What is the Origin of Arrhythmogenic Cardiomyopathy?

The name is descriptive, combining arrhythmogenic (causing arrhythmia) and cardiomyopathy (disease of the heart muscle), reflecting the primary clinical manifestations of the condition. Historically, it was often referred to as Arrhythmogenic Right Ventricular Dysplasia (ARVD), but the broader term AC is now preferred as the disease can also affect the left ventricle. Genetic studies have illuminated its origin as an inherited disorder of desmosomal proteins.

What is the Mechanism of Arrhythmogenic Cardiomyopathy?

The underlying mechanism is often traced to mutations in genes encoding desmosomal proteins, which are essential for cell-to-cell adhesion in the heart muscle. The defective proteins compromise the structural integrity of the intercalated discs, leading to myocyte death under mechanical stress. The subsequent inflammatory response and repair process result in the characteristic replacement of muscle with non-contractile, electrically inert fibrofatty tissue, creating the substrate for re-entrant ventricular arrhythmias.

Arrhythmogenic Cardiomyopathy

Meaning

Arrhythmogenic Cardiomyopathy (AC) is a progressive heart muscle disorder characterized by the replacement of healthy myocardial tissue, typically in the right ventricle, with fibrofatty tissue. This structural abnormality creates an electrical instability within the heart, significantly increasing the risk for life-threatening ventricular arrhythmias and sudden cardiac death, especially in young adults and athletes. It represents a critical area of overlap between cardiology and inherited genetic disorders.

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Arrhythmogenic Cardiomyopathy

Meaning

Origin

Mechanism

Can Personalized Hormonal Strategies Mitigate Arrhythmia Risk in Adults?

How Does Testosterone Influence Cardiac Rhythm and Arrhythmia Risk?

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