PDF(Pubmed)
Abstract：
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
摘要：
UNASSIGNED：很难评估马凡氏综合征（MFS）患者主动脉根部以外的主动脉夹层的风险。为了帮助这些患者进行风险评估，我们通过4D血流磁共振成像(MRI)研究了MFS患者的主动脉血流和壁切应力(WSS),并将结果与健康志愿者进行了比较.我们假设主动脉夹层高危人群的MFS患者会在与主动脉夹层相关的主动脉区域表现出异常的血流动力学。
未经评估：MFS患者（n=55）和健康受试者（n=25），年龄和性别相匹配,前瞻性接受4D流MRI。构建4D流动图以检测与对照相比MFS患者中升高的(定义为高于三维95%置信区间)和偏离的(定义为高于120°的矢量角度差)WSS。评估了MFS患者与主动脉夹层危险因素的单变量和多变量关联。
UNASSIGNED：WSS升高的最大发生率为20％（CI9％-31％），见于升主动脉。异常定向WSS的最大值为39％（CI26％-52％），见于内降主动脉。明显更多的男性患者在内近端降主动脉有异常定向的WSS（63％vs24％，p=0.014)。多因素分析显示,异常定向WSS与男性相关(p=0.019),和单倍不足的FBN1突变类型（p=0.040）。在60％的MFS患者之前进行过主动脉根部置换手术，升主动脉血流动力学异常。手术和非手术患者在降主动脉中的血流动力学之间没有发现显着差异。
UNASSIGNED：在MFS患者中，近端降主动脉的偏离定向WSS与已知的主动脉夹层危险因素相关，即男性和单倍体不足的FBN1突变类型。
未经评估：MFS患者（n=55）和健康受试者（n=25），年龄和性别相匹配,前瞻性接受4D流MRI。构建4D流动图以检测与对照相比MFS患者中升高的(定义为高于三维95%置信区间)和偏离的(定义为高于120°的矢量角度差)WSS。评估了MFS患者与主动脉夹层危险因素的单变量和多变量关联。
UNASSIGNED：WSS升高的最大发生率为20％（CI9％-31％），见于升主动脉。异常定向WSS的最大值为39％（CI26％-52％），见于内降主动脉。明显更多的男性患者在内近端降主动脉有异常定向的WSS（63％vs24％，p=0.014)。多因素分析显示,异常定向WSS与男性相关(p=0.019),和单倍不足的FBN1突变类型（p=0.040）。在60％的MFS患者之前进行过主动脉根部置换手术，升主动脉血流动力学异常。手术和非手术患者在降主动脉中的血流动力学之间没有发现显着差异。
UNASSIGNED：在MFS患者中，近端降主动脉的偏离定向WSS与已知的主动脉夹层危险因素相关，即男性和单倍体不足的FBN1突变类型。
