Isolated non-compaction cardiomyopathy: A review

Abstract Left ventricular non-compaction cardiomyopathy (LVNC) is a rare disease which belongs to unclassified congenital cardiomyopathies. According to the ESC classification, LVNC is characterized by a spongy appearance of myocardium due to increased trabeculation and deep intertrabecular recesses that communicate with the left ventricle. This phenotype is thought to be caused by arrest of normal endomyocardial morphogenesis. Clinical manifestations of LVNC include heart failure, thromboembolic events, arrhythmias and/or sudden cardiac death. Progression of LVNC is highly variable and prediction of prognosis is very difficult. The aim of this paper is to provide an update about the topic of isolated LVNC.