镰状细胞病患儿的心肌缺血
Myocardial ischaemia in children with sickle cell disease.
作者信息
de Montalembert M, Maunoury C, Acar P, Brousse V, Sidi D, Lenoir G
机构信息
Service de Pédiatrie Générale, Hôpital Necker Enfants Malades, 149 rue de Sèvres, 75015 Paris, France.
出版信息
Arch Dis Child. 2004 Apr;89(4):359-62. doi: 10.1136/adc.2003.027326.
BACKGROUND
The heart may be involved in children affected with sickle cell disease (SCD) via several mechanisms. Principally, chronic anaemia increases cardiac output and may cause left ventricular enlargement and cardiac insufficiency.
AIMS
To investigate whether the heart also suffers from ischaemia in SCD, as has already been shown for other organs (bone, brain, etc), and to look for risk factors predisposing to this complication.
METHODS
Twenty two children with SCD, and chest pain or ECG or echocardiographic signs (left ventricle dilation or hypokinesis) suggesting myocardial ischaemia were subjected to thallium-201 (201Tl) single photon emission computed tomography (SPECT).
RESULTS
Eight children had a normal SPECT, 14 an abnormal one. Myocardial perfusion defects were reversible in nine, fixed in five. Patients with perfusion defects tended to be older and have more severe disease. Five had had cardiac symptoms (episodes of cardiac failure in three, ventricular fibrillation in one, angina in one). Myocardial perfusion was reassessed after six months of hydroxyurea treatment in three patients, and was found to be improved.
CONCLUSIONS
Myocardial perfusion defects are present in children with SCD and may be demonstrated using SPECT. Hydroxyurea improved perfusion in three patients.
背景
患有镰状细胞病（SCD）的儿童心脏可能通过多种机制受累。主要是，慢性贫血会增加心输出量，并可能导致左心室扩大和心脏功能不全。
目的
研究SCD患儿的心脏是否也会像其他器官（骨骼、大脑等）一样出现缺血情况，并寻找易引发这种并发症的危险因素。
方法
对22例患有SCD且有胸痛或心电图或超声心动图征象（左心室扩张或运动减弱）提示心肌缺血的儿童进行铊-201（201Tl）单光子发射计算机断层扫描（SPECT）。
结果
8名儿童SPECT检查结果正常，14名异常。9例心肌灌注缺损为可逆性，5例为固定性。有灌注缺损的患者往往年龄较大且病情更严重。5例有心脏症状（3例出现心力衰竭发作，1例出现心室颤动，1例出现心绞痛）。3例患者在接受羟基脲治疗6个月后重新评估心肌灌注，发现有所改善。
结论
SCD患儿存在心肌灌注缺损，可通过SPECT显示。羟基脲改善了3例患者的灌注情况。
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