Arrhythmogenic right ventricular cardiomyopathy (ARVC)

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Learn about arrhythmogenic right ventricular cardiomyopathy, how it’s diagnosed and possible treatments of the condition.

Key points

Arrhythmogenic right ventricular cardiomyopathy (ARVC) results in abnormal heart rhythms.
In ARVC, the muscle of the right ventricle is replaced by fat and scar tissue, which blocks the electrical signal that keeps the heart beating normally.
Exercise restriction is often recommended as treatment. For high risk cases, an implantable cardiac defibrillator (ICD) may be required.

Introduction

1) An electrical signal starts in the sinoatrial (SA) node, which signals the atria to contract. 2) The electrical signal moves from the SA node to the atrioventricular (AV) node. 3) From the AV node, the electrical signal travels into the bundle of His, and then to the Purkinje fibres. 4) As this electrical signal moves through the heart, it stimulates the heart muscle to beat in a specific order; first the atria contract, followed by the ventricles.

1) In ARVC, the muscle of the right ventricle is replaced by fat and fibrous tissue. Abnormal electrical signals can start within the fibrous tissues. 2) These abnormal electrical signals can travel in the wrong direction, which may cause the heart to beat too fast. 3) The ventricles contract out of sync with each other, which means the heart cannot fill and pump normally.

"Arrhythmogenic" means causing an abnormal heart rhythm (arrhythmia). With arrhythmogenic right ventricular cardiomyopathy, the muscle of the right ventricle is replaced by fat and fibrous (scar) tissue. The electrical signal that keeps the heart beating regularly cannot pass through the abnormal fibrotic tissue, which can then cause abnormal heart rhythms.

ARVC is a genetic disease that occurs in 1 in every 5,000 people.

More information

What is cardiomyopathy

Cardiomyopathy is a disorder affecting the heart muscle. The heart may have a normal structure but there are problems in the way it develops or functions. Cardiomyopathy usually results in the heart being unable to pump effectively, also known as heart failure.

Cardiomyopathy can be caused by a number of factors, including infections, conditions affecting the body’s metabolism and genetics.

There are several different types of cardiomyopathy. The main four types are:

hypertrophic cardiomyopathy (HCM)
dilated cardiomyopathy (DCM)
restrictive cardiomyopathy (RCM)
arrhythmogenic right ventricular cardiomyopathy (ARVC).

Signs and symptoms

Symptoms of ARVC

If a child has ARVC, they may experience:

palpitations
abnormal heart rhythm
fainting
sudden cardiac arrest
heart failure

Children with ARVC need to see a special cardiologist called an electrophysiologist, who is a heart rhythm specialist. If the heart function is involved, they will need to see a heart function specialist as well. Some children are referred for assessment because of their symptoms, but more frequently diagnosis is made after another family member has been diagnosed with the disease.

Diagnosis

Diagnosis of ARVC

There is no single test that can diagnosis ARVC. A diagnosis is confirmed through a combination of a physical exam, cardiac tests and a family medical history.

Imaging of the heart, usually an MRI, to check for fatty deposits and fibrous tissue.
Heart catheterization test to obtain a sample of heart tissue. The tissue is biopsied to check for fatty deposits.
An electrocardiogram (ECG) to check for contraction, relaxation or rhythm abnormalities.
A family medical history and genetics test. Genetic abnormalities (mutations) are found in about half of all families. If a genetic abnormality causing ARVC is found in a child, it's a good idea for close family members (parents and siblings) to be tested as well.

Treatment

Treatment of ARVC

Treatment of ARVC often involves exercise restriction and occasionally, medicine. Medications to treat ARVC may include beta blockers, antiarrhythmics, and ACE-inhibitors.

For high risk cases, placement of an implantable cardiac defibrillator (ICD) may be necessary to help control the heart’s rhythm. In some cases, a heart transplant may be necessary.

Last updated: October 26th 2018