Title

3D MR spirometry in patients with cystic fibrosis

Leading partner

Erasmus

Work Package

WP1

Sites

Erasmus MC-Sophia Children’s Hospital, Rotterdam, the Netherlands

Population

30 patients (15 girls and 15 boys) with CF with different degrees of functional severity will be recruited prospectively within their annual follow-up program to undergo chest CT, spirometry and 3D MR spirometry on the same day.

Objectives

1. Validate 3D MR spirometry against spirometry in CF
2. Validate 3D MR Spirometry against chest-CT (performed as clinical follow-up)
3. Assess the regional lung structural and functional changes and mechanics in patients with CF with a broad range of disease severity using 3D MR spirometry

Examination

All included patients will have spirometry, chest-CT and 3D MR spirometry performed the same day at Erasmus MC hospital. Spirometry will be performed in two positions (sitting and supine) and during two types of respiration (spontaneous and forced). Both CT and MRI scans will be acquired using a MR compatible spirometer to standardize lung volume and breathing manoeuvres.
3D MR spirometry will be performed on a 1.5 T MR imaging system (GE Healthcare) at Erasmus MC hospital. Patients will breath freely in a supine position during MR acquisition. Dynamic 3D lung images will be reconstructed by GE (WP1) and processed by UPSaclay (WP2) to primarily produce maps of flow-volume loops and maps of fractional anisotropy strain metric.