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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患儿临床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2~10 cm,剑突下6~10 cm)。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-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 To investigate the clinical features, diagnosis points and treatment outcome of children with primary restrictive cardiomyopathy (RCM). Methods The clinical data of 5 patients with primary RCM admitted to the First Affiliated Hospital of China Medical University from 2004 to 2013 were analyzed retrospectively. Results The group of 5 patients, 3 males and 2 females; aged 6 to 12 years; pre-treatment course of 10 months to 6 years. The main symptoms were fatigue in 5 cases, palpitations, shortness of breath in 4 cases, syncope in 1 case, sudden sleep difficulty in the night, 1 case of acute abdominal pain with vomiting in 2 cases. Important signs of cheek dark red were mitral valve surface in 4 cases, heart expansion in 4 cases, Ⅱ ~ Ⅳ anterior heart area systolic murmur in 3 cases, 1 in 3 cases, the liver increased in 5 cases (right rib 2 to 10 cm, xiphoid 6 ~ 10 cm). Echocardiography: left and right atrial enlargement in 5 cases, 5 cases of ventricular filling confined, tricuspid regurgitation in 1 case and moderate to severe reflux in 2 cases, widened inferior vena cava in 5 cases, pulmonary pressure mild to moderately elevated High in 3 cases, with left ventricular systolic function reduced in 1 case. ECG around the atrium to expand in 5 cases, myocardial involvement (ST segment down) in 4 cases. As far as the heart of the three showed heart shadow increased in 5 cases, the ratio of 0.65 ~ 0.78 heart, lungs increased, blurred. The group used drug therapy (captopril, metoprolol, furosemide, spironolactone, myocardial nutrition drugs, etc.), can temporarily alleviate the condition, but limited. Currently 5 cases are still under treatment. Conclusions Most RCM are involved in the double compartment and dominated in the right ventricle. Clinical manifestations resemble constrictive pericarditis, with prominent signs of pulmonary venous and venous congestion. The current real-time two-dimensional color Doppler echocardiography is still an important evidence of diagnosis. The disease drug treatment is limited, the prognosis is poor.