我国心肌致密化不全的临床特征分析

目的了解我国心肌致密化不全（NVM）的发病现状和临床特征，提高对NVM的诊疗水平。方法通过中国期刊全文数据库检索出1994年1月至2007年7月国内报道有关NVM病例数10例以上的文献10篇（共183例），同时收录2005年2月至2007年10月先后在我院收治的11例NVM病患者，共194例进行回顾性分析。结果NVM可发生于任何年龄阶段，男性明显多于女性；该病多数单独存在，也可合并其他心脏畸形，具有一定家族发病倾向，可能与X染色体隐性遗传或常染色体显性遗传有关；病变多累及左心室，主要临床表现为渐进性的心功能障碍、心律失常及栓塞；超声心动图和磁共振在该病的诊断中具有重要价值。结论NVM是我国重要的心肌病之一，应予以高度重视，早期诊断及治疗可明显改善预后。

An investigation of noncompaction of ventricular myocardium in China

Objective To review the present situation and clinical characteristics of noncompaction of ventrieular myocardium （NVM） in China. Methods A total of 194 patients with NVM, including 183 cases reported by domestie documents from January 1994 to July 2007 by searching the Chinese Journal Full-text Database and 11 cases registered by the People＇s Hospital of Guangxi Autonomous Region from February 2005 to October 2007, were included in this review. Results NVM occurred from infancy to geriatric, with a higher incidence in males than in females. The majority of the patients had isolated NVM, whereas some cases had NVM accompanied with other congenital cardiovascular malformations. Patients with NVM had a familial cluster tendency, maybe associated to the X-chromosome recessive inheritance or autosomal dominant inheritance. Although left ventrieular apex and inferior wall were most frequent involved in patients with NVM, involvement of other left ventrieular wall and right ventricle had also been reported. The major clinical manifestations were congestive heart failure, arrhythmias and thromboembolism. Eehocardiography and Magnetic Resonance Imaging had important value in establishing diagnosis. Conclusion NVM is an important type of cardiomyopathy in China, and it should be paid more attention. Early diagnosis and treatment without delay are critical to improve the prognosis in these patients.