成人心肌致密化不全的研究进展

Study on Noncompaction of Ventricular Myocardium in Adults

成人心肌致密化不全(NVM)是一种特殊少见的心肌病,是由于胚胎发育早期正常心内膜发育停滞所致的心脏病,特征是心内膜面有粗大的肌小梁和交错的深隐窝。临床表现为充力性心力衰竭、心律失常和系统性血栓栓塞等。目前以对症支持治疗为主,预后较差。现介绍其发病机制、病理解剖学、临床特点、诊断及诊断标准、治疗等方面进展,为临床诊断及治疗提供客观依据。近年来医师对该疾病认识的不断提高,使其误诊率和漏诊率有所下降,同时使患者的预后和生活质量得到明显改善,但还存在许多争议,需要进一步探讨。

Noncompaction of the ventricular myocardium （NVM） in adults is an uncommon cardiomyopathy, due to normal endocardial development stagnation in the early embryonic development, with characteristics of the thick trabeculae and staggered deep crypts on the endocardial surface, and clinical manifestations of congestive heart failure, arrhythmias, and systemic thromboembolism. At present, NVM is mainly given symptomatic and supportive treatment, with a poor prognosis. This article introduces its pathogenesis, pathological anatomy, clinical features, diagnostic criteria, and treatment progress in order to provide an objective basis for clinical diagnosis and treatment. In recent years, physicians have increased understanding of the disease and made its rate of misdiagnosis and missed diagnosis declined, and markedly improved the prognosis and quality of life of patients.But there are still a lot of controversy about NVM, which needs to be further explored.