4.7 Article

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

Journal

JCI INSIGHT
Volume 5, Issue 2, Pages -

Publisher

AMER SOC CLINICAL INVESTIGATION INC
DOI: 10.1172/jci.insight.133400

Keywords

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Funding

  1. NIH [P30 DK065988, 4 K12 HL 119998-4]
  2. CFF [GORALS12L0, PACE GORALS16AC0, DONALD18Y7]
  3. National Center for Advancing Translational Sciences (NCATS), NIH [UL1TR002489]

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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 (F-19 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). F-19 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( 19)F MRI a potentially valuable method for the characterization of early lung disease in CF.

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