Detection of early subclinical lung disease in children with cystic fibrosis by lung ventilation imaging with hyperpolarised gas MRI

Abstract

Detection of early subclinical lung disease in children with cystic fibrosis by lung ventilation imaging with hyperpolarised gas MRI ABSTRACT Hyperpolarised 3 He ventilation-MRI, anatomical lung MRI, lung clearance index (LCI), low-dose CT and spirometry were performed on 19 children (6-16 years) with clinically stable mild cystic fibrosis (CF) (FEV 1 > −1.96), and 10 controls. All controls had normal spirometry, MRI and LCI. Ventilation-MRI was the most sensitive method of detecting abnormalities, present in 89% of patients with CF, compared with CT abnormalities in 68%, LCI 47% and conventional MRI 22%. Ventilation defects were present in the absence of CT abnormalities and in patients with normal physiology, including LCI. Ventilation-MRI is thus feasible in young children, highly sensitive and provides additional information about lung structure-function relationships.

Longitudinal Assessment of Children with Mild Cystic Fibrosis Using Hyperpolarized Gas Lung Magnetic Resonance Imaging and Lung Clearance Index

“…14 children with CF from the cohort previously reported (8) were re-assessed at a second time point between 1.3-2.0 years after baseline using helium-3 ( 3 He) ventilation MRI, MBW, spirometry and body plethysmography using the same methods as previously described (8). Patients were clinically stable for at least 4 weeks prior to both visits (free from exacerbation and needing no new treatments) and were on stable chronic medical regimens according to national guidelines.…”

“…We have previously reported cross-sectional data on a cohort of 19 children with mild CF lung disease where ventilation MRI demonstrated lung defects when abnormality was often not detectable on either CT or LCI (8). The aim of the present study was to re-assess these children at a second time point 1-2 years 3 following the baseline visit and describe any observed longitudinal changes in lung function or ventilation MRI.…”

Assessing the feasibility of hyperpolarized 129Xe multiple‐breath washout MRI in pediatric cystic fibrosis

“…Ventilation defect percent is reproducible over short time periods (i.e., up to a few weeks) in patients with CF with stable health, and longitudinal imaging (i.e., up to a few years) can identify functional changes that are not reflected in PFTs . In addition, VDP is strongly correlated with LCI, and this result has been confirmed using HP 129 Xe and 3 He MRI . It has been suggested that both VDP and LCI probe similar physiology, as VDP visualizes the spatial heterogeneity of ventilation during a static breath‐hold at a single time‐point following a single HP gas inhalation, and LCI measures the temporal heterogeneity of the tracer gas washout (i.e., nitrogen [N 2 ] or sulfur hexafluoride [SF 6 ]) during multiple exhalations.…”

Deep convolutional neural networks with multiplane consensus labeling for lung function quantification using UTE proton MRI

“…MAGNETIC RESONANCE IMAGING (MRI) of the chest can provide regional assessment of lung ventilation by inhaling oxygen (O 2 ), hyperpolarized noble gases, or fluorinated gas . Oxygen‐enhanced (OE) MRI using a 3D radial ultrashort echo time (UTE) sequence supports quantitative differentiation of diseased vs. healthy lungs using whole lung ventilation defect percent (VDP) .…”