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Arrhythmogenic right ventricular cardiomyopathy (ARVC)

What is ARVC?

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic heart condition that usually affects the right-side of the heart, but can affect the left-side, or both sides of the heart in some patients. In ARVC, part of the heart muscle is abnormal and replaced by fatty tissue and scar. ARVC may cause heart rhythm disturbances, or result in weakening of the heart muscle pumping function.

What are the Symptoms of ARVC?

Many patients with ARVC have no or minimal symptoms. The symptoms of ARVC may include palpitations, blackouts (or near-blackouts), and shortness of breath. Uncommonly, ARVC can cause an abnormally fast heart rhythm (ventricular tachycardia or fibrillation) that can cause collapse, or rarely, sudden death.

How is ARVC diagnosed?

ARVC may be seen on cardiac ultrasound (echocardiography). However, if you are thought to have ARVC, it is likely you will need to undergo further tests. These may include a cardiac magnetic resonance imaging scan (cardiac MRI); a cardiac electrophysiologic study (EPS); and/or a Holter monitor (24 hour ECG).

Family Screening and Genetic Testing

ARVC is caused by a problem with a person’s genes. It may be inherited and the pattern of inheritance is known as “autosomal dominant.” Autosomal dominant conditions are important in families, since there may be more than 1 affected person in each family. All first-degree relatives (parents, siblings and children) of patients with ARVC should be checked for the condition. Children of affected parents have a 1 in 2, or 50% chance, of inheriting the condition.

Genetic testing involves a blood test. Currently, genetic testing is able to identify the cause of ARVC in approximately 40-60% patients. A positive genetic test result in conjunction with clinical screening can be used to detect ARVC in family members.

How is ARVC Treated?

There is currently no treatment to “cure” the disease. However, most of the problems occurring in those with ARVC can be managed with the treatments below.

Lifestyle Modifications:

Since intense exercise may lead to heart rhythm disturbances, patients with ARVC should not participate in competitive sports or in high-risk activities where loss of consciousness may lead to harm, for example, scuba diving, rock climbing, sky-diving. Prolonged, frequent exercise can cause a weakness of the heart muscle (heart failure).

Medications:

You may be prescribed medications for heart rhythm disturbances, or to improve the pumping function of the heart.

Implantable Defibrillator:

In some patients with dangerous abnormal heart rhythms a defibrillator (a special type of pacemaker, please see the section on Implantable Defibrillators elsewhere on this site) may be recommended.

Catheter Ablation:

In some cases abnormal heart rhythms may be treated with catheter ablation. This technique involves placing a wire or tube into the heart from a small incision in the groin. The wire is then used to destroy any short circuits causing abnormal heart rhythms (please see the section on Catheter Ablation elsewhere on this site).

What is the Outlook of People with ARVC?

The majority of those with ARVC will lead normal and productive lives with a good quality of life. The risk of death is low in those receiving appropriate treatment.

© Drs Caroline Medi and Mark McGuire Version 1.1 28/6/15

More info: British Heart Foundation ARVC