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Left ventricular noncompaction

From SNPedia

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