Isolated Left Ventricular Arrhythmogenic Cardiomyopathy: A Case Report

• Published in: Journal of electrocardiology Vol. 50; no. 1; pp. 144 - 147
• Main Authors: Barbosa-Barros, Raimundo, MD, Pérez-Riera, Andrés Ricardo, MD PhD, de Abreu, Luiz Carlos, PhD, de Sousa-Rocha, Ricardo Paulo, MD, da Costa Lino, Danielli Oliveira, MD, Baranchuk, Adrian, MD, FACC, FRCPC, Zhang, Li, MD
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.01.2017; Elsevier Science Ltd
• Subjects: Cardiomyopathies - complications; Cardiomyopathies - diagnosis; Cardiomyopathy; Cardiovascular; Cardiovascular disease; Case reports; Diagnosis, Differential; Electrocardiogram; Electrocardiography; Electrocardiography - methods; Humans; Intercalated disc; Isolated left ventricular arrhythmogenic cardiomyopathy; Male; Middle Aged; Tachycardia, Ventricular - complications; Tachycardia, Ventricular - diagnosis; Ventricular Dysfunction, Left - complications; Ventricular Dysfunction, Left - diagnosis; electrocardiogram; intercalated disc; Isolated left ventricular arrhythmogenic cardiomyopathy; Intercalated disc; Electrocardiogram
• ISSN: 0022-0736; 1532-8430
• DOI: 10.1016/j.jelectrocard.2016.09.003

Abstract 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. In AC, T-wave inversion in lateral leads is the hallmark of left ventricular (LV) involvement. We herein report a case of isolated LV AC in which the initial ECG has typical features: fragmented QRS (fQRS), Twave inversion (TWI) in lateral leads, ventricular tachycardia from inferobasal LV wall.