Patterns of regional lung physiology in cystic fibrosis using ventilation MRI and MBW

  1. Laurie J. Smith1,2,
  2. Guilhem J. Collier1,
  3. Helen Marshall1,
  4. Paul J.C. Hughes1,
  5. Alberto M. Biancardi1,
  6. Martin Wildman3,
  7. Ina Aldag2,
  8. Noreen West2,
  9. Alex Horsley1,4 and
  10. Jim M. Wild1,5
  1. 1POLARIS, Academic Radiology, University of Sheffield, Sheffield, UK
  2. 2Sheffield Children's Hospital NHS Foundation Trust, Sheffield, UK
  3. 3Sheffield Teaching Hospital NHS Foundation Trust, Sheffield, UK
  4. 4Respiratory Research Group, Division of Infection, Immunity & Respiratory Medicine, University of Manchester, Manchester, UK
  5. 5Insigneo, Institute of in-silico medicine, Sheffield, UK
  1. Jim M. Wild, POLARIS, Academic Radiology, Department of Infection, Immunity & Cardiovascular Disease, University of Sheffield, Sheffield, UK. E-mail: j.m.wild{at}sheffield.ac.uk

Abstract

Hyperpolarised helium-3 (3He) ventilation 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). 28 patients also performed 3He-MRI at total lung capacity (TLC). 3He-MRI 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 (LCI), Scond and Sacin were calculated.

VDP and VHI at EIVt strongly correlated with LCI (r=0.89, r=0.88 respectively), Sacin (r=0.84, r=0.82) and FEV1 (r=−0.79, r=−0.78). 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 approximately 10%. At TLC, when compared to EIVt, VDP and VHI reduced in all subjects (p<0.001), demonstrating improved ventilation distribution and also regions of volume-reversible and non-reversible ventilation abnormalities.

Footnotes

This manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.

Conflict of interest: Dr. Smith reports grants from National Institute for Health Research, during the conduct of the study.

Conflict of interest: Dr. Collier has nothing to disclose.

Conflict of interest: Dr. Marshall has nothing to disclose.

Conflict of interest: Dr. Hughes has nothing to disclose.

Conflict of interest: Dr. Biancardi has nothing to disclose.

Conflict of interest: Dr. Aldag has nothing to disclose.

Conflict of interest: Dr. West has nothing to disclose.

Conflict of interest: Dr. Horsley reports grants from National Institute of Health Research, personal fees from Celtaxys Pharmaceuticals, personal fees from Vertex Pharmaceuticals, personal fees from Boehringer Ingelheim, personal fees from Chiesi Ltd, non-financial support from Innovision ApS, grants from Danish Government, grants from Cystic Fibrosis Trust, grants from Cystic Fibrosis Foundation, outside the submitted work.

Conflict of interest: Dr. Wild has nothing to disclose.

Conflict of interest: Dr. Wildman has nothing to disclose.

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