Quantitative MRI detects delayed perfusion and impact of bronchial artery dilatation on pulmonary circulation in patients with cystic fibrosis

Objectives MRI detects abnormal lung perfusion in patients with cystic fibrosis (CF). However, little is known about the contribution of bronchial arteries to lung perfusion in CF. We hypothesized that delayed perfusion can be detected by dynamic contrast-enhanced (DCE-)MRI and that bronchial artery dilatation (BAD) is associated with changes in lung perfusion. Materials and methods Morpho-functional MRI was prospectively acquired in 75 patients with CF (18.7 ± 7.6 years, range 6–39 years). Lungs and perfusion defects were segmented automatically to quantify perfusion defects in percent (QDP). Pulmonary blood flow (PBF), mean transit time (MTT), and perfusion delay were calculated for the whole lung, inside normally perfused and perfusion defect areas. Chest MRI score and BAD were assessed visually. Results QDP and PBF correlated with MRI global score (r = 0.58 and −0.53, p < 0.001). In normally perfused lung, PBF was higher (161.2 ± 77.9 mL/100 mL/min vs. 57.5 ± 26.4 mL/100 mL/min, p < 0.001), and MTT (5.4 ± 1.7 s vs. 6.9 ± 2.3 s, p < 0.001) and perfusion delay were shorter than in perfusion defect areas (4.6 ± 5.3 s vs. 13.4 ± 16.2 s, p < 0.001). ... mehr48 (64.0%) patients showed BAD, had higher QDP (44.6 ± 20.8% vs. 17.3 ± 11.0%, p < 0.001) and lower PBF (91.9 ± 54.8 mL/100 mL/min vs. 178.3 ± 77.4 mL/100 mL/min, p < 0.001) than patients without BAD. MTT was shorter (6.3 ± 1.9 s vs. 8.0 ± 2.6 s, p < 0.001), and perfusion delay was longer (13.8 ± 10.1 s vs. 12.8 ± 23.7 s, p < 0.02) inside perfusion defects of patients with BAD compared to without BAD. Conclusion Perfusion parameters correlate with lung disease severity, and perfusion defects showed delayed perfusion in patients with CF. BAD was associated with more extensive perfusion defects and reduced PBF.