Left Ventricular Non-Compaction Cardiomyopathy

Left Ventricular Non-Compaction Cardiomyopathy (LVNC) is a relatively new subtype of cardiomyopathy being recognized in children. Known as non-compaction cardiomyopathy (NC), isolated ventricular non-compaction cardiomyopathy (IVNC) and spongiform cardiomyopathy, it is a rare form estimated to affect about 1.2 per million children under the age of 10.

In this form of cardiomyopathy, the heart muscle appears to have a spongy or non-compacted appearance. Because the heart muscle has not compacted like it should have, deep grooves (trabecular recesses or trabeculations) appear in the muscle wall of the heart’s main pumping chamber (left ventricle). Non-compaction can present with normal heart function or with heart dysfunction. With LVNC, the heart muscle may not squeeze normally and there may be poor contraction and/or poor filling of the heart.

Causes of Left Ventricular Non-Compaction Cardiomyopathy

With LVNC, there is a failure of the heart to transform from spongy muscle fibers into compacted solid muscle layers. What causes this disruption in heart development in the womb is still unknown. LVNC has been classified as genetic in origin and several genes linked to other forms of cardiomypathy are also thought to be associated with this condition.

Causes

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