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Abstract #0660

Functional 3D-UTE MRI of the Lungs for Monitoring Ventilation Inhomogeneity in Patients with Cystic Fibrosis on Treatment with CFTR Modulators

Julius Heidenreich1, Philipp Josef Kuhl1, Corona Metz1, Andreas Max Weng1, Jan-Peter Grunz1, Thomas Benkert2, Helge Hebestreit3, Thorsten Alexander Bley1, Herbert Köstler1, and Simon Veldhoen1
1Department of Diagnostic and Interventional Radiology, University Hospital Würzburg, Würzburg, Germany, 2Siemens Healthineers GmbH, Erlangen, Germany, 3Department of Pediatrics, University Hospital Würzburg, Würzburg, Germany


Single breath-hold 3D-UTE-MRI was proposed for simultaneous evaluation of lung morphology and function. Sixteen patients with cystic fibrosis (CF) underwent initial and follow-up 3D-UTE-MRI in 2019 and 2021. In line with a previous study, referenced voxelwise fractional ventilation (FV) provided a marker that correlates strongly with the lung clearance index (LCI), reflecting ventilation inhomogeneity. Seven patients received a new drug-combination (ivacaftor/elexacaftor/tezacaftor) with significantly improved lung function during clinical follow-up. Similarly, significantly decreased ventilation inhomogeneity was derived from functional MRI. Thus, single breath-hold 3D-UTE MRI is a valuable tool for monitoring ventilation inhomogeneity in patients with CF during drug treatment.

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