ABIM, Internal Medicine and Cardiovascular Diseases, University of Southern California, Keck School of Medicine, Division of Cardiovascular Medicine, Los Angeles, CA 90033, USA.
Prog Cardiovasc Dis. 2010 Jan-Feb;52(4):264-73. doi: 10.1016/j.pcad.2009.11.001.
According to the World Health Organization classification of cardiomyopathies, left ventricular noncompaction is still an unclassified cardiomyopathy. In 2006, the American Heart Association classified this entity as a primary cardiomyopathy of genetic origin. In 2008, the European Society of Cardiology updated the classification scheme similar to the World Health Organization classification. At present, there is no consensus on the diagnostic criteria, and diagnosis is based on the morphologic features identified by cardiac imaging studies or at autopsy. Due to lack of standardization of the diagnostic criteria and little awareness of this condition among clinicians, the true prevalence of this disease is not clear. There is no specific therapy for this condition. However, it seems prognosis is much better than initially reported. The current status of diagnosis, prognosis, and management of isolated noncompaction in adults is discussed in this review.
根据世界卫生组织（WHO）的心肌病分类，左心室心肌致密化不全仍属于未分类的心肌病。2006 年，美国心脏协会（AHA）将这种病变归类为原发性遗传性心肌病。2008 年，欧洲心脏病学会（ESC）更新了类似的分类方案，与世界卫生组织的分类方案相似。目前，关于诊断标准尚无共识，诊断基于心脏影像学检查或尸检确定的形态学特征。由于诊断标准缺乏标准化，且临床医生对此病认识不足，因此该病的真实患病率尚不清楚。目前尚无针对这种疾病的特定疗法。然而，其预后似乎比最初报道的要好得多。本文就成人孤立性心肌致密化不全的诊断、预后和治疗现状进行了讨论。
