高原地区小儿右室肥厚型心肌病的临床特点及病因初探

CLINICAL MANIFECTATIONS AND ETIOLOGICAL EVELUATION OF RIGHT VENTRICULAR HYPERTOTRIC CARDIOMYOPATHY OF CHILDREN LIVING AT HIGH ALTITUDE

目的：为提高对高原地区小儿右室肥厚型心肌病（RHCM）的认识和初步探讨可能的病因;方法：对高原地区20例小儿RHCM的临床、ECG、胸片、超声心动图（UCG）及1例尸解资料进行分析，其中UCG各参数与正常值比较，20例中10例估测肺动脉收缩压（PASP）、RPEP／AT与10例高原性心脏病（HACD）、20例健康儿比较。10例血硒含量与10例健康儿比较;结果：其特点如下：①发病年龄为8.75±4.85月，男性多见（75％）;②临床表现为充血性心力衰竭;③胸片及ECG无确诊意义;④UCG示右室及室间隔明显肥厚（P＜0.001），部分合并左室肥厚（P＜0.001），不伴有肺动脉高压（P＜0.001），右室舒张功能受损A／E＞1（95％）;⑤合并右定扩大者预后差（病死率为20％）;结论：10例血硒含量偏低0.061±0.021mg／L（P＜0.05），初步推测发病与低硒有关。

To improve the clinical recognition of right ventricular hypertrophic cardiomyopathy (RHCM)of children living at high altitude and evaluate the probable etiology. Metheds:The data of clinical findings chest radiogiaph,ECG and echocardiography in 20 patients and autopsy of 1 of them were examined. Figures of UCG were compared with mormal figures. PASP,RPEP/AT from 10 of 20 cases. 10 cases with high altitude heart desease and 20 of normal children were contrasted. Blood-selenium of 10 cases and 10 cases of health children was measssured. Results and Conclution: The characterastic was as following as 1 the age was 8.75±4.85M, mainly male (75%) ;2 Clinical presentation was congestive heart failure; 3 Chest picture and ECG manifectations had no decisive diagnostic value;4 UCG showed there were RV and IVS significant hypertrophy (P<0.001 ),partly associating with LV hypertrophy (55% ), RV diastolic disfuction A/E >1(95% )without high pulmonary artery pressure; 5 RHCM with Dialated RV was linked with poor prognosis (mortality rate 20%). Low level of blood-selenium could be the etiology of RHCM on the basis of the figure from 10 cases of RHCM (0.061±0.021mg/L).