Arrhythmogenic Right Ventricular Dysplasia (ARVD)

Also known as arrhythmogenic right ventricular cardiomyopathy

ARVD stands for Arrhythmogenic Right Ventricular Dysplasia. Arrhythmogenic means causing an arrhythmia.  The right ventricle is the chamber of the heart that is affected and dysplasia means there is an abnormality of the structure.  The right ventricle is dilated and contracts poorly.  As a result, the ability of the heart to pump blood is usually weakened. Patients with ARVD often have arrhythmias (abnormal heart rhythms), which can increase the risk of sudden cardiac arrest or death.

ARVD is a specific type of cardiomyopathy (a disorder of the cardiac muscle).

Simply put, ARVD is a genetic, progressive heart condition in which the muscle of the right ventricle is replaced by fat and fibrosis, which causes abnormal heart rhythms.  ARVD is estimated to affect one in 5,000 people. The disease can affect both men and women.  Although it is a relatively uncommon cause of sudden cardiac death, it accounts for up to one fifth of sudden cardiac death in people under 35 years of age.

The cause of ARVD is unknown.  ARVD can occur with no family history, although often it runs in families. A family history of ARVD is present in at least 30 to 50 percent of cases.  Therefore, it is recommended that family members of an ARVD patient should be tested.  First- and second-degree family members (parents, siblings, children, grandchildren, uncle, aunt, nephew, niece) should be evaluated carefully for this form of cardiomyopathy, even in the absence of symptoms.

Symptoms:

Sudden Cardiac Death - can be the first sign of ARVD

Ventricular arrhythmias: Irregular heart rhythms originating in the ventricles or lower chambers of the heart - The most common is ventricular tachycardia.

Palpitations: Fluttering in the chest due to abnormal heart rhythms

Dizziness, lightheadedness, or fainting caused by irregular heart rhythms

Heart failure- shortness of breath with activity, inability to carry out normal activities without fatigue, swelling in the legs, ankles and feet (edema)

The symptoms of ARVD are usually a result of an arrhythmia. Many people do not know they have an arrhythmia.  There are many different symptoms of an arrhythmia and healthy people without ARVD can have these symptoms.  When you feel your heart speed up or slow down or feel it pounding, it is called palpitation.  Palpitations are a normal response to fright or exertion but can, in other circumstances, be abnormal.  If the change in rhythm makes it difficult for the heart to pump blood, other symptoms can occur such as lightheadedness and fainting (also called syncope).  Arrhythmias can also impair the flow of blood to the heart muscle and cause chest pain, which is also called angina.  An arrhythmia can also cause sudden death if the heart cannot pump enough blood to its own muscle and to the lungs and body.  Fortunately, sudden death is not a common complication, but the risk must be considered when deciding on the treatment.

Sometimes people with ARVD develop symptoms of heart failure.  Heart failure means that the heart muscle is not pumping blood through the body effectively.  Symptoms include swelling of the legs, feet, and abdomen; feelings of shortness of breath while lying down and while exercising, and feelings of extreme fatigue.

In addition to these common symptoms of arrhythmias and heart failure other symptoms individuals with ARVD have reported include nausea, dizziness, heart fluttering, heart racing, etc.

Additional Information

John Hopkins Medicine  www.arvd.com

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