Division of Cardiology, University of Miami Miller School of Medicine, Children's Heart Center, Holtz Children's Hospital, Florida, USA.
Am J Cardiol. 2012 May 1;109(9):1358-64. doi: 10.1016/j.amjcard.2012.01.001. Epub 2012 Feb 15.
Conventional 2-dimensional, M-mode, and spectral Doppler echocardiographic techniques have documented abnormal ventricular function in adults with sickle cell disease (SCD), but assessments in children are conflicting. Tissue Doppler echocardiography (TDE) provides additional information about myocardial function. Two-dimensional, M-mode, tricuspid regurgitation jet velocity (TRJV) data, and tissue Doppler echocardiographically derived myocardial velocity measurements of left ventricular (LV) and right ventricular function were taken from children with SCD compared to those of similar healthy historical controls and correlated with clinical characteristics and hemoglobin levels. Compared to 55 controls, 54 children with SCD (mean age 14.2 years, range 6 to 21) had a larger left ventricle, greater LV mass, and higher LV fractional shortening; 30% had increased pulmonary artery pressure (TRJV ≥2.5 m/s). Conventional echocardiographic measurements of LV systolic function and spectral Doppler measurements of LV and right ventricular diastolic function were essentially normal, but TDE indicated that 31% of SCD children had evidence of LV diastolic dysfunction (peak early diastolic velocity of LV inflow Doppler/peak early diastolic velocity at lateral mitral valve annulus >8), a finding that correlated with lower hemoglobin levels. Although decreasing hemoglobin levels in children with SCD correlated with LV hypertrophy, LV dilation, and LV diastolic dysfunction, long-term transfusion or hydroxyurea therapy did not affect these measurements. In conclusion, 1/3 of children with SCD had tissue Doppler echocardiographic evidence of LV diastolic dysfunction, which was correlated with hemoglobin levels. Adding serial assessments of ventricular function with TDE to conventional echocardiography may detect early cardiac changes, especially in children with severe anemia.
传统的二维、M 型和频谱多普勒超声心动图技术已经记录了镰状细胞病（SCD）成人的心室功能异常，但儿童的评估结果存在矛盾。组织多普勒超声心动图（TDE）提供了心肌功能的额外信息。从 SCD 儿童中获取了二维、M 型、三尖瓣反流速度（TRJV）数据和组织多普勒超声心动图衍生的左心室（LV）和右心室功能的心肌速度测量值，并与类似的健康历史对照进行了比较，并与临床特征和血红蛋白水平相关。与 55 名对照相比，54 名 SCD 患儿（平均年龄 14.2 岁，范围 6 至 21 岁）的左心室更大，LV 质量更大，LV 缩短分数更高；30%的患儿肺动脉压升高（TRJV≥2.5m/s）。LV 收缩功能的传统超声心动图测量值和 LV 和右心室舒张功能的频谱多普勒测量值基本正常，但 TDE 表明 31%的 SCD 患儿有 LV 舒张功能障碍的证据（LV 流入多普勒的早期舒张峰值速度/外侧二尖瓣环的早期舒张峰值速度>8），这一发现与较低的血红蛋白水平相关。尽管 SCD 儿童的血红蛋白水平下降与 LV 肥厚、LV 扩张和 LV 舒张功能障碍相关，但长期输血或羟基脲治疗并未影响这些测量值。总之，1/3 的 SCD 儿童有组织多普勒超声心动图证据表明存在 LV 舒张功能障碍，这与血红蛋白水平相关。在传统超声心动图的基础上，通过 TDE 对心室功能进行连续评估可能会发现早期心脏变化，尤其是在严重贫血的儿童中。
