Proton MRI assessment of lung structure and function in people with cystic fibrosis

Abstract

<b>Introduction:</b> There is an urgent need for highly sensitive diagnostics to match advances in pulmonary therapeutics, particularly in cystic fibrosis (CF). Non-contrast Ultrashort Echo Time (UTE) and phase-resolved functional lung (PREFUL) MRI provide high resolution structural and regionally-localized functional data, respectively. <b>Aim:</b> To use UTE and PREFUL MRI to assess lung structure and function in people with CF (pwCF). <b>Methods:</b> 7 pwCF (12-32 years; n=4 homozygous for p.Phe508del mutation) were imaged on a Philips 3T Ingenia MRI scanner. PREFUL was analysed using k-means segmentation to generate ventilation maps and quantify regional lung function as ventilation defect percentage (VDP). <b>Results:</b> Bronchiectatic and cystic changes were demonstrated with UTE. PREFUL VDP ranged from 8.09-42.23% (median: 19.4%). Ventilation defects were seen in all pwCF, including those with FEV₁ &gt; 100% (n=3).(Fig 1) <b>Conclusion:</b> This study demonstrates comprehensive lung MRI assessment in CF. UTE provides structural imaging without ionizing radiation. PREFUL MRI provides sensitive and regionally localized functional data, similar to ¹²⁹Xe MRI,&nbsp;but is easier to perform. Together, these techniques&nbsp;could be used to assess response to therapeutics and potentially disease progression in pwCF in&nbsp;the future.