St. George's, University of London, Department of Cardiac and Vascular Sciences, London, UK.
Hellenic J Cardiol. 2010 Nov-Dec;51(6):501-11.
several studies have documented increased aortic stiffness in patients with Marfan syndrome (MFS) using echocardiography and magnetic resonance imaging. Recent studies have also shown primary myocardial impairment in MFS. We investigated whether left ventricular (LV) function could be further impaired when acting against a stiff vascular system.
twenty-six MFS patients (mean age 30 ± 2 years, 17 males) and 30 normal controls were examined. Mitral annular displacement, as a surrogate for LV systolic function, was evaluated from septal, anterolateral, anterior and inferior regions using M-mode and tissue Doppler imaging. Septal/anterolateral and anterior/inferior M-mode displacement measurements were normalised by dividing them by the longitudinal inner distance obtained at end diastole from the 4- and 2-chamber views, respectively. Carotid-femoral and carotid-radial (CF and CR) pulse wave velocities (PWV) were determined using an automated applanation tonometry device. Central aortic pressure was assessed by recording radial waveforms with the tonometer and central waveforms were reconstructed using a generalised transfer function.
CF- and CR-PWV were significantly increased in the patient group (p<0.001), whilst mitral annular displacement measurements were significantly reduced (p<0.001, all regions). Regression analysis demonstrated that the disease status and CF-PWV were strongly associated with reduced LV systolic function (p<0.001, p=0.002, respectively).
our study showed reduced LV systolic function and increased aortic stiffness in MFS patients. The efficiency of a fibrillin-1 deficient heart may be further reduced by ejection into a stiff vascular system. Care should be taken to ensure that any treatment regime addresses both increased aortic stiffness and myocardial dysfunction in MFS.
几项研究使用超声心动图和磁共振成像记录了马凡综合征（MFS）患者主动脉僵硬增加。最近的研究还表明 MFS 存在原发性心肌损伤。我们研究了当左心室（LV）对抗僵硬的血管系统时，LV 功能是否会进一步受损。
共纳入 26 例 MFS 患者（平均年龄 30 ± 2 岁，男性 17 例）和 30 例正常对照者。使用 M 型和组织多普勒成像，从间隔、前外侧、前壁和下壁区域评估二尖瓣环位移，作为 LV 收缩功能的替代指标。通过将间隔/前外侧和前壁/下壁 M 型位移测量值分别除以 4 腔和 2 腔视图获得的舒张末期纵向内距离进行标准化。使用自动平板测压设备测量颈动脉-股动脉（CF）和颈动脉-桡动脉（CR）脉搏波速度（PWV）。使用测压计记录桡动脉波形来评估中心动脉压，并使用广义传递函数重建中心波形。
患者组 CF 和 CR-PWV 显著增加（p<0.001），而二尖瓣环位移测量值显著降低（p<0.001，所有区域）。回归分析表明，疾病状态和 CF-PWV 与 LV 收缩功能降低密切相关（p<0.001，p=0.002）。
我们的研究显示 MFS 患者 LV 收缩功能降低和主动脉僵硬增加。射血进入僵硬的血管系统可能会进一步降低缺乏原纤维蛋白-1的心脏的效率。在制定任何治疗方案时，都应注意同时解决 MFS 中主动脉僵硬和心肌功能障碍的问题。
