Abnormal aortic hemodynamics are associated with risk factors for aortic complications in patients with marfan syndrome.

Abstract

It is difficult to assess the risk for aortic dissection beyond the aortic root in patients with Marfan syndrome (MFS). To aid risk assessment in these patients, we investigated aortic flow and wall shear stress (WSS) by 4D flow magnetic resonance imaging (MRI) in patients with MFS and compared the results with healthy volunteers. We hypothesized that MFS patients with a high-risk profile for aortic dissection would show abnormal hemodynamics in aortic regions associated with aortic dissection. MFS patients (n=55) and healthy subjects (n=25), matched for age and sex, prospectively underwent 4D flow MRI. 4D flow maps were constructed to detect elevated (defined as higher than the three-dimensional 95% confidence interval) and deviant directed (defined as vector angle differences higher than 120°) WSS in MFS patients as compared to the controls. Univariate and multivariate associations with risk factors for aortic dissection in MFS patients were assessed. The maximum incidence for elevated WSS was 20% (CI 9%-31%) and found in the ascending aorta. The maximum for deviant directed WSS was 39% (CI 26%-52%) and found in the inner descending aorta. Significantly more male patients had deviant directed WSS in the inner proximal descending aorta (63% vs 24%, p=0.014). Multivariate analysis showed that deviant directed WSS was associated with male sex (p=0.019), and a haplo-insufficient FBN1 mutation type (p=0.040). In 60% of MFS patients with a previous aortic root replacement surgery, abnormal hemodynamics were found in the ascending aorta. No significant differences between hemodynamics were found in the descending aorta between operated and non-operated patients. Deviant directed WSS in the proximal descending aorta is associated with known risk factors for aortic dissection in MFS patients, namely male sex and a haploinsufficient FBN1 mutation type.