Stanford Center for Inherited Cardiovascular Disease

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)

Arrhythmogenic right ventricular cardiomyopathy/dysplasia is also known as arrhythmogenic right ventricular dysplasia, arrhythmogenic right ventricular dysplasia/cardiomyopathy, ARVD, ARVD/C and arrhythmogenic cardiomyopathy, AC.

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a rare disease of the heart muscle that can be associated with dangerous heart rhythms. It is caused by abnormalities of structures that connect heart muscle cells together. These structures are known as desmosomes.

As the disease progresses, the heart muscle especially on the right of the heart becomes replaced by fatty tissue. This is known as “dysplasia”, which can cause dangerous heart rhythms, and leads in some cases to heart muscle weakness (“cardiomyopathy”). It is a genetic disease that runs in families. The gene mutation causing the condition can be identified about half the time.

Symptoms of ARVC/D include a strong or irregular heart beat (palpitations), chest pain or shortness of breath. Investigations include an electrical tracing of the heart (electrocardiogram), an ultrasound (or echocardiogram), an exercise test on a treadmill or bike, and a magnetic resonance imaging scan (MRI). Also, two longer kinds of heart rhythm monitor are used. On or before the first visit, a family pedigree is drawn.

Genetic testing is usually performed for ARVC/D. Treatments for ARVC/D include medications, implantation of a defibrillator (ICD) to protect against dangerous heart rhythms or in very rare cases, heart transplantation.

At the Stanford Center for Inherited Cardiovascular Disease, we have a special interest in patients and families with ARVC/D.

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