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An Introduction to Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C)

« seminars
Poster
July 2, 2008 - 2:30 pm

Speaker: Dr. Kristene Myklak - University of Colorado, Denver
Moderator: Dr. Marco Lazzarino, CBM and TASC
Location: Meeting Room CBM, Q Building, 1st floor
               AREA Science Park, Basovizza (Trieste)

ABSTRACT

Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) is an unusual condition characterized by the replacement of the right myocardium with fat and fibrous tissue. ARVD has a wide spectrum of clinical presentations, including mechanical dysfunction and various forms of ventricular arrhythmias. It is a cause of sudden death, mostly in young people and in athletes.

ARVD has been shown to be inherited in as many as 50% of patients. In the United States the, prevalence of ARVD is estimated to be 1 in 10,000. However, in certain regions of Italy, such as Padua and Venezia the prevalence is estimated to be as high as 4-6 in 10,000. This makes ARVD the most common cause of sudden death in competitive athletes in the Veneto region.

Several theories have been proposed for the pathogenesis and etiology of ARVD, however, neither is well characterized. It is currently understood that specific proteins comprising the desmosome, located at intercalated discs of heart muscle, are involved in the inherited forms of ARVD. The function of these desmosomal proteins needs to be carefully studied in order to gain a better understanding of the pathogenesis and etiology of ARVD.