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What is Arrhythmogenic Right Ventricular Cardiomyopathy?

Arrhythmogenic right ventricular cardiomyopathy can be described as a disorder that is characterized by the progressive replacement of the regular ventricle muscle cells with fat and fibrous tissue. At first, this disorder may only affect a few specific areas of the right ventricle, but with time, it becomes global and could affect your entire ventricle.

Arrhythmogenic right ventricular cardiomyopathy (AVRC) first came to light in the late 1970s and the Cardiovascular Group at the University of Padova, Italy developed expertise in this particular condition. The number of AVRC cases reported each year is on the rise, probably because of the improvements in diagnostic techniques. Health experts claim that this disorder is the second most common cause of sudden death in younger people. This is largely because of the difficulty in detecting the condition in its initial stages. You may suffer from arrhythmogenic right ventricular cardiomyopathy for several years, without experiencing any symptoms. Unfortunately, there is no cure for this condition, though there are treatment options available, which aim to control arrhythmia.

Causes

The exact causes of arrhythmogenic right ventricular cardiomyopathy are still not clear, but the condition is said to be heredity. The genes that lead to this disorder are passed on from one generation to the next. You have a 50% chance of inheriting the abnormal gene if one of your parents have had this disorder.

Symptoms

In the initial stages, arrhythmogenic right ventricular cardiomyopathy is usually asymptomatic. However, as the disorder progresses, you may experience certain symptoms such as -

• Rapid heartbeat
• Lightheadedness or dizziness
• Fainting spells
• Swelling in the ankles, legs and feet
• Fatigue

Age is an important factor and in case you are over the age 50 years, you may experience additional signs that indicate this disorder. The symptoms of arrhythmogenic right ventricular cardiomyopathy in the elderly include -

• Shortness of breath with the slightest exertion, or even during periods of rest
• Severe pain in the chest

The signs and symptoms of arrhythmogenic right ventricular cardiomyopathy usually tend to worsen, unless you undergo treatment.

Occasionally, this condition could also result in sudden death. Therefore, you need to seek emergency medical attention as soon as you notice any of the symptoms mentioned above. Since this condition could be heredity, your doctor may also advise your family members to undergo the required diagnostic tests.

How is it diagnosed?

For an accurate diagnosis of arrhythmogenic right ventricular cardiomyopathy, you will need to consult a cardiologist with special expertise. Your doctor will conduct a physical exam and will probably make a note of the symptoms you have been experiencing. If your doctor suspects that you are suffering from this disorder, you may be asked to undergo -

• A chest x-ray: Images of your heart will be studied for any abnormalities
• Echocardiogram: Sound waves are used to produce images of your heart and these images are examined for proper functioning, size and motion.
• Electrocardiogram (ECG): Electrode patches are attached to your chest in this non-invasive procedure, to measure your heart’s electrical impulses. This test will detect any disturbance in the electrical activity of your heart, like abnormal heart rhythms and injuries, if any.
• Catheterization and Biopsy: A thin tube or catheter is inserted through the groin and passed through the blood vessels, into your heart, from where a small tissue sample is extracted. This tissue is sent to the lab for further tests. During this procedure the examiner will also measure the pressure within the chambers of your heart to see how forcefully blood is pumped through the heart. Images of the artery are also obtained during the cardiac catheterization, to ensure that there is no blockage.
• Cardiac MRI: Magnetic fields and radio waves are used in this test, to produce images of your heart. This test is generally only conducted if echocardiogram images are not very helpful.
• Blood Tests: When your heart is under a lot of stress or pressure, a certain protein called B-type Natriuretic Peptide (BNP), present in your blood rises. A blood test is therefore conducted to measure the BNP levels in your blood.

In addition to the tests mentioned above, the diagnosis of arrhythmogenic right ventricular cardiomyopathy may also require blood tests to check kidney function, anemia and thyroid problems. Unfortunately, arrhythmogenic right ventricular cardiomyopathy can sometimes be missed in spite of conducting the tests mentioned above.

Treatment

Arrhythmogenic right ventricular cardiomyopathy treatment is aimed at preventing the condition from getting worse and controlling the symptoms. At first, your doctor may prescribe drugs to improve the health of your heart. These medicines could include -

• Angiotensin-Covering Enzymes (ACE) inhibitors
• Angiotensin receptor blockers
• Beta blockers
• Digoxin
• Diuretics or water pills

If the drug treatment does not seem successful, other specialized treatment options may be recommended, some of which include -

• Catheter ablation
• Surgical ablation
• Septal ablation

Have a detailed discussion with your doctor about the different options, in order to identify which one is the most suitable for you.

References

• Koji Fukuzawa, Alessandro Zorzi, Federico Migliore, Ilaria Rigato, Barbara Bauce, Cristina Basso, Gaetano Thiene, Domenico Corrado, Arrhythmogenic Right Ventricular Cardiomyopathy, Cardiac Electrophysiology Clinics, Volume 2, Issue 4, December 2010, Pages 571-586, ISSN 1877-9182, 10.1016/j.ccep.2010.09.006.
• Ventricular arrhythmia; Genetics; Catheter ablation; Implantable cardioverter defibrillator
• Patrick T. Ellinor, Calum A. MacRae, Ludwig Thierfelder, Arrhythmogenic Right Ventricular Cardiomyopathy, Heart Failure Clinics, Volume 6, Issue 2, April 2010, Pages 161-177, ISSN 1551-7136, 10.1016/j.hfc.2009.12.003.
• Srijita Sen-Chowdhry, Sanjay K. Prasad, Petros Syrris, Ricardo Wage, Deirdre Ward, Robert Merrifield, Gillian C. Smith, David N. Firmin, Dudley J. Pennell, William J. McKenna, Cardiovascular Magnetic Resonance in Arrhythmogenic Right Ventricular Cardiomyopathy Revisited: Comparison With Task Force Criteria and Genotype, Journal of the American College of Cardiology, Volume 48, Issue 10, 21 November 2006, Pages 2132-2140, ISSN 0735-1097, 10.1016/j.jacc.2006.07.045.
• Fiorella Calabrese, Cristina Basso, Elisa Carturan, Marialuisa Valente, Gaetano Thiene, Arrhythmogenic right ventricular cardiomyopathy/dysplasia: is there a role for viruses?, Cardiovascular Pathology, Volume 15, Issue 1, January-February 2006, Pages 11-17, ISSN 1054-8807, 10.1016/j.carpath.2005.10.004.
• Carol Gemayel, Antonio Pelliccia, Paul D Thompson, Arrhythmogenic right ventricular cardiomyopathy, Journal of the American College of Cardiology, Volume 38, Issue 7, December 2001, Pages 1773-1781, ISSN 0735-1097, 10.1016/S0735-1097(01)01654-0.