Electrocardiographic and clinical characteristics of idiopathic restrictive cardiomyopathy in children
Electrocardiographic and clinical characteristics of idiopathic restrictive cardiomyopathy in children
复制标题
DOI：
10.1253/circj.71.1534
复制
发表时间：
2007-10-01
影响因子：
3.3
通讯作者：
Echigo, Shigeyuki
中科院分区：
文献类型：
--
作者：
Hayashi, Tamaki;Tsuda, Etsuko;Echigo, Shigeyuki
背景特发性限制性心肌病(RCM)不是单一的疾病，是一种罕见的疾病。方法与结果回顾分析1978~2005年收治的12例儿童RCM患者的临床特点及病程。确诊年龄从4个月到12岁(中位数4岁)。7例因心电图异常确诊的患者年龄4~12岁。三名2岁以下的婴儿出现心脏增大。8例(67%)出现12导联ST-T段倾斜抬高和T波晚期峰形改变。3例运动中ST段压低的患者在放射性核素心肌灌注成像上无灌注缺陷。2例患者接受了原位心脏移植。其余10例患者中，7例已死亡：4例猝死，3例死于右心衰竭。3例左室壁肥厚的患者存活时间延长。1、2、3年存活率分别为78%、52%、26%。结论ST-T段倾斜抬高和T波晚峰型是心肌梗死的特征性改变，反映了限制性生理学改变，提示心肌复极异常。死亡方式为肺动脉高压所致心力衰竭或室性心律失常所致猝死。
Background Idiopathic restrictive cardiomyopathy (RCM) is not a single disease and is rare.Methods and Results The clinical features and clinical course of 12 pediatric patients with RCM seen between 1978 and 2005 were retrospectively analyzed. The age at diagnosis ranged from 4 months to 12 years (median 4 years). The age of 7 patients diagnosed because of an abnormal electrocardiogram (ECG) ranged from 4 to 12 years. Three infants less than 2 years old presented with cardiomegaly. Obliquely elevated ST-T segments and the late peak T waves on 12-lead ECG were present in 8 patients (67%). Three patients with ST depression during exercise had no perfusion defects on radioisotope myocardial perfusion imaging. Two patients underwent orthotopic heart transplantation. Of the remaining 10 patients, 7 have died: 4 died suddenly and 3 died of right heart failure. Three patients with a hypertrophic left ventricular wall had a prolonged survival. The probability of survival at 1, 2 and 3 years was 78%, 52% and 26%, respectively.Conclusions Obliquely elevated ST-T segments and the late peak T wave on ECG are characteristic, and reflect the restrictive physiology, which may indicate abnormalities of repolarization of ventricular muscle. The mode of death was either heart failure from pulmonary hypertension or sudden death from presumed ventricular arrhythmia.