儿童心肌致密化不全20例临床分析

Clinical analysis of noncompaction of ventricular myocardium in 20 children

摘要:

目的：探讨儿童心肌致密化不全（noncompaction of the ventricular myocardium，NVM）的临床特点及治疗效果。方法：对2007年9月至2012年11月期间收治的20例NVM患儿的临床表现、辅助检查（胸部X线片、心电图、超声心动图、心脏磁共振等）、临床治疗以及随访结果等资料进行回顾性分析，并对部分患儿进行随访。结果：20例NVM患儿中，有家族史者2例，以心功能不全为主要临床表现者16例（80%），心律失常10例（50%），无血栓及血栓事件发生；胸部X线检查示心脏扩大者12例（60%），20例患儿超声心动图均提示心脏NVM典型表现，其中单纯左室病变17例，双室病变2例，合并先天性心脏病9例（45%），合并有扩张型心肌病（dilated cardiomyopathy，DCM）3例（15%）及心内膜弹力纤维增生症（endocardial fibroelastosis，EFE）4例（20%）；20例NVM首次住院治疗后，16例好转出院，4例放弃治疗；20例NVM患者平均随访7.86个月（1~15月），其中5例（5%）死亡，主要死因为心力衰竭加重；8例长期随访者心功能不全的临床症状明显缓解，改良Ross评分较前降低，定期复查心脏彩超提示心功能改善尚可，左室射血分数（left ventricular ejection fraction，EF）改善明显，差异有统计学意义（t=-3.248，P=0.018）；左室短轴缩短率（shortening fraction，FS）、收缩末期左室内径（left ventricular end diastolic diameter，LVED）、收缩末期右室内径（right ventricular end diastolic diameter，RVED）及非致密化心肌厚度/致密化心肌厚度比值（ratio of noncompacted to compacted myocardial layers，N/C）差异均无统计学意义（t=-1.348，P＝０.２２６；t=0.343，P=0.744；t=0.969，P=0.370；t=0.467，P=0.657）。结论：儿童NVM以心功能不全为主要表现，超声心动图是诊断NVM的主要手段；NVM在经积极内科对症治疗后临床症状可得到缓解，短期心脏功能改善可，但总体预后较差。

Abstract:

Objective：To investigate the clinical features and therapeutic effects of noncompaction of ventricular myocardium（NVM） in children. Methods：From September 2007 to November 2012，20 cases of NVM admitted in the Children’s Hospital of Chongqing Medical University were retrospectively analyzed. The retrospective analysis was carried out according to clinical features，auxiliary examinations（chest X-ray，electrocardiogram（ECG），ultrasonic cardiogram（UCG） and cardiac magnetic resonance（CMR），etc），clinical treatments and follow-up results，etc. Results：Among the 20 cases，2 cases had family history，16 cases（80%） showed cardiac insufficiency，10 cases（50%） demonstrated arrhythmia，and thrombosis or embolic events did not occur during this time. Chest X-ray of 12 cases（60%） showed cardiomegaly. Typical changes of NVM were seen in UCG of all patients：17 cases had only left ventricular pathological changes，2 cases had double ventricular pathological changes，9 cases（45%） had combination of congenital heart disease，3 cases（15%） had combination of dilated cardiomyopathy（DCM），4 cases（20%） had combination of endocardial fibroelastosis（EFE）. After all 20 children being treated in the hospital for the first time，16 children were cured and discharged from the hospital and 4 children gave up the treatment. The average follow-up time of the 20 patients was 7.86 months（1-15 months）. Five children died of the aggravation of heart failure. During the follow-up，symptoms of 8 children were significantly alleviated and modified Ross score was lower than that before treatment. Regular UCG recheck showed that the cardiac function was improved well and ejection fraction of left ventricular can be seen by UCG successfully after the treatment，with statistic significances（t=-3.248，P=0.018）. No statistical difference in shortening fraction of left ventricular，left ven－tricular end diastolic diameter，right ventricular end diastolic diameter and ratio of noncompacted to compacted myocardial layers after the treatment was observed（t=-1.348，P＝０.２２６；t=0.343，P=0.744；t=0.969，P=0.370；t=0.467，P=0.657）. Conclusion：Cardiac insuffi－ciency with different degrees is the chief complaint of NVM patients. The main auxiliary examination for the diagnosis of NVM is color Doppler electrocardiography. With active internal medicine treatment，clinical symptoms and left ventricular dysfunction can be greatly improved in short-term，but the long-term follow-up shows a poor prognosis in conclusion.