Summary of notable 1H and xenon 129 (129Xe) magnetic resonance imaging findings. In subject 05 (left column), general intralobular septal thickening and focal areas of subpleural fibrosis (red arrowheads) were noted on the 1H UTE scan and on the most concurrent clinical chest CT scan. 129Xe ventilation was uniform for subject 01 (second column, top; 129Xe ventilation defect percentage = 2.2%), despite reductions in spirometry values (FEV1 = 75%, FEV1/FVC = 78%). However, subject 06 (second column, bottom), who also had a symmetric decrease in pulmonary function test measures (FEV1 = 78%, FEV1/FVC = 78%), had heterogeneous ventilation with noticeable deficits (yellow arrowhead) and had an elevated 129Xe ventilation defect percentage of 12%, suggestive of some degree of airflow obstruction. Barrier-uptake and red blood cell (RBC)-transfer maps from 129Xe gas-exchange magnetic resonance imaging are shown for two subjects. Subject 07 (third column; 93% Dl CO Hb-adjusted) had mostly normal barrier-uptake and RBC-transfer maps, with some anterior regions of Barrierlow and RBClow that were likely due to gravitational effects being shown. In comparison, the 129Xe gas-exchange maps for subject 04 (fourth column; 45% Dl CO Hb-adjusted) were very heterogeneous, with marked decreases in the barrier 129Xe signal (top; Barrierlow = 51%) and both abnormal decreases and abnormal increases in RBC transfer (bottom; RBClow = 52%, RBChigh = 18%) being shown. Barrierhigh = lung volume in the highest 2 bins for the barrier compartment; Barrierlow = lung volume in the lowest 2 bins for the barrier compartment; CT = computed tomography; MRI = magnetic resonance imaging; RBChigh = lung volume in the highest 2 bins for the RBC compartment; RBClow = lung volume in the lowest 2 bins for the RBC compartment; UTE = ultra-short echo time.

Reprinted with permission of the American Thoracic Society. Copyright © 2022 American Thoracic Society. All rights reserved. Cite: Author(s)/Year/Title/Journal title/Volume/Pages. The American Journal of Respiratory and Critical Care Medicine is an official journal of the American Thoracic Society.

129Xe ventilation and apparent diffusion coefficient (ADC) maps in three representative lymphangioleiomyomatosis cases demonstrating the spectrum of disease severity investigated. The 129Xe ventilation defect percentage values for subjects 05 (top row), 11 (middle row), and 18 (bottom row) were 14.8%, 15%, and 24.3%, respectively. A single slice of the ADC maps for each of these subjects is shown in the right column. 129Xe ADC, calculated from diffusion-weighted imaging, is a surrogate measurement of alveolar airspace size, and the color bar shows increasing ADC value and thus airspace size. The 129Xe ADC values were 0.040 ± 0.016, 0.048 ± 0.022, and 0.066 ± 0.027 cm2/s, compared with approximately 0.036 cm2/s for age-matched healthy adults (17). FEV1 = forced expiratory volume in 1 second.

Reprinted with permission from Ann Am Thorac Soc. 2019 Aug: 16(8): 1008-1016. Walkup et.al. Cyst Ventilation Heterogeneity and Alveolar Airspace Dilation as Early Disease Markers in Lymphangioleiomyomatosis.

Example image slices for 129Xe (a.) and UTE (b.) images in CF subjects. Subject 14 (left) is an example of a subject a relatively healthy subject with only LCI2.5 being slightly elevated. Subject 18 (middle) is an example of a subject with elevated LCI2.5, VDP and structural abnormalities despite normal FEV1. Subject 26 (right) is an example subject who also had an abnormal FEV1 %-predicted. In (a.), regions circled in red illustrate examples of ventilation defects and in (b.), example regions are circled in orange for combined bronchiectasis/wall thickening, yellow for only bronchiectasis and blue for mucus plugging. Each image provides the subject's FEV1 %-predicted, LCI2.5, VDP, and Brody score.

Reprinted with permission from Journal of Cystic Fibrosis. Volume 20, Issue 3, May 2021, Pages 533-539. Walkup et.al. Sensitive structural and functional measurements and 1-year pulmonary outcomes in pediatric cystic fibrosis.