Arrhythmogenic Right Ventricular Cardiomyopathy

Arrhythmogenic right ventricular cardiomyopathy (ARVC), is also, known as Arrhythmogenic right ventricular dysplasia (ARVD). It is a type of nonischemic cardiomyopathy. Cardiomyopathy is a class of diseases, in which the heart muscles do not function properly. ARVC is a type of cardiomyopathy, which may be hereditary. In ARVC, the right ventricular muscles are replaced by fats/fibrous fats, which leads to an increase in the heartbeat.

Causes of Arrhythmogenic Right Ventricular Cardiomyopathy

The pumping of the blood in the body is a cycle, that involves four chambers of the heart, i.e. the left atrium, right atrium, left ventricle and right ventricle. The function of right ventricle is to pump blood to the lungs. In the lungs, the oxygen is absorbed in the blood, and then the oxygenated blood is collected in the left ventricle, through the left atrium. This blood is then supplied to all the organs of the body. When the muscle tissues in the right ventricle are replaced by fat tissues, the right ventricle is unable to pump the blood to the lungs in a normal pattern and it inflates. This leads to irregular heartbeats i.e. arrhythmia.

What causes arrhythmogenic right ventricular cardiomyopathy is still unknown. ARVC is mostly inherited, in an autosomal dominant pattern. In autosomal dominant inheritance, a person inherits a faulty gene from one parent; while, in autosomal recessive inheritance, a faulty gene is inherited from both the parents. In autosomal dominant inheritance, there are 50% chances of the offspring being affected by the disease. Autosomal recessive inheritance of ARVC is rarely found. The genes that cause ARVC are not yet discovered.

Symptoms of Arrhythmogenic Right Ventricular Cardiomyopathy

ARVC does not show early symptoms. In fact, many a times, it goes undiagnosed till the patient suffers from a heart problem. In rare cases, ARVC symptoms are observed in childhood. Maximum cases are found to show symptoms at any age between 30 and 40. The symptoms may vary for every case of ARVC. Following are some of the symptoms of arrhythmogenic right ventricular cardiomyopathy.

• Arrhythmia
• Fatigue
• Palpitation
• Heart failure

Diagnosis of Arrhythmogenic Right Ventricular Cardiomyopathy

Since the gene that may cause ARVC is not yet undiscovered, it becomes difficult to diagnose ARVC at the earliest. The physician studies the medical and family history of the patient in the first stage of the disease. Since ARVC can be inherited, the physician will focus more on family history. However, not all the ARVC cases are inherited. Following are the tests performed to diagnose ARVC:

Magnetic Imaging Resonance (MRI): It gives a cross-sectional image of the heart. It does not help in complete diagnosis of ARVC, because it provides a very confined information about the fat in the ventricle.
Electrocardiogram: It helps in confirmation of arrhythmia.
Echocardiogram: Sound waves are used to create an image of heart. The image should focus more on right ventricle.

Radionuclide angiography, MUGA (Multiple-gated acquisition) scan and Electrophysiology are some other tests performed to diagnose ARVC. Diagnosis of ARVC is not very easy. The diagnosis is confirmed, when the patient has a damaged right ventricle, has a family history of ARVC and if fat is present in the right ventricle.

Treatment of Arrhythmogenic Right Ventricular Cardiomyopathy

ARVC is a heart disease that cannot be cured, but the symptoms can be reduced by giving proper treatment to the patient. The treatment focuses on arrhythmia and avoiding heart failure or sudden death. The treatments help reduce the death risks. Medications called antiarrhythmics are usually considered as the best treatment for arrhythmia. ICD (Implantable cardioverter defibrillators) are implanted in patients having severe ARVC. ICD regulates the normal heartbeat. To destroy the fat cells in the ventricle, radiofrequency ablation therapy along with other therapy is used sometimes.

There is no any prevention method for ARVC. 1 in 5000 is affected by ARVC. ARVC may cause sudden, unexplained death. It is found mostly in the younger generation. Athletes are said to be at high risk of ARVC. If the gene responsible for ARVC is discovered, gene therapy may prove useful to treat ARVC.