Lalande A, Khau Van Kien P, Walker P M, Zhu L, Legrand L, Claustres M, Jeunemaître X, Brunotte F, Wolf J E
Groupe Imagerie Médicale, LE2I, UMR CNRS 5158, Faculté de Médecine de Dijon, Université de Bourgogne, Dijon, France.
J Magn Reson Imaging. 2008 Nov;28(5):1180-7. doi: 10.1002/jmri.21565.
To evaluate aortic elasticity with MRI on young asymptomatic individuals with mutation of the smooth muscle myosin heavy chain in whom aortic enlargement is not present.
Aortic compliance, aortic distensibility, and pulse wave velocity (PWV) were semiautomatically measured from MRI in 8 asymptomatic subjects having a mutation of the MYH11 gene (M+) and 21 nonmutated relatives (M-) of similar age, sex, and blood pressure characteristics.
Despite a similar aortic diameter in both groups, the aortic compliance and distensibility were significantly lower in M+ subjects compared with M- (0.84+/-0.33 versus 2.03+/-0.54 mm2/mmHg, 1.18+/-0.62 10(-3) versus 5.11+/-1.58 10(-3) mmHg(-1), respectively), and PWV was significantly higher (5.35+/-1.53 versus 3.60+/-0.64 m.s(-1)). A threshold aortic compliance value of 1.3 mm2/mmHg separated the two groups. The receiver operating characteristics curve analysis indicated an optimal threshold of 2.9 10(-3) mmHg(-1) for aortic distensibility (sensitivity: 87.5%, specificity: 90%), and of 4.4 m.s(-1) for PWV (sensitivity: 75%, specificity: 100%).
Young asymptomatic adults with MYH11 mutation have an aortic compliance impairment which is not detectable by the sole measurement of the aortic size. Aortic compliance measurement might be part of routine examination in patients suspected of inherited aortic disease even with a normal aortic diameter.
在无主动脉扩张的年轻无症状平滑肌肌球蛋白重链突变个体中，利用磁共振成像（MRI）评估主动脉弹性。
对8名有MYH11基因突变的无症状受试者（M+）和21名年龄、性别及血压特征相似的无突变亲属（M-），通过MRI半自动测量主动脉顺应性、主动脉扩张性和脉搏波速度（PWV）。
尽管两组主动脉直径相似，但M+受试者的主动脉顺应性和扩张性显著低于M-受试者（分别为0.84±0.33对2.03±0.54mm2/mmHg，1.18±0.62×10(-3)对5.11±1.58×10(-3)mmHg(-1)），且PWV显著更高（5.35±1.53对3.60±0.64m·s(-1)）。主动脉顺应性阈值为1.3mm2/mmHg可区分两组。受试者工作特征曲线分析表明，主动脉扩张性的最佳阈值为2.9×10(-3)mmHg(-1)（敏感性：87.5%，特异性：90%），PWV的最佳阈值为4.4m·s(-1)（敏感性：75%，特异性：100%）。
有MYH11突变的年轻无症状成年人存在主动脉顺应性损害，仅通过测量主动脉大小无法检测到。即使主动脉直径正常，主动脉顺应性测量也可能是疑似遗传性主动脉疾病患者常规检查的一部分。
