Noncompaction cardiomyopathy

Noncompaction cardiomyopathy (NCCM) is a genetic myocardial disorder, which is characterized by a two-layered ventricle wall with a thin compact outer layer and a noncompacted inner layer, with prominent trabeculations and deep intratrabecular recesses communicating with the ventricle cavity without any contact to the coronary system. Before the initial description as isolated left ventricle cardiomyopathy (ILVCN) in 1984 by Engberding and Bender, the morphological characteristics had been described only in association with other congenital cardiac disorders, such as atresia of the semilunar valves. The disease usually involves the myocardium of the left ventricle but involvement of the right ventricular has recently been shown. Due to delayed diagnosis and therapy, in advanced stages NCCM can result in heart failure. Life-threatening complications, such as malignant arrhythmia with sudden cardiac death and embolic events have been observed in patients with NCCM. A multimodal investigation including echocardiography and cardiac magnet resonance tomography (CMR) as well as a focused analysis of symptoms can allow a valid diagnosis.