儿童原发性限制型心肌病5例临床特征及诊治探讨

Clinical characteristics and diagnosis and treatment of children's primary restrictive cardiomyopathy

目的探讨儿童原发性限制型心肌病（RCM）的临床特征、诊断要点和治疗转归。方法对2004--2013年中国医科大学附属第一医院收治的5例原发性RCM患儿}I钏秉资料进行回顾性分析。结果本组5例中，男3例、女2例；年龄6～12岁；来诊前病程10个月至6年。主要症状有乏力5例，活动后心悸、气短4例，发生晕厥1例，夜间睡中突发呼吸困难1例，急性腹痛伴呕吐2例。重要体征有面颊暗红色呈二尖瓣面容4例，心界扩大4例，心前区Ⅱ．Ⅳ级收缩期杂音3例，三音律1例，肝脏增大5例（右肋下2—10cm，剑突下6～10cm）。心脏超声检查：左右心房扩大5例、心室充盈受限5例、三尖瓣中度返流1例及中一重度返流2例，下腔静脉增宽5例，肺动脉压力轻至中度升高3例，伴左室收缩功能减低1例。心电图示左右心房扩大5例，心肌受累（ST段下移）4例。心脏远达三位像示心影增大5例，心胸比值0．65～0．78，肺纹理增多、模糊。本组采用药物治疗（卡托普利、美多洛尔、呋噻米、螺内酯、心肌营养药等），能使病情暂时缓解，但作用有限。目前5例仍在治疗随访中。结论RCM多为双室受累且以右室为主。临床表现酷似缩窄性心包炎，以肺静脉和体静脉淤血引发征象为突出。目前实时二维彩色多普勒超声心动图检查所见仍然是诊断的重要依据。本病药物治疗效果有限，预后不良。

Objective Discuss the clinical characteristics, diagnosis and therapy of children＇s primary restrictive cardio- myopathy. Methods Make a retrospective analysis of clinical data of 5 children with the disease who were admitted to hospital between 2004 and 2013. Results Five children（3 males and 2 females） aged 6 - 12 years-old were included in the study, whose courses were from 10 months to 6 years before visiting our hospital.The main symptoms were as follows ： 5 had fatigue, 4 had short breath and palpitation after activity, 1 syncope, 1 nocturnal paroxysmal dyspnea, 2 acute stomachache and vomit. Important signs were as follows ： 4 had mitral faces, 4 had cardiac enlargement, 3 systolic murmur of II - IV levels in precordium, 3 triple rhythm, 5 hepatomegal （2 - 10 cm below the fight costal margin, 6 - 10 cm below xiphoid process）. Echocardiography showed： 5 had enlargement of two atriums, 5 had restricted ventricular filling, 1 moderate tricuspid re- gurgitation, 2 moderate-severe, 5 inferior vena cava broadening, 3 light-moderate pulmonary hypertension, 1 left ventricular systolic dysfunction （EF46%）.ECG showed ： 5 enlargement of two atriums, 4 myocardial i schemia （ST segment moving down ）. X-rays showed increased heart shadow, cardiothoracic ratio 65% - 78%, increased and blur pulmonary markings.all the sick children received medicine treatment （captopril, metoprolol, furosemide, spironolactone, myocardial nutritional drugs, etc）, and symptoms could be relieved temporarily. Five children were still in follow-up. Conclusion In restrictive cadiomyopathy, usually two ventricles are involved and mainly right ventricle.Clinical manifestations are like constrictive pericarditis, and main features are congestion of pulmonary vein and systemic veins.Now real-time enchocardiography is still the main di- agnostic basis. Therapeutic effect of drugs is limited and the disease has bad Droznosis.