Department of Cardiovascular Medicine, Cleveland Clinic Foundation, Cleveland, Ohio.
Department of Internal Medicine, Cleveland Clinic Foundation, Cleveland, Ohio.
J Am Coll Cardiol. 2015 Aug 4;66(5):578-85. doi: 10.1016/j.jacc.2015.06.017.
Isolated left ventricular noncompaction (ILVNC) is a cardiomyopathy that was first described in 1926 as a "spongy myocardium." The disorder results from intrauterine arrest of compaction of the loose interwoven meshwork of the fetal myocardial primordium and subsequent persistence of deep trabecular recesses in the myocardial wall. The classical clinical presentation is a triad of heart failure, arrhythmias, and embolic events from mural thrombi. ILVNC has been associated with several autosomal dominant, X-linked, and mitochondrial genetic mutations that are also shared among other cardiomyopathies. Over the past decade, ILVNC has been subject to intensive research, as it increases the risk for sudden cardiac death. This review focuses on the current understanding of ILVNC in adult populations and attempts to provide organized insight into the disease process, screening, diagnosis, management, role of device therapy, and prognosis.
孤立性左室心肌致密化不全（ILVNC）是一种心肌病，于 1926 年首次被描述为“海绵状心肌”。该病是由于胎儿心肌原基疏松交织网致密化过程中的宫内阻滞，以及随后心肌壁中深小梁隐窝的持续存在所致。典型临床表现为心力衰竭、心律失常和来自壁血栓的栓塞事件三联征。ILVNC 与几种常染色体显性遗传、X 连锁遗传和线粒体基因突变有关，这些基因突变也存在于其他心肌病中。在过去的十年中，ILVNC 一直是研究的热点，因为它会增加心源性猝死的风险。本综述重点介绍了成人人群中 ILVNC 的最新认识，并试图对疾病过程、筛查、诊断、管理、装置治疗的作用和预后提供系统的见解。
