Left ventricular noncompaction

Non-compaction cardiomyopathy (NCC), also called spongiform cardiomyopathy, is a rare congenital cardiomyopathy that affects both children and adults. It results from the failure of myocardial development during embryogenesis. During development, the majority of the heart muscle is a sponge-like meshwork of interwoven myocardial fibers. As normal development progresses, these trabeculated structures undergo significant compaction that transforms them from spongy to solid. This process is particularly apparent in the ventricles, and particularly so in the left ventricle. Noncompaction cardiomyopathy results when there is failure of this process of compaction. Because the consequence of non-compaction is particularly evident in the left ventricle, the condition is also called left ventricular noncompaction. Other hypotheses and models have been proposed, none of which is as widely accepted as the noncompaction model. Symptoms range greatly in severity. Most are a result of a poor pumping performance by the heart. The disease can be associated with other problems with the heart and the body.Wikipedia

The following types of left ventricular noncompaction (LVNC's) are recognized by OMIM, along with their associated genes and modes of inheritance (AD = autosomal dominant; XLR = X-linked recessive):

Locus (OMIM)	Gene	Inheritance
LVNC1	DTNA	AD
LVNC3	LDB3	AD
LVNC4	ACTC1	AD
LVNC5	MYH7	AD
LVNC6	TNNT2	AD
LVNC7	MIB1	AD
LVNC8	PRDM16	AD
LVNC9	TPM1	AD?
LVNC10	MYBPC3	AD
Barth	TAZ	XLR
?	OBSCN	AD