Abdul-Mohsen Mohammed Fakhry
University of Dammam and King Fahd Hospital of the University, Dammam, PO Box 40032, Alkhobar 31952.
J Saudi Heart Assoc. 2012 Oct;24(4):217-24. doi: 10.1016/j.jsha.2012.05.001. Epub 2012 Jun 12.
Sickle cell disease (SCD) is a chronic, inherited haemoglobin disorder, associated with recurrent vaso-occlusive and haemolytic crises and chronic tissue ischemia which may adversely affect any organ system. Our objectives were to evaluate the left ventricular (LV) systolic and diastolic functions in Saudi patients with SCD originally from the Eastern Province of Saudi Arabia.
Prospective hospital based echocardiography study on adolescent and adult patients with SCD.
Forty-five patients with SCD were recruited for echocardiographic study while 45 patients, matched for age and sex, served as controls. Left and right ventricular dimensions and LV wall thicknesses, LV mass index (LVMI) and LV contractility variables were obtained. Left atrial dimension and volume and pulmonary artery systolic pressure (PASP) were also estimated. We also evaluated parameters of LV diastolic function, including early and late mitral flow velocities (E and A wave respectively), E/A ratio, deceleration time (MVDT), A wave duration (MVA D), LV isovolumic relaxation time (IVRT), and tissue Doppler velocities, such as lateral annular e' wave, a' wave, e'/a' ratio and E/e' ratio.
There were increases in the LV dimensions, LV volumes, stroke volume, and LVMI of the SCD patients. The preload was increased (LV diastolic volume) and afterload was decreased (low diastolic blood pressure). The LVEF was equivalent, though there was evidence of LV diastolic dysfunction in 24%, and pulmonary hypertension (PH) in 40% of the SCD patients. The mean left atrial volume (LAV) was also increased in the SCD patients.
LV diastolic dysfunction (heart failure with preserved ejection fraction) and PH may complicate cases of the Arab-Indian haplotype of SCD.
镰状细胞病（SCD）是一种慢性遗传性血红蛋白疾病，与反复的血管阻塞性和溶血性危机以及慢性组织缺血相关，可能对任何器官系统产生不利影响。我们的目的是评估沙特阿拉伯东部省份原发性SCD患者的左心室（LV）收缩和舒张功能。
基于医院的前瞻性超声心动图研究，对象为青少年和成年SCD患者。
招募45例SCD患者进行超声心动图研究，同时选取45例年龄和性别匹配的患者作为对照。获取左、右心室尺寸、LV壁厚度、LV质量指数（LVMI）和LV收缩性变量。还估计左心房尺寸和容积以及肺动脉收缩压（PASP）。我们还评估LV舒张功能参数，包括二尖瓣早期和晚期血流速度（分别为E波和A波）、E/A比值、减速时间（MVDT）、A波持续时间（MVAD）、LV等容舒张时间（IVRT）以及组织多普勒速度，如侧方环e'波、a'波、e'/a'比值和E/e'比值。
SCD患者的LV尺寸、LV容积、每搏输出量和LVMI增加。前负荷增加（LV舒张容积），后负荷降低（低舒张压）。LVEF相当，不过有证据表明24%的SCD患者存在LV舒张功能障碍，40%的患者存在肺动脉高压（PH）。SCD患者的平均左心房容积（LAV）也增加。
LV舒张功能障碍（射血分数保留的心力衰竭）和PH可能使阿拉伯 - 印度单倍型SCD病例复杂化。
