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Overview of Arrhythmogenic Cardiomyopathies


L. Brent Mitchell

, MD, Libin Cardiovascular Institute of Alberta, University of Calgary

Reviewed/Revised Jan 2023

Although any dilated or hypertrophic cardiomyopathy (see Overview of Cardiomyopathies Overview of Cardiomyopathies A cardiomyopathy is a primary disorder of the heart muscle. It is distinct from structural cardiac disorders such as coronary artery disease, valvular disorders, and congenital heart disorders... read more ) can produce cardiac and systemic factors that predispose to a number of different arrhythmias, including bradyarrhythmias, atrial and ventricular tachyarrhythmias, and sudden death, certain cardiomyopathies are particularly prone to do so. These are referred to as arrhythmogenic cardiomyopathies and include

Some of these disorders have genetic causes, which are typically inherited but are sometimes acquired mutations.

The signs and symptoms of the arrhythmogenic cardiomyopathies are not unique and include those of heart failure itself, along with those of bradyarrhythmias and tachyarrhythmias. Heart failure manifestations include exertion intolerance secondary to dyspnea and/or fatigue, orthopnea, and/or peripheral edema. Arrhythmias may cause palpitations, light headedness, syncope, cardiac arrest, and sudden death.

Evaluation typically includes family history, ECG, chest x-ray, echocardiography, and cardiac MRI. If a specific form of arrhythmogenic cardiomyopathy is suspected, other investigations may be indicated, including signal-averaged ECG, exercise testing, ambulatory electrocardiographic monitoring, chest CT, electrophysiologic study, angiography, and/or endomyocardial biopsy. Genetic testing is usually done when a genetic cause is suspected; first-degree relatives of confirmed cases should be screened clinically and with genetic testing when a specific mutation is found.

NOTE: This is the Professional Version. CONSUMERS: View Consumer Version
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