Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C)

Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C)

Article
Heart & Vascular Health
Contributed byMaulik P. Purohit MD MPHMar 08, 2018

The topic Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) you are seeking is a synonym, or alternative name, or is closely related to the medical condition Arrhythmogenic Right Ventricular Cardiomyopathy.

Quick Summary:

  • Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a rare, primary, inherited disorder of the heart, characterized by the disintegration of the myocardium (heart muscle). Since ARVC is inherited, a family history of the condition is a major risk factor for being diagnosed with it
  • ARVC accounts for about 20% of sudden cardiac deaths in individuals under 35 years of age. This genetic disorder affects men more than women, and is extremely uncommon in children below age 10
  • Mutations in genes involved in the structure and function of desmosomes are known to cause Arrhythmogenic Right Ventricular Cardiomyopathy. Desmosomes are the junctions between cells that facilitate cell-cell adhesion
  • Abnormal desmosomes cause the muscle cells to separate and scar tissue (containing fibrous and fatty tissue) to form. This compromises the pumping of blood, particularly when an individual is engaged in physical activity
  • At least 5 different gene mutations are known to cause abnormal desmosome functioning. Most Arrhythmogenic Right Ventricular Cardiomyopathy cases occur in an autosomal dominant pattern
  • In rare cases, gene mutations causing ARVC can also be inherited in an autosomal recessive manner. Such cases are known to arise from “cardio-cutaneous” syndromes (such as Naxos disease, Carvajal syndrome, etc.)
  • The signs and symptoms of Arrhythmogenic Right Ventricular Cardiomyopathy may include shortness of breath during a physical activity or while sleeping, arrhythmia, low blood pressure, and fluid retention in organs and tissues
  • The diagnosis of Arrhythmogenic Right Ventricular Cardiomyopathy involves a thorough physical examination, symptom assessment, evaluation of family medical history, echocardiography (ECG), electrocardiography (EKG), stress test, imaging studies, and right ventricular angiogram. If required, a biopsy of the heart muscle may be ordered
  • Some potential complications of Arrhythmogenic Right Ventricular Cardiomyopathy include aberrant heartbeats (arrhythmia), sudden cardiac arrest, development of blood clot, stroke, and fluid build-up in tissues and organs
  • Depending on the severity of the condition, Arrhythmogenic Right Ventricular Cardiomyopathy may be treated using medications, non-surgical procedures, and surgical implants. Heart transplantation may be recommended in some serious cases
  • Since the condition is inherited, it is currently not possible to prevent it from taking place. However, individuals with a family history of ARVC may benefit from genetic testing and counseling before planning for a baby
  • The prognosis for Arrhythmogenic Right Ventricular Cardiomyopathy is favorable, if the condition is diagnosed early and treated promptly. The presence of severe complications is known to adversely affect outcomes

Please find comprehensive information on Arrhythmogenic Right Ventricular Cardiomyopathy regarding definition, distribution, risk factors, causes, signs & symptoms, diagnosis, complications, treatment, prevention, prognosis, and additional useful information HERE.

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Maulik P. Purohit MD MPH picture
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Maulik P. Purohit MD MPH

Assistant Medical Director, Medical Editorial Board, DoveMed Team

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