The diagnosis of ARVC/D is readily apparent when patients have the typical features including ECG changes and morphological abnormalities limited to the right ventricle. Since the right ventricular structure and contractile pattern is asymmetrical it can be difficult to differentiate normal from mild abnormalities of this complex ventricular chamber [1, 2]. This may cause uncertainty in differentiating ARVC/D from normal variants or from idiopathic ventricular tachycardia arising from the right ventricular outflow tract, a condition that is not hereditary and has a good prognosis [3-6]. These problems were recognized in the decade after the clinical profile of ARVDC/D was first described . To address these uncertainties a Task Force was assembled that proposed diagnostic criteria for ARVC/D. The Task Force agreed that there was no single test that is sufficiently specific and sensitive to be used to establish this diagnosis. Therefore, major and minor criteria were selected and it was proposed that the diagnosis of ARVC/D be made using combinations of these criteria  (Table 11.1). The strength of these guidelines is that they have been extremely useful in providing standardized criteria to establish the diagnosis of ARVC/D. Since the guidelines were published, there has been a large accumulation of experience in the diagnosis of ARVC/D as well as improvements in diagnostic imaging techniques including 2D echocardiography, MRI, and angiography.
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