Arrhythmogenic right ventricular cardiomyopathy

Overview

Tell me about arrhythmogenic right ventricular cardiomyopathy.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic heart condition that is characterized by progressive replacement of the heart muscle (myocardium) with an combination of abnormal fat and scar tissue called "fibrofatty tissue". This process increases the chance that a person will have heart rhythm abnormalities such as ventricular tachycardia (a fast, abnormal heart rate). Abnormal heart rhythms can lead to sudden death, particularly in young individuals and athletes. ARVC affects the bottom right side of the heart (the right ventricle) but may affect the bottom left side of the heart (left ventricle) as well. ARVC can affect individuals differently from family to family and from individual to individual. The average age at diagnosis is 31 years, but children as young as 4 years old to as old as 64 years old have been found to have ARVC. If diagnosed early and treated aggressively, people with this condition often do quite well.

McNally E, MacLeod H, Dellefave-Castillo L. Arrhythmogenic Right Ventricular Cardiomyopathy. 2005 Apr 18 [Updated 2017 May 25]. In: Adam MP, Ardinger HH, Pagon RA, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2019. Available from: https://www.ncbi.nlm.nih.gov/books/NBK1131/

Fogoros RN, MDArrhythmogenic Right Ventricular Cardiomyopathy - AVRC. August 10, 2019 https://www.verywellhealth.com/arrhythmogenic-cardiomyopathy-exercise-1746292. accessed 11NOV19

Tichnell C, James CA, Murray B, Tandri H, Sears SF, Calkins H. Cardiology patient page. Patient's guide to arrhythmogenic right ventricular dysplasia/cardiomyopathy: past to present. Circulation. 2014 Sep 2;130(10):e89-92. https://www.ahajournals.org/doi/10.1161/CIRCULATIONAHA.113.004845 Accessed 11NOV19

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