(Also called arrhythmogenic right ventricular cardiomyopathy)
ARVD is a rare form of cardiomyopathy in which the heart muscle of the right ventricle (RV) is replaced by fat and/or fibrous tissue. 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 usually diagnosed at a young age (usually less than 40 years) and may cause sudden cardiac death in athletes. Symptoms may include:
The cause of ARVD is unknown. It occurs in about 1 in 5,000 people. 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 all first- and second-degree family members (parents, siblings, children, grandchildren, uncle, aunt, nephew, niece) be evaluated carefully for this form of cardiomyopathy, even in the absence of symptoms.
Researchers have found two patterns of inheritance for ARVD:
Not all the specific genes have been identified as a cause for ARVD. Researchers are attempting to identify the specific gene mutations and locations of chromosomes associated with ARVD.
ARVD may also be related to non-genetic causes such as congenital abnormalities (affecting the right ventricle), viral or inflammatory myocarditis.
ARVD is diagnosed based your on medical history, physical exam, and tests (echocardiogram, Holter monitor, electrophysiologic testing, cardiac MRI, and/or cardiac CT scan.
Cardiac Magnetic Resonance Imaging (MRI) is recommended for the diagnosis of ARVD, as it visualizes fibro-fatty infiltration of the right ventricular (RV) myocardium. It also can show increased RV dimensions as well as wall-motion abnormalities. Unfortunately, the image quality frequently is limited due to artifact from irregular heart rhythms. For this reason, the patient may also undergo a cardiac CT scan to confirm or rule out the diagnosis of ARVD. This imaging test can detect characteristic abnormalities of the RV myocardium or the RV cavity. Unlike MRI, a CT scan can be performed if the patient has an implanted device (e.g. pacemaker, defibrillator).
The diagnosis of ARVD is confirmed if the patient has these characteristics:
There is no known curative treatment for ARVD. Treatment is usually directed at controlling the patient's ventricular arrhythmias and managing heart failure. The primary goal of treatment is preventing sustained ventricular arrhythmias and/or sudden death. Thus, antiarrhythmic drug therapy is the most frequently used therapy. Frequent ventricular arrhythmias that have not been successfully treated with medical therapy may be treated with radiofrequency ablation. Patients who are thought to be at risk for sudden death are usually treated with an implantable defibrillator (ICD).
Patients with ARVD need to ask their physicians about exercise or participation in sports.
Doctors vary in quality due to differences in training and experience; hospitals differ in the number of services available. The more complex your medical problem, the greater these differences in quality become and the more they matter.
Clearly, the doctor and hospital that you choose for complex, specialized medical care will have a direct impact on how well you do. To help you make this choice, please review our Miller Family Heart and Vascular Institute Outcomes.
Choosing a doctor to treat your abnormal heart rhythm depends on where you are in your diagnosis and treatment. The following Heart and Vascular Institute Sections and Departments treat patients with Arrhythmias:
The Heart and Vascular Institute also has specialized centers to treat certain populations of patients:
For younger patients with abnormal heart rhythms:
See: About Us to learn more about the Sydell and Arnold Miller Family Heart & Vascular Institute.
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This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or healthcare provider. Please consult your healthcare provider for advice about a specific medical condition. This document was last reviewed on: 04/23/2019