Dynamic perfluorinated gas MRI reveals abnormal ventilation despite normal FEV1 in cystic fibrosis

JCI Insight. 2020 Jan 30;5(2):e133400. doi: 10.1172/jci.insight.133400.


We hypothesized that dynamic perfluorinated gas MRI would sensitively detect mild cystic fibrosis (CF) lung disease. This cross-sectional study enrolled 20 healthy volunteers and 24 stable subjects with CF, including a subgroup of subjects with normal forced expiratory volume in the first second (FEV1; >80% predicted, n = 9). Dynamic fluorine-19-enhanced MRI (19F MRI) were acquired during sequential breath holds while breathing perfluoropropane (PFP) and during gas wash-out. Outcomes included the fraction of lung without significant ventilation (ventilation defect percent, VDP) and time constants that described PFP wash-in and wash-out kinetics. VDP values (mean ± SD) of healthy controls (3.87% ± 2.7%) were statistically different from moderate CF subjects (19.5% ± 15.5%, P = 0.001) but not from mild CF subjects (10.4% ± 9.9%, P = 0.24). In contrast, the fractional lung volume with slow gas wash-out was elevated both in subjects with mild (9.61% ± 4.87%; P = 0.0066) and moderate CF (16.01% ± 5.01%; P = 0.0002) when compared with healthy controls (3.84% ± 2.16%) and distinguished mild from moderate CF (P = 0.006). 19F MRI detected significant ventilation abnormalities in subjects with CF. The ability of gas wash-out kinetics to distinguish between healthy and mild CF lung disease subjects makes 19F MRI a potentially valuable method for the characterization of early lung disease in CF. This study has been registered at ClinicalTrials.gov (NCT03489590).

Keywords: Diagnostic imaging; Pulmonology.

MeSH terms

  • Adult
  • Aged
  • Cross-Sectional Studies
  • Cystic Fibrosis / diagnostic imaging*
  • DNA-Binding Proteins
  • Female
  • Fluorocarbons / chemistry*
  • Humans
  • Kinetics
  • Lung / diagnostic imaging*
  • Magnetic Resonance Imaging / methods*
  • Male
  • Middle Aged
  • Transcription Factors
  • Ventilation
  • Young Adult


  • DNA-Binding Proteins
  • FEV protein, human
  • Fluorocarbons
  • Transcription Factors
  • perflutren

Associated data

  • ClinicalTrials.gov/NCT03489590