Arrhythmogenic right ventricular dysplasia, a rare condition, carries a high risk of abnormal heart rhythms that can be fatal for young adults. Most people with this disorder have a genetic mutation. Early diagnosis and treatment is best so your healthcare provider can help you avoid irregular heart rhythms with medicines and procedures.
Arrhythmogenic right ventricular dysplasia is a rare form of cardiomyopathy. With this condition, fat and/or fibrous tissue replaces damaged heart muscle in your right ventricle. Your right ventricle stretches out, becomes thin and contracts poorly. As a result, your heart has a weakened ability to pump blood.
The fat or fibrous tissues that replace your heart muscle can get in the way of heartbeat signals. People with arrhythmogenic right ventricular dysplasia often have arrhythmias (abnormal heart rhythms), which can increase their risk of sudden cardiac arrest or death.
Arrhythmogenic right ventricular dysplasia is also called arrhythmogenic right ventricular cardiomyopathy (ARVC). Because it can affect your left ventricle as well, it’s often called arrhythmogenic cardiomyopathy (ACM).
If you have ARVD, you can go through three stages over time:
Healthcare providers usually see arrhythmogenic right ventricular dysplasia in teens or young adults. This condition is the reason some young athletes have sudden cardiac arrest. Some studies say this condition happens more often in people assigned male at birth.
Arrhythmogenic right ventricular dysplasia occurs in about 1 in 1,000 to 1 in 5,000 people. It can happen with no family history, although it often runs in families. An estimated 30,000 to 200,000 people in the United States have arrhythmogenic right ventricular dysplasia.
You may not have arrhythmogenic right ventricular dysplasia symptoms early on. But there’s still a risk of sudden cardiac death.
Symptoms may include:
Symptoms may start between your 20s and 50s.
Healthcare providers typically diagnose arrhythmogenic right ventricular dysplasia in people at a young age (usually before age 40). It may cause sudden cardiac death in athletes.
About 60% of people with arrhythmogenic right ventricular dysplasia have a genetic mutation. Researchers have found at least 13 genes that can have mutations that cause this condition.
These abnormal genes harm proteins that help heart muscle cells connect to each other and communicate. Because of this, heart muscle cells in your right ventricle can separate and die. This can happen more often during times of stress or exertion.
A family history of arrhythmogenic right ventricular dysplasia is present in at least 30% to 50% of cases. Because of this, a healthcare provider should check all first- and second-degree family members (parents, siblings, children, grandchildren, uncles, aunts, nephews and nieces) of someone who has ARVD. Relatives who are teens or older should see a provider even if they don’t have symptoms.
Researchers have found two patterns of inheritance for arrhythmogenic right ventricular dysplasia:
ARVD also may be related to other causes such as:
Your healthcare provider can diagnose arrhythmogenic right ventricular dysplasia based on your medical history, physical exam and tests.
They can make an arrhythmogenic right ventricular dysplasia diagnosis if you have a combination of certain issues, such as:
Depending on how many of these issues you have, your provider may make a definite, borderline or possible diagnosis. Your provider may recommend genetic testing in some, but not all, cases.
Tests to diagnose arrhythmogenic right ventricular dysplasia include:
There isn’t a cure for arrhythmogenic right ventricular dysplasia, but your healthcare provider will work to:
Arrhythmogenic right ventricular dysplasia treatments include:
You may need more than one type of treatment for this condition during your lifetime.
While you’re taking warfarin, you’ll need to get regular blood tests. Your healthcare provider will want to make sure you’re getting the right amount of warfarin. Based on your test results, your provider may change your dose.
Catheter ablation is successful at first in the majority of people. However, abnormal heart rhythms come back in 60% of people because their disease has gotten worse.
ICD wires can move out of place or stop working right.
Keep communicating with your healthcare provider during your treatment. They can tell you if you’re taking the right dose of medicine. Also, going to follow-up appointments will help them catch any issues that develop with your condition.
Getting screened early for arrhythmogenic right ventricular dysplasia is the best way to reduce your risk if a relative has the condition. Noninvasive tests can tell you if you’re at risk of abnormal heart rhythms. If you’re at risk, your healthcare provider can work with you to make a treatment plan.
Arrhythmogenic right ventricular dysplasia life expectancy varies, depending on when you get a diagnosis. It’s best to get a diagnosis at an early age and get the treatment that prevents irregular heart rhythms. The condition gets worse with time.
Without treatment for arrhythmogenic right ventricular dysplasia, your right ventricle can fail. Then your left ventricle can fail later. This can lead to heart failure and atrial fibrillation. Researchers believe your outlook is worse when ARVD affects both ventricles.
With treatment, you can get the help you need to lessen the burden on your heart and prevent dangerous heart rhythms.
Yes. A study reported that some people were older than 65 at diagnosis. Researchers estimate one-fifth of people with ARVC get symptoms after age 50.
Today, the long-term outlook for people with ARVD is good. This is because of advanced imaging like CT and MRI that allow for an earlier diagnosis and treatment.
However, sudden death can happen to people who haven’t been diagnosed or aren’t getting the treatment they need. ARVD causes 11% of sudden cardiac deaths — and 22% of sudden cardiac deaths in athletes — aged 35 and younger.
To make things easier on your heart, you can make some changes in how you live. Things you can do include:
Because exertion makes arrhythmogenic right ventricular dysplasia worse, check with your healthcare provider before exercising. You shouldn’t take part in competitive sports, except possibly low-intensity ones.
If you have arrhythmogenic right ventricular dysplasia, you’ll need regular appointments for the rest of your life. Your healthcare provider will want to make sure you’re getting the right treatment. Also, your provider will need to check your ICD regularly if you have one.
If you see someone collapse and they don’t respond when you ask if they’re OK, call 911. Then start CPR, even if it’s just the hands-only type.
It’s a good idea to ask people who live with you to take a CPR class if you’re at risk of cardiac arrest.
A note from Cleveland Clinic
Because it can be tricky to get an arrhythmogenic right ventricular dysplasia diagnosis before you have symptoms, it’s important to know your family history. If your family is at risk because of a relative’s medical history with this condition, your healthcare provider can do screenings.
You’ll have the best prognosis when you get treatment early before arrhythmogenic right ventricular dysplasia progresses. It may give you peace of mind if the people who live with you get CPR training. That way, they’ll know what to do if you need help.
Last reviewed by a Cleveland Clinic medical professional on 06/22/2022.
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