Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy


Description

Arrhythmogenic right ventricular dysplasia is an extremely uncommon kind of heart muscle disease. In this disorder, the right ventricle's damaged heart muscle is replaced by fat and/or fibrous tissue. Your right ventricle enlarges, thins out, and contracts inefficiently. As a result, your heart's capacity to pump blood is diminished. The prevalence of ARVD/C is estimated to be 1:5000. According to studies, up to 20% of those who die suddenly from cardiac causes have Arrhythmogenic right ventricular dysplasia, and this number is significantly higher among athletes. Although it frequently runs in families, it can also happen without a familial history.

Symptoms

Symptoms of arrhythmogenic right ventricular dysplasia may appear between the ages of 20 and 50.

Palpitations
Fainting
Shortness of breath
Dizziness or lightheadedness
Chest pain
Sudden cardiac death


Causes

At least 30% to 50% of patients have a familial history of arrhythmogenic right ventricular dysplasia
A genetic mutation affects 50% to 60% of those who develop arrhythmogenic right ventricular dysplasia.
Viral infection (myocarditis or inflammation of the heart muscle)

Diagnostics

Based on your medical history, physical examination, and testing, your doctor can determine whether you have arrhythmogenic right ventricular dysplasia.

Diagnosis can be made in case you have a combination of certain conditions:

Abnormal Electrocardiogram
Abnormal right ventricle is seen through heart imaging
Family history of arrhythmogenic right ventricular dysplasia
Arrhythmias


Treatment

Arrhythmogenic right ventricular dysplasia has no known treatment, although your cardiologist will try to:

1. Reduction of mortality, either from heart failure or arrhythmic sudden cardiac death.

2. Preventing disease progression that results in heart failure and malfunction of the right, left, or both ventricles.

3. Symptom and quality of life improvements.

4. Reducing symptoms of heart failure.



Therapeutic options consist of:

Lifestyle changes (Patients with a confirmed diagnosis of ARVD/C are advised not to take part in competitive sports.)
Pharmacological treatment including antiarrhythmic agents, β-blockers, and heart failure drug therapy. (Ex: Amiodarone)
Catheter ablation
Implantable cardioverter-defibrillator placement
Heart transplantation

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