儿童心肌致密化不全的临床特点与预后分析

Clinical characteristics and prognosis of children with ventricular noncompaction

目的 探讨儿童心肌致密化不全的临床特点以及预后.方法 对2012年1月至2018年5月在中国医科大学附属盛京医院确诊为心肌致密化不全的34例患儿（依据年龄将患儿分为1岁以下的婴儿组和1岁以及1岁以上的年长组）的临床表现、实验室检查以及预后进行回顾性分析.结果心肌致密化不全的患儿平均年龄为3岁2个月,男女比例2.4：1,其中32例为左室心肌致密化不全,1例为右室心肌致密化不全,1例为双心室致密化不全.婴儿组和年长组相比,家族史阳性率、心律失常及血栓发生率无明显统计学差异.心功能不全为多数患儿的首诊原因,而年长儿多因乏力就诊.53％（18/34）患儿左室射血分数（left ventricular ejection fraction,LVEF）明显下降,且非致密化层与致密化层（non-compacted layer to compacted layer,N/C）比值和LVEF呈负相关（r=-0.74,P〈0.001）.65％的患儿心电图有不同程度的改变.随访中,1例患儿肺栓塞死亡.35％（12/34）患儿LVEF经治疗无明显改善.COX比例风险模型结果显示N/C比值是心肌致密化不全预后不良的独立危险因素（OR=14.46,95％CI 1.712-120.234,P〈0.05）.结论 儿童心肌致密化不全临床表现多样,预后不一,早期诊断和治疗、预防并发症以及定期随访较为重要.

Objective To investigate the clinical features and prognosis in ventricular noncompaction children. Methods Thirty-four cases who diagnosed with ventricular noncompaction were included in this study in Shengjing Hospital of China Medical University from January 2012 to May 2018. According to age, the children were divided into infantile type（ age 〈1 year old） and juvenile type（ age≥1 year old） . We ana-lyzed the clinical features,laboratory tests and prognosis. Results The average age at diagnosis was 3 years and 2 months. The sex ratio was 2. 4：1. Of these,32 cases were left ventricular noncompaction,1 was right ventricular noncompaction, and 1 was biventricular noncompaction. There were no statistically significant differences in family history,arrhythmia,and thrombotic events between infantile type and juvenile type. Heart failure was the first reason for most children,while older children often presented with fatigue when at diag-nosis. More than half of them showed significant left ventricular ejection fraction（LVEF） decreasing,and non-compacted layer to compacted layer（N/C） ratio showed negative correlation with LVEF（r= -0. 74, P〈0. 001）. Sixty-five percent（22/34） of the patients presented abnormal electrocardiogram. During the follow-up,one patient died of pulmonary embolism. No significant LVEF improvement was found in 35%（12/34） of the patient. The COX proportional hazards model showed that N/C ratio was an independent risk factor for poor prognosis of ventricular noncompaction（OR=14. 46,95%CI 1. 712 -120. 234,P〈0. 05）. Conclusion Children with ventricular noncompaction showed different clinical features and prognosis. Early diagnosis,treatment and long term follow up are key issues for the prognosis.