Arrhythmogenic Right Ventricular Cardiomyopathy

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Synonyms

ARVC,

Overview

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a heritable heart muscle disease where the myocardium progressively degrades and is replaced by fat and fibrous tissue. This scarring disrupts electrical signals, causing dangerous ventricular arrhythmias, a high risk of sudden cardiac death (especially in athletes), and potential heart failure.

Symptoms

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a genetic heart disease where muscle tissue is replaced by scar tissue, disrupting electrical signals. Symptoms primarily include heart palpitations, dizziness, fainting, shortness of breath, and chest pain. In some cases, sudden cardiac arrest may be the first and only sign.
Symptoms usually become apparent in teens or young adults (often between ages 10 and 40) and are frequently triggered by physical exertion or emotional stress.

Causes

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is primarily a genetic heart muscle disease. Mutations cause heart cell anchors (desmosomes) to fail. Under physical stress, muscle cells detach and die, leaving behind scar and fatty tissue. This structural damage trig

Prevention

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) prevention focuses on halting disease progression and avoiding Sudden Cardiac Death (SCD). The absolute cornerstone of management is strict avoidance of competitive sports and endurance training, alongside the use of an Implantable Cardioverter-Defibrillator (ICD) for high-risk individuals.

Diagnosis

Diagnosing Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is complex. There is no single pathognomonic test; rather, it is established using the standardized International Task Force (ITF) criteria. A diagnosis relies on a combination of imaging, electrocardiograms (ECGs), arrhythmia monitoring, genetic testing, and family history.

Prognosis

Some patients will have a stable functioning heart for decades, while others may have spells of arrhythmias that require changes in medication or ablations. Research has shown that the long-term outlook for most people with ARVD/C is relatively good.

Treatment

Treatment for Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) focuses on preventing life-threatening arrhythmias, reducing sudden cardiac death risk, and managing heart failure. It typically involves complete avoidance of vigorous exercise, antiarrhythmic medications, Implantable Cardioverter-Defibrillators (ICDs), catheter ablation, and in advanced cases, heart transplantation.