

Bauman G, Bieri O (2016) Matrix pencil decomposition of time-resolved proton MRI for robust and improved assessment of pulmonary ventilation and perfusion. Magn Reson Med 77:336–342

Article  PubMed  Google Scholar 

Ilicak E, Ozdemir S, Zapp J et al (2023) Dynamic mode decomposition of dynamic MRI for assessment of pulmonary ventilation and perfusion. Magn Reson Med 90:761–769

Article  PubMed  Google Scholar 

Voskrebenzev A, Gutberlet M, Klimeš F et al (2018) Feasibility of quantitative regional ventilation and perfusion mapping with phase-resolved functional lung (PREFUL) MRI in healthy volunteers and COPD, CTEPH, and CF patients. Magn Reson Med 79:2306–2314

Article  PubMed  CAS  Google Scholar 

Munidasa S, Zanette B, Couch M et al (2022) Inter- and intravisit repeatability of free-breathing MRI in pediatric cystic fibrosis lung disease. Magn Reson Med 89:2048–2061

Article  PubMed  Google Scholar 

Munidasa S, Couch MJ, Rayment JH et al (2021) Free-breathing MRI for monitoring ventilation changes following antibiotic treatment of pulmonary exacerbations in paediatric cystic fibrosis. Eur Respir J 57(4):2003104. https://doi.org/10.1183/13993003.03104-2020

Article  PubMed  Google Scholar 

Couch MJ, Munidasa S, Rayment JH et al (2020) Comparison of functional free-breathing pulmonary 1H and hyperpolarized 129Xe magnetic resonance imaging in pediatric cystic fibrosis. Acad Radiol 28:e209–e218

Article  PubMed  Google Scholar 

Munidasa S, Zanette B, Dumas MP et al (2024) Comparison of 3D UTE free-breathing lung MRI with hyperpolarized 129Xe MRI in pediatric cystic fibrosis. Magn Reson Med 93:775–787

Article  PubMed  PubMed Central  Google Scholar 

Klimeš F, Plummer JW, Willmering MM et al (2025) Quantifying spatial and dynamic lung abnormalities with 3D PREFUL FLORET UTE imaging: a feasibility study. Magn Reson Med 93:1984–1998

Article  PubMed  PubMed Central  Google Scholar 

Klimeš F, Voskrebenzev A, Gutberlet M et al (2024) Effect of CFTR modulator therapy with elexacaftor/tezacaftor/ivacaftor on pulmonary ventilation derived by 3D phase-resolved functional lung MRI in cystic fibrosis patients. Eur Radiol 34:80–89

Article  PubMed  Google Scholar 

Dohna M, Voskrebenzev A, Klimeš F et al (2024) PREFUL MRI for monitoring perfusion and ventilation changes after elexacaftor-tezacaftor-ivacaftor therapy for cystic fibrosis: a feasibility study. Radiol Cardiothorac Imaging 6:e230104

Article  PubMed  PubMed Central  Google Scholar 

Miller MR, Hankinson J, Brusasco V et al (2005) Standardisation of spirometry. Eur Respir J 26:319–338

Article  PubMed  CAS  Google Scholar 

Robinson PD, Latzin P, Verbanck S et al (2013) Consensus statement for inert gas washout measurement using multiple- and single-breath tests. Eur Respir J 41:507–522

Article  PubMed  CAS  Google Scholar 

Voskrebenzev A, Klimeš F, Wacker F et al (2024) Phase-resolved functional lung MRI for pulmonary ventilation and perfusion (V/Q) assessment. J Vis Exp 9(210). https://doi.org/10.3791/66380

Klimeš F, Voskrebenzev A, Gutberlet M et al (2019) Free-breathing quantification of regional ventilation derived by phase-resolved functional lung (PREFUL) MRI. NMR Biomed 32:e4088

Article  PubMed  Google Scholar 

Behrendt L, Smith LJ, Voskrebenzev A et al (2022) A dual-center and dual-vendor comparison study of automated perfusion-weighted phase-resolved functional lung magnetic resonance imaging with dynamic contrast-enhanced magnetic resonance imaging in patients with cystic fibrosis. Pulm Circ 12:e12054

Article  PubMed  PubMed Central  CAS  Google Scholar 

Pöhler GH, Klimeš F, Behrendt L et al (2020) Repeatability of phase-resolved functional lung (PREFUL)-MRI ventilation and perfusion parameters in healthy subjects and COPD patients. J Magn Reson Imaging 53:915–927

Article  PubMed  Google Scholar 

Zanette B, Schrauben EM, Munidasa S et al (2022) Clinical feasibility of structural and functional MRI in free-breathing neonates and infants. J Magn Reson Imaging 55:1696–1707

Article  PubMed  Google Scholar 

Stanojevic S, Kaminsky DA, Miller MR et al (2022) ERS/ATS technical standard on interpretive strategies for routine lung function tests. Eur Respir J 60:2101499

Article  PubMed  Google Scholar 

Perrem L, Stanojevic S, Shaw M et al (2021) Lung clearance index to track acute respiratory events in school-age children with cystic fibrosis. Am J Respir Crit Care Med 203:977–986

Article  PubMed  CAS  Google Scholar 

Kaireit TF, Kern A, Voskrebenzev A et al (2020) Flow volume loop and regional ventilation assessment using phase-resolved functional lung (PREFUL) MRI: comparison with 129Xe ventilation MRI and lung function testing. J Magn Reson Imaging 53:1092–1105

Article  PubMed  Google Scholar 

Goralski JL, Chung SH, Ceppe AS et al (2022) Dynamic perfluorinated gas MRI shows improved lung ventilation in people with cystic fibrosis after elexacaftor/tezacaftor/ivacaftor: an observational study. J Clin Med 11:6160

Article  PubMed  PubMed Central  CAS  Google Scholar 

Bec R, Reynaud-Gaubert M, Arnaud F et al (2022) Chest computed tomography improvement in patients with cystic fibrosis treated with elexacaftor-tezacaftor-ivacaftor: early report. Eur J Radiol 154:110421

Comments (0)

No login
gif