Department of Cardiology, Hospital Universitario Puerta de Hierro Majadahonda, Manuel de Falla, 1, 28222, Madrid, Spain.
Department of Cardiac Surgery, Hospital Universitario Puerta de Hierro Majadahonda, Madrid, Spain.
Int J Cardiovasc Imaging. 2021 Sep;37(9):2735-2745. doi: 10.1007/s10554-021-02247-7. Epub 2021 Apr 19.
Previous studies using conventional echocardiographic measurements have reported subclinical left ventricular (LV) diastolic abnormalities in patients with Marfan syndrome (MFS). Left atrial (LA) strain allows an accurate categorization of LV diastolic dysfunction. We aimed to characterize LV myocardial performance in a cohort of MFS patients using STE-derived measurements (LV and LA strain) along with conventional echocardiographic parameters. We studied 127 adult patients with MFS (no prior cardiac surgery or significant valvular regurgitation) and 38 healthy controls. We performed detailed echocardiograms and selected left atrial reservoir strain (LASr) as a surrogate of impaired relaxation. Additionally, we searched for possible determinants of LASr in patients with MFS, with a special focus on the elastic properties of the aorta. In spite of lower E-wave, septal and lateral e' velocities and average E/e' ratio in MFS patients, all participants had normal diastolic function according to current guidelines. MFS patients exhibited reduced LV global longitudinal strain (19.3 ± 2.6 vs 21.6 ± 2.1%, p < 0.001) and reduced LASr (32.9 ± 8.5 vs 43.3 ± 7.8%, p < 0.001) compared to controls. In the MFS cohort, we found weak significant (p < 0.05) correlations between LASr and certain parameters: E/A ratio (R = 0.258), E wave (R = 0.226), aortic distensibility (R = 0.222), stiffness index (R = - 0.216), LV ejection fraction (R = 0.214), lateral e' (R = 0.210), LV end-systolic volume index (R = - 0.210), LV global longitudinal strain (R = 0.201), septal e' (R = 0.185). After multivariate analysis, only LV end-systolic volume index and E/A ratio maintained a weak independent association with LASr (R = - 0.220; p = 0.017 and R = 0.199; p = 0.046, respectively). In conclusion, LASr is reduced in patients with MFS, which may represent an early stage of LV diastolic dysfunction. LASr is not determined by the elastic properties of the aorta, suggesting that impaired myocardial relaxation is a primary condition in MFS.
先前使用传统超声心动图测量的研究报告显示，马凡综合征（MFS）患者存在亚临床左心室（LV）舒张功能异常。左心房（LA）应变可准确分类 LV 舒张功能障碍。我们旨在使用 STE 衍生的测量值（LV 和 LA 应变）以及传统超声心动图参数来描述 MFS 患者的 LV 心肌功能。我们研究了 127 名成年 MFS 患者（无先前心脏手术或明显瓣膜反流）和 38 名健康对照者。我们进行了详细的超声心动图检查，并选择左房储备应变（LASr）作为舒张功能障碍的替代指标。此外，我们还在 MFS 患者中寻找 LASr 的可能决定因素，特别关注主动脉的弹性特性。尽管 MFS 患者的 E 波、间隔和侧壁 e'速度以及平均 E/e'比值较低，但根据当前指南，所有参与者均具有正常的舒张功能。与对照组相比，MFS 患者的 LV 整体纵向应变（19.3 ± 2.6%与 21.6 ± 2.1%，p < 0.001）和 LASr（32.9 ± 8.5%与 43.3 ± 7.8%，p < 0.001）均降低。在 MFS 组中，我们发现 LASr 与某些参数之间存在弱显著相关性（p < 0.05）：E/A 比值（R = 0.258）、E 波（R = 0.226）、主动脉可扩张性（R = 0.222）、僵硬度指数（R = -0.216）、LV 射血分数（R = 0.214）、侧壁 e'（R = 0.210）、LV 收缩末期容积指数（R = -0.210）、LV 整体纵向应变（R = 0.201）、间隔 e'（R = 0.185）。多元分析后，只有 LV 收缩末期容积指数和 E/A 比值与 LASr 存在弱的独立相关性（R = -0.220；p = 0.017 和 R = 0.199；p = 0.046）。总之，MFS 患者的 LASr 降低，这可能代表 LV 舒张功能障碍的早期阶段。LASr 不受主动脉弹性特性的影响，表明心肌舒张功能障碍是 MFS 的主要病变。
