How Common is ARVD?
ARVD or arrhythmogenic right ventricular dysplasia is a rare condition of cardiomyopathy. It is not a typical disease. The occurrence of the condition sees the replacement of the right ventricular heart muscle with that of fibrous tissue or fat. Due to this, the right ventricular contracts body. As a consequence, the heart sees a reduction in pumping the blood as needed. Patients diagnosed with arrhythmogenic right ventricular dysplasia often suffer from arrhythmias, which increases the threat of sudden cardiac arrest.
It is still unknown what causes arrhythmogenic right ventricular dysplasia. According to researchers, the occurrence is rare and affects only one among 5,000 people. It is possible for an individual to display symptoms of arrhythmogenic right ventricular dysplasia with no family history. However, it can still occur. ARVD diagnosed because of family history is present in at least 50% of the cases. Because of this, undergoing evaluation became necessary for first and second-degree family members to check for the presence of cardiomyopathy even if one does not show any symptoms.
The two patterns of inheritance of ARVD are:
Autosomal Dominant – where the inheritance is from one parent and is more prevalent in a few geographic locations
Autosomal Recessive – characterization is because of the symptoms of ARVD as described above and one form is Naxos disease where the skin on the soles of the feet and hands become thick.
Symptoms of Arrhythmogenic Right Ventricular Dysplasia
Diagnosis of arrhythmogenic right ventricular dysplasia helps in preventing cardiac death. Symptoms include:
- Ventricular arrhythmias
- Increased palpitations
- Sudden cardiac death
- Heart failure
A doctor diagnosis for arrhythmogenic right ventricular dysplasia based on the physical examination, medical history of the patient, and a few tests such as cardiac MRI, cardiac CT scan, echocardiogram, Holter monitor, and electrophysiology testing. Most doctors opt for cardiac magnetic resonance imaging as it helps in visualization of the fibro-fatty substances present in the right ventricular myocardium. The test further helps in showing right ventricular dimensions and wall-motion abnormalities. As the image quality differs based on the heart rhythms, the doctor also asked the patient to undergo cardiac CT scan to confirm the presence of the illness or rule out the same.
Treatment of Arrhythmogenic Right Ventricular Dysplasia
There is no definite treatment available to cure arrhythmogenic right ventricular dysplasia. Doctors often focus on controlling the symptoms experienced by the patient. In many of the situations, the treatment aims at controlling the ventricular arrhythmias, which helps in preventing heart failure. It is possible to use radiofrequency ablation to treat frequent ventricular arrhythmias. When doctors note is that a patient is at high risk for sudden death, the treat the disease by choosing implantable defibrillator.
Pharmacology management involves suppression of arrhythmia and preventing the formation of thrombus. Beta blockers are the conventional medicines prescribed by the doctor. A doctor performs a series of ambulatory Holter monitoring to ensure that there is a reduction in the arrhythmic events. Depending on the improvement displayed by the patient, the doctor alters the medicines accordingly.
When the conventional medicines fail, doctors choose catheter ablation, which has a high success rate of 90%. However, opting for the treatment becomes difficult because arrhythmogenic regular dysplasia is progressive. Furthermore, recurrence is standard due to the creation of new arrhythmogenic foci.
An implantable cardioverter defibrillator is a useful tool against sudden cardiac arrest. However, due to the cost of the ICDs, doctors do not place it in all the individuals diagnosed with arrhythmogenic right ventricular dysplasia.