Kiotsekoglou Anatoli, Saha Samir, Moggridge James C, Kapetanakis Venediktos, Govindan Malini, Alpendurada Francisco, Mullen Michael J, Nassiri Dariush K, Camm John, Sutherland George R, Bijnens Bart H, Child Anne
Department of Cardiac and Vascular Sciences, St George's University of London, London, UK.
Echocardiography. 2011 Apr;28(4):416-30. doi: 10.1111/j.1540-8175.2010.01359.x.
To investigate the presence of any regional myocardial deformation abnormalities in Marfan syndrome (MFS) and determine the benefits of using advanced echocardiography compared to conventional techniques.
Myocardial dysfunction in MFS may be caused by extracellular matrix remodeling thus, resulting in uniform reduced functionality. However, increased aortic stiffness may cause segmental ventricular abnormalities. Strain rate imaging (SRI) constitutes a validated technique to assess regional deformation in various clinical conditions. With this in mind, we aimed to investigate biventricular function in MFS using SRI.
Forty-four MFS patients (mean age 30 ± 12 years, 26 men) and 49 controls without valvular disease were examined using SRI. Ejection fraction (EF) was calculated by the Simpson's biplane method. Biventricular deformation was assessed by measuring strain/strain rate. Strain values were divided by left ventricular (LV) end-diastolic volume to adjust LV deformation for geometry changes providing a strain index (SI). Aortic stiffness was evaluated using the β-stiffness index.
EF (%) was reduced in MFS patients (59 ± 5 vs 72 ± 4, P < 0.001), whereas β-stiffness was increased (P < 0.001). LV radial and LV and right ventricular (RV) long-axis strain values (%) were reduced in the patient group (70 ± 17 vs 93 ± 10; 19 ± 2 vs 25 ± 2; 30 ± 9 vs 36 ± 8, respectively, P < 0.001). Strain rate measurements were also reduced (P < 0.001). In a multiple regression analysis, MFS diagnosis was negatively associated with LV SI (-0.262 [-0.306, -0.219], P < 0.001). β-Stiffness was negatively associated with SI obtained from the septum, inferior and anterior walls. ROC analyses demonstrated that SRI, when compared with conventional echocardiography, had higher sensitivity and specificity in predicting biventricular dysfunction in MFS.
Our study showed a uniform reduction in biventricular deformation in MFS. These findings suggest that assessment of myocardial function using advanced echocardiographic techniques could be more accurate in MFS patient evaluation than conventional echocardiography alone.
研究马方综合征（MFS）患者是否存在局部心肌变形异常，并确定与传统技术相比，使用先进超声心动图技术的优势。
MFS患者的心肌功能障碍可能由细胞外基质重塑引起，进而导致整体功能下降。然而，主动脉僵硬度增加可能导致节段性心室异常。应变率成像（SRI）是一种经过验证的技术，可用于评估各种临床情况下的局部变形。基于此，我们旨在使用SRI研究MFS患者的双心室功能。
对44例MFS患者（平均年龄30±12岁，26例男性）和49例无瓣膜疾病的对照者进行SRI检查。采用双平面 Simpson法计算射血分数（EF）。通过测量应变/应变率评估双心室变形。将应变值除以左心室（LV）舒张末期容积，以调整LV变形以适应几何形状变化，从而得到应变指数（SI）。使用β僵硬度指数评估主动脉僵硬度。
MFS患者的EF（%）降低（59±5 vs 72±4，P<0.001），而β僵硬度增加（P<0.001）。患者组LV径向应变值、LV和右心室（RV）长轴应变值（%）降低（分别为70±17 vs 93±10；19±2 vs 25±2；30±9 vs 36±8，P<0.001）。应变率测量值也降低（P<0.001）。在多元回归分析中，MFS诊断与LV SI呈负相关（-0.262[-0.306，-0.219]，P<0.001）。β僵硬度与从室间隔、下壁和前壁获得的SI呈负相关。ROC分析表明，与传统超声心动图相比，SRI在预测MFS患者双心室功能障碍方面具有更高的敏感性和特异性。
我们的研究表明MFS患者双心室变形普遍降低。这些发现表明，在MFS患者评估中，使用先进超声心动图技术评估心肌功能可能比单独使用传统超声心动图更准确。
