儿童心肌致密化不全20例临床分析

发布时间：2019-04-21 12:59

【摘要】：目的：探讨儿童心肌致密化不全（noncompaction of the ventricularmyocardium,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（X线胸片、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11例（55%），合并有扩张性心肌病（dilatedCardiomyopathy,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月-15月），其中5例死亡，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（leftventricular ejection fraction，EF）改善明显，差异有统计学意义（P＜0.05）；左室短轴缩短率(Shortening Fraction, FS)较治疗前增加，收缩末期左室内径（left ventricular diameter，LVD）、右室内径（right ventricular diameter,RVD）及非致密化心肌厚度/致密化心肌厚度比值（ratio of noncompacted to compacted myocardiallayers，NC/C）较治疗前缩小，但差异均无统计学意义（P＞0.05）。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 myocardial densification in children (noncompaction of the ventricularmyocardium,NVM). Methods: from September 2007 to November 2012, the clinical manifestations and auxiliary examination (X-ray chest film, electrocardiogram, echocardiography, cardiac magnetic resonance, etc.) of 20 children with NVM were analyzed. Clinical treatment and follow-up results were retrospectively analyzed, and some children were followed up. Results: of the 20 children with NVM, 2 had family history, 16 (80%) had cardiac insufficiency, 10 (50%) had arrhythmias, and no thrombus and thrombus events occurred. Chest X-ray examination showed cardiac enlargement in 12 cases (60%). Echocardiographic findings in 20 cases showed typical manifestations of cardiac NVM, including 17 cases of simple left ventricular lesions, 2 cases of biventricular lesions, and 11 cases of congenital heart disease (55%). There were 3 cases (15%) with dilated cardiomyopathy (dilatedCardiomyopathy,DCM) and 4 cases (20%) with endocardial elastosis (endocardial fibroelastosis,EFE). After the first hospitalization of 20 cases of NVM, 16 cases were improved and 4 cases gave up treatment, and 20 cases of NVM were followed up for an average of 7.86 months (January-15 months), 5 of them died due to the aggravation of heart failure. The clinical symptoms of 8 long-term follow-up patients with cardiac insufficiency were relieved obviously, and the modified Ross score was lower than that before. Regular reexamination of heart color Doppler suggested that cardiac function could be improved, and left ventricular ejection fraction (leftventricular ejection fraction,EF) could be improved obviously, and left ventricular ejection fraction (LVEF) was improved. The difference was statistically significant (P < 0.05). Left ventricular short axis shortening rate (Shortening Fraction, FS) increased before treatment, and left ventricular inner diameter (left ventricular diameter,LVD), right ventricular diameter (right ventricular diameter,RVD) and undensified myocardial thickness / densified myocardial thickness ratio (ratio of noncompacted to compacted myocardiallayers,) were increased at the end of systolic period. NC/C was smaller than that before treatment, but there was no significant difference (P > 0.05). Conclusion: the main manifestation of NVM in children is cardiac insufficiency, and echocardiography is the main method to diagnose NVM. The clinical symptoms of NVM can be relieved after active medical treatment, and the short-term cardiac function can be improved, but the overall prognosis is poor.
【学位授予单位】：重庆医科大学
【学位级别】：硕士
【学位授予年份】：2013
【分类号】：R725.4

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