Isolated left ventricular arrhythmogenic cardiomyopathy: A case report
Raimundo Barbosa-Barros1, Andrés Ricardo Pérez-Riera2, Luiz Carlos de Abreu2
1Coronary Center of the Hospital de Messejana Dr. Carlos Alberto Studart Gomes, Fortaleza, CE, Brazil.
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, and ventricular tachycardia from inferobasal LV wall.
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