Search

MRI was performed on 1.5 T Philips Achieva MRI (Philips Medical Systems, Best, The Netherlands). PC-MRI with through-plane velocity encoding (Venc = 150 cm/s) and free-breathing was performed perpendicular to the ascending and descending aorta at the level transecting the pulmonary trunk. Another acquisition (Venc = 100 cm/ s) was performed at the abdominal aorta. PWV was determined for the aortic arch (AA), distal aorta (DA) and total aorta using the transit-time method described by Grotenhuis.1 Brachial-cuff systolic and diastolic blood pressure (BP) were obtained. Distensibility (= luminal area change/(diastolic luminal area × pulse pressure)) and Stiffness Index (= ln [(BPSystolic/BPDiastolic)/(diameter change/diastolic diameter)]) were determined at the ascending aorta in gradient-echo PC-MRI magnitude images. Reduced wall compliance is expressed as increased PWV and SI and decreased Dist. PWV, Dist and SI were compared in Marfan and controls using paired ttests. Age relation was determined by linear regression.

Summary By using a comprehensive MRI-approach in patients with Marfan syndrome, increased regional pulse wave velocity in the ascending aorta can predict in 42% of the cases aortic luminal growth in the ascending aorta, while normal PWV can predict in 89% absence of luminal growth. Background The leading cause of premature death in patients with Marfan syndrome (MFS) is aortic dissection after progressive dilatation due to local increased wall stiffness, occurring most prominently in the ascending aorta. Aortic pulse wave velocity (PWV) is a marker of wall stiffness [Nollen, et al. Eur Heart J 2004]. Regional PWV can be accurately determined from in-plane multi-directional velocity-encoded (VE) MRI [Westenberg, et al. JMRI 2010]. The study objective was to test whether regional PWV can predict regional aorta dilatation at 2year follow-up (FU) in MFS.