Echo learning : NON COMPACTION CARDIOMYOPATHY VIDEOS

**Medical Videos** · 12-28-2012 04:30 PM

Non-compaction cardiomyopathy is a rare congenital cardiomyopathy that affects both children and adults.[1] 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.

The diagnosis of LVNC is based on cardiac testing, family history, medical history, and physical exam.

Cardiac testing: An echocardiogram is the most common test used to diagnose LVNC, as the trabeculations within the left ventricle and overall squeeze of the heart can be measured with this test.
Family history: A family tree should be constructed with specific attention to cardiomyopathy, rhythm problems, sudden cardiac or unexplained death, cardiac surgery or presence of other cardiac disease in relatives.
Medical history: A history of heart failure symptoms including feeding issues, heart rhythm problems and exercise intolerance.
Physical exam: A thorough physical exam will be done to evaluate for signs of a cardiac problem. Assessment of skeletal muscle strength will be performed since problems with skeletal muscle may occur with cardiac muscle disease.