Purpose
The purpose of this study is to assess the intra‐ and interscan repeatability of free‐breathing phase‐resolved functional lung (PREFUL) MRI in stable pediatric cystic fibrosis (CF) lung disease in comparison to static breath‐hold hyperpolarized 129‐xenon MRI (Xe‐MRI) and pulmonary function tests.
Methods
Free‐breathing 1‐hydrogen MRI and Xe‐MRI were acquired from 15 stable pediatric CF patients and seven healthy age‐matched participants on two visits, 1 month apart. Same‐visit MRI scans were also performed on a subgroup of the CF patients. Following the PREFUL algorithm, regional ventilation (RVent) and regional flow volume loop cross‐correlation maps were determined from the free‐breathing data. Ventilation defect percentage (VDP) was determined from RVent maps (VDPRVent), regional flow volume loop cross‐correlation maps (VDPCC), VDPRVent ∪ VDPCC, and multi‐slice Xe‐MRI. Repeatability was evaluated using Bland–Altman analysis, coefficient of repeatability (CR), and intraclass correlation.
Results
Minimal bias and no significant differences were reported for all PREFUL MRI and Xe‐MRI VDP parameters between intra‐ and intervisits (all P > 0.05). Repeatability of VDPRVent, VDPCC, VDPRVent ∪ VDPCC, and multi‐slice Xe‐MRI were lower between the two‐visit scans (CR = 14.81%, 15.36%, 16.19%, and 9.32%, respectively) in comparison to the same‐day scans (CR = 3.38%, 2.90%, 1.90%, and 3.92%, respectively). pulmonary function tests showed high interscan repeatability relative to PREFUL MRI and Xe‐MRI.
Conclusion
PREFUL MRI, similar to Xe‐MRI, showed high intravisit repeatability but moderate intervisit repeatability in CF, which may be due to inherent disease instability, even in stable patients. Thus, PREFUL MRI may be considered a suitable outcome measure for future treatment response studies.