Stanford Center for Inherited Cardiovascular Disease

Left Ventricular Non-Compaction (LVNC)

Left ventricular non-compaction (LVNC) is a rare genetic disease of the heart muscle. Compaction is a process that occurs during the development of the heart when a baby is in the womb. If this process is not complete, the inside of the heart muscle (the one in contact with the blood) will look spongy or “trabeculated”. Normally, it is smooth. Originally, non-compaction was diagnosed very rarely. It seemed to be associated with dangerous heart rhythms and, often, severe weakness of the heart muscle.

More recently, newer imaging techniques with higher resolution mean that we notice trabeculation in the heart much more often. As such, many patients who will have a much less severe disease course are diagnosed with this condition. Sometimes the term “hyper-trabeculation” is used to distinguish the finding of sponginess from the condition known to cause dangerous rhythms and heart muscle weakness.

Newer genetic tests have also revealed that in many cases where hyper-trabeculation is prominent, mutations of the same genes that cause hypertrophic cardiomyopathy can be found, suggesting some overlap between these conditions. Medications can be used to treat the dangerous rhythms or heart muscle weakness associated with LVNC.

Sometimes, if the trabeculations are deep, a blood thinning medication is used. In some cases, implantation of a defibrillator may be required to treat dangerous rhythms. In very severe cases, heart transplantation is sometimes required but this is rare.

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