Arrhythmogenic Right Ventricular Cardiomyopathy

Arrhythmogenic right ventricular cardiomyopathy (ARVC), also known as arrhythmogenic right ventricular dysplasia, right ventricular dysplasia and right ventricular cardiomyopathy, is the least common type of cardiomyopathy in children. The prevalence of ARVD has been estimated at 1 to 2,500. Typically the condition presents in teens or young adults; it is extremely uncommon in children under the age of 10. The disease is more common in males than females.

With ARVD, there is a problem with the connections between heart cells, and damaged heart cells are replaced with scar tissue and fat. The resulting scar tissue leads to abnormal electrical activity (arrhythmias) and problems with the heart's contraction. In the early stages of the disease, the right side of the heart may appear to be thickened in certain patches but later becomes dilated with a thinner wall. Eventually, the muscles in the left side of the heart are also reduced and thin layers of fat and fibrous tissue appear.

As a result of these structural changes, there may be abnormal heart rhythms and weakening of the heart’s pumping function. Those with ARVC are at a higher risk for life-threatening arrhythmias (ventricular fibrillation) and sudden cardiac arrest.

Causes of ARVC

The exact cause of ARVC is not always known, but it is believed to be an inherited disease. In 30-50 percent of cases, the disease runs in the family. ARVC also can occur secondary to Naxos syndrome and Carvajal syndrome, rare disorders that affect the skin, hair and nails.

Left Ventricular Non-Compaction Cardiomyopathy

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