我国心室肌致密化不全的荟萃分析

Meta-analysis of noncompaction of ventricular myocardium in China

目的了解我国心室肌致密化不全(noncompactionofventricularmyocardium,NVM)的发病现状及其临床特征、诊治方法,为临床进一步认识和诊治NVM提供依据。方法利用中国期刊全文数据库(CN-KI)、重庆维普(VIP)和万方数据库检索1989年1月～2006年6月国内报道的NVM文献81篇计300例,结合该院收治的1例共计301例病例进行分析。结果NVM可发生于任何年龄,多见于中青年。男性发病率明显高于女性。临床表现主要为进行性的心力衰竭(67.1%),其次为心律失常(9.3%)和栓塞。病变累及心脏的发生率依次为单独累及左室(82.4%)、左右室均累及(9.6%)和单独累及右室(8.0%)。35例(11.2%)合并其他先天性心脏畸形。13.9%患者具有家族遗传性。82.1%的患者曾被误诊为其他疾患,主要误诊为扩张型心肌病(69%)。经胸心脏超声检查是主要的诊断方法,但核磁共振(MRI)等检查对于部分病例同样重要。结论NVM是一种少见的未分类心肌病,有独特的病理和影像学改变,误诊率极高。应重视和充分认识其特征以提高临床诊治NVM水平。

【Objective】To explore the present situation, clinical characteristics and methods of diagnosis of non-compaction of ventricular myocardium （NVM） in China. 【Methods】300 cases reported by civil documents （81 docu-ments） from January 1989 to June 2006 by searching the Chinese full text database of CNKI, VIP and wanfang, and 1 case diagnosed by our hospital in 2006 were included in present study. Retrospective meta-analysis was done. 【Results】The age of onset of clinical manifestation of NVM ranged from infancy to geriatric age. Incidence in male was higher than in females. The common clinical presentations were chronic congestive heart failure （67.1%）, ar-rhythmia （9.3%） and thromboembolism. 27 cases were asymptomatic. Though NVM occurred in left and / or right ventricular, the most frequent sites affected were left ventricle only （82.4%）, involvement of right ventricle solo and both ventricular wall occurred in 24 （8%） and 29 （9.6%） cases, respectively. 35 cases （11.2%） complicated with oth-er congenital heart malformations. Familial NVM were discovered in 42 cases （13.9%）. Abnormal electrocardiogram （ECG） was showed in 298 out of 301 cases, included ventricular premature beats and tachycardia in 90 cases （30.2%）. 87 out of 106 cases （82.1%） were misdiagnosed, most cases were misdiagnosed as idiopathic diluted car-diomyopathy （69%）. Echocardiography had important value in establishing the diagnosis of NVM. 【Conclusion】 NVM is a rare cardiomyopathy which has characteristic echocardiographic and MRI manifestations and specific pathologic changes that could distinguish from other cardiomyopathy.