Inherited Cardiac Muscle Disorders: Left Ventricular Noncompaction

Left ventricular noncompaction (LVNC) is a type of structural cardiac abnormality that displays genotypic and phenotypic heterogeneity. Morphologically, it is characterized by prominent ventricular trabeculae and deep intertrabecular recesses. It has traditionally been thought to be related to intrauterine arrest of myocardial development. While it is classified as a primary cardiomyopathy of genetic origin by the American Heart Association, the European Society of Cardiology classification defines it as an unclassified cardiomyopathy. Originally thought to be a rare disease seen mainly in children, there has been increasing identification in adults likely due to increased awareness and advances in cardiovascular imaging. Furthermore, although it is often associated with other congenital cardiac anomalies it can also be seen in association with dilated, hypertrophic and restrictive cardiomyopathies and in the absence of any cardiac defects.