BACKGROUND: The original Task Force Criteria from 1994 for the clinical diagnosis of ARVC were highly specific and based on structural, histological, EKG, and familial features of disease. However, recommendations for clinical diagnosis and management of ARVC are sparse and lacked sensitivity for early disease.

CASE REPORT: Ventricular electrical instability and sudden cardiac death are the hallmarks of ARVC, and are often present before structural abnormalities. In this case report, we describe a patient who had detectable electrical abnormalities and structural changes that remained unchanged for over 10 years.

CONCLUSIONS: The disease progression in this case was defined as the development of a new 2010 TFC, which was absent at enrolment in 1994 and in 2008.

Keywords: Arrhythmogenic Right Ventricular Dysplasia, Electrocardiography, Magnetic Resonance Imaging