AP-HP, Department of Cardiology, Henri Mondor Teaching Hospital, 51 Avenue Maréchal de Lattre de Tassigny, Creteil F-94000, France.
School of Medicine, Paris-Est University (UPEC), 61 avenue du Général de Gaulle, Créteil F-94000, France.
Eur Heart J. 2016 Apr 7;37(14):1158-1167. doi: 10.1093/eurheartj/ehv555. Epub 2015 Oct 29.
Cardiac involvement is common in sickle cell disease (SCD). Studies are needed to establish haematological determinants of this involvement and prognostic markers. The aim of the study was to identify haematological factors associated with cardiac involvement in SCD and their impact on prognosis.
This longitudinal observational study was performed on 1780 SCD patients with SS or S-β(0)-thalassemia referred to our centre. Six hundred fifty-six met our inclusion criteria (availability of a blood-workup and echocardiogram obtained <1 year apart, no heart valve surgery and no current pregnancy). Median age was 31 (interquartile range, 25-40) years, and median haemoglobin (Hb) was 87 (80-95)g/L. Left ventricular (LV) dilation, left atrial dilation, cardiac index (CI) >4 L/min/m(2), LV ejection fraction <55%, and tricuspid regurgitant velocity (TRV) ≥2.5 m/s were found in 35, 78, 23, 8.5, and 17% of patients, respectively. Compared with other patients, those in the fourth quartiles (Q4) of LV end-diastolic dimension index (LVEDDind) and left atrial dimension index (LADind) and those with high CI had significantly lower Hb, % foetal Hb (HbF), and red blood cell (RBC) counts; and significantly higher lactate dehydrogenase, bilirubin, and %dense RBCs. Independent haematologic determinants of Q4 LVEDDind and LADind were low RBC count and %HbF; high %dense RBCs were associated with LADind. Low %HbF and RBC count were associated with high CI. High %dense RBCs or no α-thalassemia gene deletion was associated with greater severity of anaemia and cardiac dilation and with higher CI. During the median follow-up of 48 (32-59) months, 50 (7.6%) patients died. Tricuspid regurgitant velocity ≥ 2.5 m/s was a predictor of mortality. The risk of death increased four-fold when left ventricular ejection fraction <55% was present also (P = 0.0001).
Cardiac dilation and CI elevation in patients with SCD are associated with haematologic variables reflecting haemolysis, RBC rigidity, and blood viscosity. Tricuspid regurgitant velocity ≥ 2.5 and LV dysfunction (even mild) predict mortality.
镰状细胞病（SCD）常伴有心脏受累。需要研究确定这种受累的血液学决定因素和预后标志物。本研究旨在确定与 SCD 心脏受累相关的血液学因素及其对预后的影响。
本研究为一项纵向观察性研究，纳入了 1780 名接受我院治疗的 SS 或 S-β（0）-地中海贫血合并 SCD 的患者。656 名患者符合纳入标准（在 1 年内进行了血液检查和超声心动图检查，且未行心脏瓣膜手术或当前未妊娠）。中位年龄为 31 岁（四分位距 25-40 岁），中位血红蛋白（Hb）为 87（80-95）g/L。左心室（LV）扩张、左心房扩张、心指数（CI）＞4 L/min/m2、LV 射血分数＜55%和三尖瓣反流速度（TRV）≥2.5 m/s 的患者分别占 35%、78%、23%、8.5%和 17%。与其他患者相比，LV 舒张末期内径指数（LVEDDind）和左心房内径指数（LADind）第 4 四分位数（Q4）的患者、CI 较高的患者 Hb、胎儿血红蛋白（HbF）%和红细胞（RBC）计数明显更低，乳酸脱氢酶、胆红素和 %致密 RBC 明显更高。LVEDDind 和 LADind 的 Q4 独立血液学决定因素是 RBC 计数低和 %HbF 高；高 %致密 RBC 与 LADind 相关。低 %HbF 和 RBC 计数与 CI 高相关。高 %致密 RBC 或无α-地中海贫血基因突变与贫血和心脏扩张严重程度增加以及 CI 高相关。在中位随访 48（32-59）个月期间，50（7.6%）名患者死亡。TRV≥2.5 m/s 是死亡的预测因素。当存在左心室射血分数＜55%时，死亡风险增加 4 倍（P=0.0001）。
SCD 患者的心脏扩张和 CI 升高与反映溶血、RBC 刚性和血液粘度的血液学变量相关。TRV≥2.5 和 LV 功能障碍（即使轻度）预测死亡。
