Arrhythmogenic Right Ventricular DysplasiaCardiomyopathy Type 1 : A Light on Molecular Mechanisms

Abstract EN

Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is an inherited cardiomyopathy associated with cardiac arrhythmias originating in the right ventricle, heart failure, and sudden cardiac death.

Development of ARVD/C type 1 has been attributed to differential expression of transforming growth factor beta 3 (TGFβ3).

Several mechanisms underlying the molecular basis of ARVD/C type 1 have been proposed.

Evaluating previously described mechanisms might elucidate how TGFβ3 contributes to disease progression in ARVD/C type 1.

Here we review how TGFβ3 can induce fibrogenesis through Smad and/or β-catenin signaling.

Moreover, the role of apoptosis is addressed.

Finally the extent to which the immune system has been demonstrated to be a modulating and amplifying agent in the onset and progression of ARVD/C in general is discussed.