Isolated left ventricular arrhythmogenic cardiomyopathy: A case report.

Left ventricular involvement in the advanced stage of arrhythmogenic cardiomyopathy (AC) is an evolution of this desmosomopathy. Mutation in intercalated discs explains the phenotypic variability. Abnormal trafficking of the intercalated discs at the site of end-to-end contacts between cardiomyocytes and the canonical Wnt/β-catenin and Hippo signaling pathways have been implicated.

The use of fontaine leads in the diagnosis of arrhythmogenic right ventricular dysplasia.

We report a case of a 68-year-old man admitted to the emergency department with syncope preceded by rapid palpitations. His admission ECG demonstrated a sustained ventricular tachycardia (VT) originating from the right ventricular outflow tract (RVOT). This report highlights the importance of distinguishing ventricular tachycardia caused by arrhythmogenic right ventricular dysplasia (ARVD) from the more benign idiopathic RVOT-VT.