Abstract
Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a disease characterized by fatty replacement of the right ventricular muscle (1). The residual myocardial fibers are often embedded in fibrous tissue. This substrate is conducive to right ventricular re-entrant arrhythmias. Initially this condition was called dysplasia because it was thought to represent an abnormality of development of the right ventricle. Subsequently it has been classified as a cardiomyopathy since the right ventricular myocardium may be normal at birth with fatty replacement progressive after birth. Since the pathogenesis of this disease is not exactly clear, the two terms are frequently combined as ARVD/C.
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Marcus, F.I., Ott, P. (2000). Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy. In: Berul, C.I., Towbin, J.A. (eds) Molecular Genetics of Cardiac Electrophysiology. Developments in Cardiovascular Medicine, vol 231. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-4517-0_15
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