Abnormal aortic hemodynamics are associated with risk factors for aortic complications in patients with marfan syndrome. (December 2022)

• Record Type: Journal Article
• Authors: van Andel, Mitzi M.; van Ooij, Pim; de Waard, Vivian; Gottwald, Lukas M.; van Kimmenade, Roland R.J.; Scholte, Arthur J.; Dickinson, Michael G.; Zwinderman, Aeilko H.; Mulder, Barbara J.M.; Nederveen, Aart J.; Groenink, Maarten
• Is Part Of: IJC heart & vasculature. Volume 43(2023)
• Publication Date: 2022-12
• Subjects: Wall Shear Stress -- Marfan syndrome -- Aortic dissection -- Clinical Applications -- Quantitative Imaging; CI confidence interval -- CMR cardiac magnetic resonance -- DN dominant-negative -- FBN1 fibrillin-1 -- HI haploinsufficient -- IRQ interquartile range -- MAP mean arterial pressure -- MFS Marfan syndrome -- MRI magnetic reasonace imaging; Cardiovascular system -- Diseases -- Periodicals; Cardiovascular system -- Pathophysiology -- Periodicals; 616.1005
• DOI: 10.1016/j.ijcha.2022.101128
• Languages: English
• ISSNs: 2352-9067

Abstract

Highlights: Hemodynamic parameters obtained from 4D flow MRI are useful for risk stratification in MFS patients. Especially in combination with additional risk factors, such as male sex, and a HI mutation type. Deviant directed WSS in the descending aorta was more frequently seen in male MFS patients, and patients with a HI mutation type. 60% of MFS patients with previous aortic root replacement showed abnormal hemodynamics in the ascending aorta. Abstract: Background: 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. Methods: 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. Results: 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. Conclusion: 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.