Noncompaction cardiomyopathy

Non-compaction cardiomyopathy (NCC), also called spongiform cardiomyopathy.

A rare congenital cardiomyopathy affecting both children and adults.

Due to failure of myocardial development during embryogenesis.

During embryologic development, the heart muscle is a sponge-like meshwork of interwoven myocardial fibers that undergo compaction that transforms them from spongy to solid tissue.

The process is most evident in the ventricles, and particularly the left ventricle, when there is failure of this process of compaction.

Noncompaction cardiomyopathy results when there is failure of this process of compaction.

Symptoms vary greatly but result of a poor pumping performance by the heart.

In noncompaction there is excessively prominent trabeculations, and is diagnosed when the trabeculations are more than twice the thickness of the underlying ventricular wall on echocardiographic studies.

A genetic cardiomyopathy, with mutations in LDB3 having been described, and has been seen in combination with 1q21.1 deletion syndrome.

Estimated incidence of 0.12 cases per 100,000.

Characterized anatomically by deep trabeculations in the ventricular wall.

Clinically changes include systolic and diastolic dysfunction, associated at times with systemic embolic events.

Associated symptoms vary widely from asymptomatic to severe heart failure.

May be diagnosed in patients still in an asymptomatic state.

The process is present from birth, may only manifest itself later in life.

Adults more likely to suffer from heart failure than children.

Common symptoms associated with a reduced pumping performance of the heart include those associated with heart failure.

High likelihood of misdiagnosis.

Long-term prognosis unknown as many are asymptomatic.

Management is similar to management programs for other types of cardiomyopathies which include the use of ACE inhibitors, beta blockers and aspirin therapy, and installation of pacemaker for those thought to be at a high risk of arrhythmia problems.

In severe cases with heart failure, a heart valve operation, or a heart transplant may be required.

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