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Arrhythmogenic right ventricular dysplasia

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Related Terms
  • Arrhythmia, arrhythmogenic right ventricular cardiomyopathy, ARVC, ARVD, beta-blocker, cardiomyopathy, chest pain, heart failure, heartbeat, inherited condition, ischemia, muscle deterioration, Naxos disease, right ventricle, sudden cardiac death.

Background
  • Arrhythmogenic right ventricular dysplasia (ARVD) is a disorder that affects the muscles of the heart. The heart is divided into four chambers: the right ventricle, left ventricle, right atrium, and left atrium. In patients with ARVD, the right ventricle or left ventricle (the chambers responsible for pumping blood to the lungs or other parts of the body) may be affected. Patients with ARVD undergo a progressive deterioration of muscle in the ventricle, and the muscle is replaced by fat and scar tissue.
  • Due to the deterioration of heart muscle, patients with ARVD typically experience arrhythmias, which are abnormal heart rhythms. Arrhythmia can lead to a speeding up or slowing down of the heart. If the arrhythmia is more severe, it can affect the ability of the heart to pump blood, which might result in lightheadedness, fainting, or chest pain.
  • In some cases, ARVD may result in sudden death if the heart is not able to pump enough blood to its own muscles, lungs, and the rest of the body. However, sudden death in patients with ARVD is not very common. Patients with ARVD who are under 35 and athletic are at the highest risk for sudden death. It is often recommended that patients with ARVD do not overexert themselves when exercising.
  • ARVD affects about one in 5,000 people. It can affect both men and women, but it is more common in men. Symptoms of ARVD are rarely seen in infants or young children. Symptoms of ARVD generally appear in teenagers or adults.
  • There is currently no cure for ARVD, but a number of options exist to treat the condition. Some options include medications that can reduce the occurrence of arrhythmias and a device called an implantable cardioverter defibrillator. A cardioverter defibrillator can be implanted in a patient's chest. It uses small electric shocks to reduce the severity of arrhythmias.

Signs and symptoms
  • Symptoms of arrhythmogenic right ventricular dysplasia (ARVD) are rarely seen in infants or young children. Symptoms of ARVD generally appear in teenagers or adults.
  • ARVD is a disorder that affects the muscles of the heart. The heart is divided up into four chambers, and in patients with ARVD, the right ventricle or left ventricle (the chambers responsible for pumping blood to the lungs or other parts of the body) is affected. Patients with ARVD undergo a progressive deterioration of muscle in the ventricle and the muscle is replaced by fat and scar tissue. This may eventually lead to enlargement of the ventricles in the heart.
  • Due to the deterioration of heart muscle, patients with ARVD typically experience arrhythmias, which are abnormal heart rhythms. Arrhythmia can lead to a slowing down of the heart, or a speeding up of the heart, which may feel like the heart is pounding or racing.
  • If an arrhythmia is more severe, it can affect the ability of the heart to pump blood, which might cause lightheadedness, breathlessness, fainting, nausea, or dizziness.
  • Chest pain (also called angina) may result when the heart's ability to pump blood becomes impaired. Chest pain usually results from a reduced flow of blood (ischemia) to heart muscle. In some cases, the chest pain may be severe and may feel like a heart attack.

Diagnosis
  • It is difficult to diagnose arrhythmogenic right ventricular dysplasia (ARVD). There is no single test that can conclusively diagnose the condition. Diagnosis of ARVD is usually based on the results of a variety of different tests, which are described below.
  • Electrocardiogram (ECG): ECG is a test that uses electrodes to measure the electrical activity of the heart. It can detect abnormal rhythms in the heart.
  • Exercise stress test: This is a test in which a patient walks on a treadmill. It can be used to determine what effect exercise has on a patient's heart and what level of exercise is appropriate for a patient. Because a patient's need for oxygen increases as more time is spent on the treadmill, the test can measure the reaction of the heart to this increased need. During the test, ECG is used to measure the electrical activity of the heart and blood pressure is also monitored. Abnormal results during the test may indicate that a patient is experiencing arrhythmias or increased stress on the heart.
  • Echocardiogram: An echocardiogram uses sound waves to produce a moving picture of the heart. It is a noninvasive test that can be used to look at the chambers of the heart to see if there are any structural abnormalities (such as a thinning of the muscle or an enlargement of the ventricle) and to see if the heart is pumping normally.
  • Cardiac MRI: A cardiac MRI is a noninvasive imaging procedure that uses a magnetic field and radio waves to produce a detailed image of the heart. This test can be used to assess the structure of the heart in detail and check for any structural abnormalities in the ventricle.
  • Holter monitor: A holter monitor is a device that has small electrodes attached to a recording monitor. The electrodes are kept attached to a patient's chest for a 24-hour period to record the electrical heart activity, and the recording monitor can be kept in the patient's pocket for ease. This can be used to detect any abnormalities in a patient's heart rhythm over a longer period of time. Patients wearing a Holter monitor usually keep a log of their activities, so that comparisons can be made between the level of physical activity and heart activity.
  • Biopsy: If ARVD is suspected, a biopsy may be performed. In a biopsy, a small amount of the heart tissue is surgically removed and checked for physical signs of deterioration. Because heart deterioration can be patchy in ARVD, a biopsy may not always be able to detect the disease.
  • Genetic testing: Genetic testing exists to check for defects in some genes that have been linked to ARVD, such as plakophilin-2. Genetic testing is usually recommended for patients who have a family history of ARVD.

Complications
  • Heart failure: A condition called heart failure may develop in some patients with arrhythmogenic right ventricular dysplasia (ARVD). Symptoms of heart failure include swelling of the legs, feet, or abdomen, shortness of breath, and feelings of extreme fatigue. Heart failure results when the heart does not supply the rest of the body with enough blood.
  • Sudden cardiac death: In some cases, ARVD may result in sudden death, if the heart is not able to pump enough blood to its own muscles, lungs, and the rest of the body. However, sudden death in patients with ARVD is not very common. Patients with ARVD who are under 35 and athletic are at the highest risk for sudden death. The reasons for this are unclear.

Treatment
  • Currently, there is no known cure for arrhythmogenic right ventricular dysplasia (ARVD). Treatment generally focuses on trying to reduce the symptoms, especially arrhythmia.
  • Medications: Medications called antiarrhythmic agents have been developed that can reduce the severity and intensity of heart arrhythmias. A commonly used antiarrhythmic agent is called a beta-blocker, which blocks proteins located on the heart called beta receptors. Blocking beta receptors reduces the action of the hormone epinephrine, which results in a relaxed beating of the heart. Some common beta-blockers are atenolol (Tenormin®), bisoprolol (Zebeta®), metoprolol (Lopressor®), and carvedilol (Coreg®). Side effects of beta-blockers may include fatigue, cold hands and feet, and sleep disturbances. Another class of drugs, called ACE-inhibitors, may be used by some patients. ACE-inhibitors make it easier for the heart to pump blood by enlarging blood vessels and reducing blood pressure.
  • Avoid stimulants: Patients with ARVD should avoid stimulants such as nicotine or caffeine, as these substances may increase the risk of arrhythmia.
  • Reduce exercise: It is often recommended that patients with ARVD do not overexert themselves when exercising. Too much exercise can increase the strain on the heart and may lead to sudden cardiac death. Patients should engage only in mild forms of exercise, all of which should be discussed with a doctor.
  • Implantable cardioverter defibrillator (ICD): An ICD is a device that is implanted in the body of a patient in order to measure the rhythm of the heart. Whenever the heart rhythm becomes abnormal, the ICD can deliver an electric shock to the patient's heart, returning the rhythm to normal. An ICD is similar to a pacemaker, but it is generally recommended for longer term use and for more serious conditions, due to its ability to give off higher energy pulses.
  • Surgical ablation: The specific area of the heart that has undergone deterioration may be surgically removed from a patient with ARVD. This procedure may be successful in treating arrhythmias caused by deterioration in a specific area. Because ARVD is a progressive disease, however, this is not considered a permanent cure.
  • Heart transplant: In very severe cases, a heart transplant may be used to treat ARVD. A heart transplant is often only performed after other treatments have been tried and found to be ineffective.

Integrative therapies
  • Note: Currently, there is insufficient evidence available on the safety and effectiveness of integrative therapies for the prevention or treatment of arrhythmogenic right ventricular dysplasia (ARVD). The integrative therapies listed below should be used only under the supervision of a qualified healthcare provider and should not be used in replacement of other proven therapies or preventive measures.
  • Unclear or conflicting scientific evidence:
  • L-carnitine: L-carnitine, or acetyl-L-carnitine, is an amino acid found in the body. L-carnitine has been reported to be beneficial in maintaining a healthy heart. Although preliminary results are promising, well-designed clinical trials investigating the effect of L-carnitine on arrhythmias are lacking in the available literature. High quality clinical research is needed in this area.
  • Omega-3 fatty acids: There is evidence from multiple clinical studies supporting the intake of omega-3 fatty acid (also known as fish or fish oil) supplements for a healthy heart. Fish oil supplements have been reported to lower triglycerides and reduce the risk of death, heart attack, and stroke in people with known heart disease. Fish oil may also slow the buildup of atherosclerotic plaques (hardening of the arteries) and lower blood pressure slightly. There is promising evidence that omega-3 fatty acids may decrease the risk of cardiac arrhythmias. Additional research is needed in this area specifically before a firm conclusion can be reached. Omega-3 fatty acids may increase the chances of bleeding, especially if the individual is taking blood-thinning medications, such as aspirin or warfarin (Coumadin®). It is important to choose fish and fish oils that are free of heavy metals such as mercury and lead.
  • Traditional Chinese Medicine: Traditional Chinese medicine (TCM) is a broad term encompassing many different methods and traditions of healing, such as acupuncture, herbal medicines, cupping, and moxibustion. They share a common heritage of technique or theory rooted in ancient Chinese philosophy (Taoism) which dates back over 5,000 years. TCM herb combinations have been used to stabilize arrhythmia after viral myocarditis (inflammation of the heart). However, research designs have been weak and more studies of better design are needed before recommendations can be made.
  • Fair or negative scientific evidence:
  • Aconite: Isolated constituents from aconite (Aconitum napellus) have been used in heart arrhythmias. However, the toxic effects, including dizziness and flushing of the face, associated with the administration of aconite limit its ability to be used clinically as an agent to treat bradycardias (slow heart rate). The clinical relevance of using aconite in clinical practice is limited until further large, randomized controlled studies are conducted.
  • Traditional or theoretical uses lacking sufficient evidence:
  • Integrative therapies used in the management of arrhythmias that have historical or theoretical uses but lack sufficient clinical evidence include: abuta (Cissampelos pareira), astragalus (Astragalus membranaceus), bael fruit (Aegle marmelos), bloodroot (Sanguinaria canadensis), cordyceps (Cordyceps sinensis), devil's claw (Harpagophytum procumbens), dong quai (Angelica sinensis), hawthorn (Crataegus oxyacantha), hypnotherapy, lemon balm (Melissa officinalis), massage, meditation, mistletoe (Viscum album), white horehound (Marrubium vulgare), and yohimbe bark extract (Pausinystalia yohimbe).

Prevention
  • There are currently no known ways to prevent arrhythmogenic right ventricular dysplasia (ARVD).

Author information
  • This information has been edited and peer-reviewed by contributors to the Natural Standard Research Collaboration (www.naturalstandard.com).

Bibliography
  1. ARVD Resource at John Hopkins Medicine. . Accessed January 30, 2008.
  2. Cardiomyopathy Organization. . Accessed January 30, 2008.
  3. Cleveland Clinic. . Accessed January 30, 2008.
  4. Marcus F, Towbin JA. The mystery of arrhythmogenic right ventricular dysplasia/cardiomyopathy: from observation to mechanistic explanation. Circulation. 2006 Oct 24;114(17):1794-5.
  5. Dalal D, Molin LH, Piccini J, et al. Clinical features of arrhythmogenic right ventricular dysplasia/cardiomyopathy associated with mutations in plakophilin-2. Circulation. 2006 Apr 4;113(13):1641-9.
  6. Mullens W, Pison L, Vandervoort P, et al. Multiple exercise induced syncopal episodes in a young woman due to arrhythmogenic right ventricular dysplasia. Europace. 2005 Mar;7(2):154-7.
  7. Natural Standard: The Authority on Integrative Medicine. . Copyright © 2008.
  8. Sako H, Hadama T, Miyamoto S, et al. Successful surgical treatment of heart failure and ventricular tachycardia in a patient with arrhythmogenic right ventricular dysplasia with cardiomyopathy. Circ J. 2005 Aug;69(8):996-9.
  9. van Tintelen JP, Entius MM, Bhuiyan ZA, et al. Plakophilin-2 mutations are the major determinant of familial arrhythmogenic right ventricular dysplasia/cardiomyopathy. Circulation. 2006 Apr 4;113(13):1650-8.

Causes
  • Inheritance: Several gene mutations have been associated with arrhythmogenic right ventricular dysplasia (ARVD). Individuals who inherit mutant copies of these genes are at greater risk for developing ARVD.
  • In some cases, only one mutated gene copy (from either the mother or the father) needs to be inherited to put an individual at an increased risk for developing the condition. Scientists have found that inheriting one of these defective genes does not always cause ARVD to develop. Therefore, they believe that a combination of genes, as well as other environmental factors (for example, exercise or diet), may influence the development of ARVD. In some cases, two copies (one from the mother and one from the father) need to be inherited in order for the disease to develop.
  • Plakophilin-2, which functions in the development and organization of the heart, is one gene that has been identified in ARVD. Most genes that have been linked to ARVD, such as plakophilin-2, appear to be important for the adhesion of cells in the heart to each other. Other genes linked to ARVD include plakoglobin and desmoplakin. However, the specific mechanism by which the mutation of these genes leads to ARVD is not known.
  • Viruses: Some evidence suggests that viral infection may also lead to ARVD. Viral infection of the heart may lead to a heightened immune response against the wall of the heart, which may eventually lead to deterioration and ARVD. Adenovirus and Coxsackievirus have been found in patients with ARVD and may be involved in causing the disease in some cases.

Risk factors
  • Arrhythmogenic right ventricular dysplasia (ARVD) is considered a genetic disease. Mutations, or defects, in several different genes have been shown to increase the risk for developing ARVD. If either the mother or father of an individual has one of these defective genes, that individual would have a 50% chance of inheriting that gene and being at increased risk for developing ARVD.
  • However, scientists have found that inheriting one of these defective genes does not always cause ARVD to develop. Other environmental factors, such as exercise or diet, may also influence the development of ARVD but specifics are yet unknown.
  • ARVD can affect both men and women, but it is more common in men. Symptoms of ARVD are rarely seen in infants or young children. Symptoms of ARVD generally appear in teenagers or adults.

Copyright © 2011 Natural Standard (www.naturalstandard.com)


The information in this monograph is intended for informational purposes only, and is meant to help users better understand health concerns. Information is based on review of scientific research data, historical practice patterns, and clinical experience. This information should not be interpreted as specific medical advice. Users should consult with a qualified healthcare provider for specific questions regarding therapies, diagnosis and/or health conditions, prior to making therapeutic decisions.

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