We have quantified the extent of ventilation impairment in lungs due to specific pathologies associated with cystic fibrosis (CF) lung disease using ultrashort echo-time (UTE) MRI to identify structural abnormalities and hyperpolarized (HP) 129Xe MRI to identify ventilation deficits. We found that bronchiectasis demonstrates the best correlation with lung function decline, as measured by the percent predicted forced expiratory volume in 1 second (FEV1% predicted) and demonstrated the greatest deficit in HP 129Xe signal within corresponding defective regions. However, the greatest volume-percentage of defects identified were due to mucus plugging.
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