Longitudinal Functional Magnetic Resonance Imaging to Assess Lung Disease in Cystic Fibrosis.

RATIONALE: Matrix pencil decomposition magnetic resonance imaging (MP-MRI) enables quantitative assessment of lung ventilation and perfusion and was shown to correlate with contrast-enhanced (CE) perfusion MRI.

OBJECTIVES

To determine the longitudinal course of MP-MRI parameters in people with cystic fibrosis (pwCF).

METHODS

Morpho-functional MRI including CE perfusion MRI and MP-MRI were performed twice in 136 pwCF with a median (range) baseline age of 14.5 (0.1-56.0) years to obtain the morpho-functional MRI score. Perfusion defect percentage (QDP) and ventilation defect percentage (VDP) were calculated by automated software. Additionally, lung clearance index (LCI) and forced expiratory volume in one second (FEV1) z-score were assessed. The participants were divided in subgroups depending on the clinical course according to cystic fibrosis transmembrane conductance regulator modulator therapy, pulmonary exacerbation status and Pseudomonas aeruginosa infection status.

RESULTS

In the total cohort, VDP improved at follow up. Change in VDP correlated with change in MRI global score, MRI CE perfusion score and FEV1 z-score. Additionally, change in QDP correlated with change in MRI global score, MRI morphology score, MRI CE perfusion score and FEV1 z-score. QDP and VDP remained stable in clinically stable pwCF (n = 55) and improved in clinically improved pwCF (n = 67). In the stable and improved group, change in QDP correlated moderately with change in MRI CE perfusion score.

CONCLUSIONS

MP-MRI assesses stability and improvement of lung perfusion and ventilation in pwCF. Changes correlate with those of established MRI scores. Therefore, QDP and VDP are sensitive longitudinal outcome parameters for future clinical studies.