TY - JOUR T1 - Patterns of regional lung physiology in cystic fibrosis using ventilation magnetic resonance imaging and multiple-breath washout JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.00821-2018 VL - 52 IS - 5 SP - 1800821 AU - Laurie J. Smith AU - Guilhem J. Collier AU - Helen Marshall AU - Paul J.C. Hughes AU - Alberto M. Biancardi AU - Martin Wildman AU - Ina Aldag AU - Noreen West AU - Alex Horsley AU - Jim M. Wild Y1 - 2018/11/01 UR - http://erj.ersjournals.com/content/52/5/1800821.abstract N2 - Hyperpolarised helium-3 (3He) ventilation magnetic resonance imaging (MRI) and multiple-breath washout (MBW) are sensitive methods for detecting lung disease in cystic fibrosis (CF). We aimed to explore their relationship across a broad range of CF disease severity and patient age, as well as assess the effect of inhaled lung volume on ventilation distribution.32 children and adults with CF underwent MBW and 3He-MRI at a lung volume of end-inspiratory tidal volume (EIVT). In addition, 28 patients performed 3He-MRI at total lung capacity. 3He-MRI scans were quantitatively analysed for ventilation defect percentage (VDP), ventilation heterogeneity index (VHI) and the number and size of individual contiguous ventilation defects. From MBW, the lung clearance index, convection-dependent ventilation heterogeneity (Scond) and convection–diffusion-dependent ventilation heterogeneity (Sacin) were calculated.VDP and VHI at EIVT strongly correlated with lung clearance index (r=0.89 and r=0.88, respectively), Sacin (r=0.84 and r=0.82, respectively) and forced expiratory volume in 1 s (FEV1) (r=−0.79 and r=−0.78, respectively). Two distinct 3He-MRI patterns were highlighted: patients with abnormal FEV1 had significantly (p<0.001) larger, but fewer, contiguous defects than those with normal FEV1, who tended to have numerous small volume defects. These two MRI patterns were delineated by a VDP of ∼10%. At total lung capacity, when compared to EIVT, VDP and VHI reduced in all subjects (p<0.001), demonstrating improved ventilation distribution and regions of volume-reversible and nonreversible ventilation abnormalities.Ventilation distribution on MRI improves at TLC and two distinct patterns of regional lung disease in CF are highlighted, where abnormal FEV1 is associated with VDP >10%. Ventilation MRI and MBW are highly correlated. http://ow.ly/NvyS30lOP4O ER -