Change in functional lung MRI and multiple breath washout outcomes over one year in children with cystic fibrosis

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<b>Background:</b> Lung MRI is a promising tool to monitor early changes in cystic fibrosis (CF). However, there are no data on the ability of functional MRI to assess disease progression in children with CF. <b>Aim:</b> To evaluate changes in functional lung MRI and lung function over one year in children with CF. <b>Methods:</b> Children with CF performed multiple breath washout, spirometry and functional lung MRI at baseline and one year later while clinically stable. We evaluated associations between change in ventilation defect percentage (VDP) and perfusion defect percentage (QDP) calculated from Matrix Pencil-MRI with changes in FEV₁, lung clearance index (LCI) and structural MRI-Eichinger Score. <b>Results:</b> Thirty-seven children (mean(SD) age: 10.7 (±3.7) years) had repeated measurements after one year (mean time: 13.0 (±3.7) months). There were no changes in outcomes over the study period: VDP: -0.73%, [95%CI:-2.3; 0.8]; QDP: -0.85%, [-2.2; 0.5]; FEV₁: 0.02 z-scores, [-0.2; 0.3]; LCI: -0.44 units, [-1.1; 0.2]; Eichinger-Score: 0.38 [-0.4; 1.2]. The change in VDP correlated moderately with the change in LCI (r: 0.44, p=0.02) and FEV1 (r: -0.51, p=0.007), but less with Eichinger (r:0.34, p=0.079). The change in QDP correlated moderately with change in LCI (r: 0.40, p=0.03) and Eichinger (r:0.39, p=0.045), but not with FEV₁ (r: -0.29, p=0.14). In most patients, changes were congruent, in some changes differed. <b>Conclusion:</b> Changes in ventilation and perfusion defects from MP-MRI were associated with changes in LCI over one year in most children with CF. In children with incongruent changes, functional MRI added complimentary information that helped to understand lung function changes.