P1804 Phenotypic overlap between Brugada syndrome and arrhythmogenic cardiomyopathy

• Published in: European heart journal cardiovascular imaging 2020-01, Vol.21 (Supplement_1)
• Main Authors: Scheirlynck, E, Droogmans, S, Sieira, J, De Asmundis, C, Motov, A, Edvarsen, T, Koulalis, J, Lie, ØH, Chivulescu, M, Chierchia, G B, Brugada, P, Cosyns, B, Haugaa, K H
• Format: Article
• Language: English
• ISSN: 2047-2404; 2047-2412
• DOI: 10.1093/ehjci/jez319.1157

Abstract Background Brugada syndrome (BrS) and arrhythmogenic cardiomyopathy (AC) are genetic cardiac diseases characterized by high risk for sudden cardiac death. Although BrS and AC are different clinical entities, experimental research and clinical cases suggest a phenotypic overlap. Purpose We aimed to assess the prevalence of structural and electrical AC diagnostic criteria in BrS patients. Methods In this multicentre study we acquired electrocardiograms (ECG) and transthoracic echocardiography in BrS patients between May and September 2018. We assessed the right ventricular outflow tract (RVOT) diameter, fractional area change (FAC) from echocardiography, and depolarization and repolarization criteria from ECG using parameter cut offs and definitions according to 2010 AC Task Force criteria. Results We included 123 BrS patients [54 (40-62) years old, 63 (51%) women]. Although, by definition, no akinesia, dyskinesia or aneurisms were present, 61 (50%) BrS patients had major criteria for dilated RVOT and/or reduced FAC, and 39 (32%) had at least one minor RVOT or FAC criterion (Figure). ECG showed minor repolarization and/or depolarization criteria in 30 (24%) BrS patients. No epsilon waves or major T-wave inversions were observed. Conclusion Half of BrS patients had evident major RVOT dilation and/or reduced FAC and one quarter had minor electrical features of AC. These findings suggest a significant phenotypical continuum between BrS and AC. Abstract P1804 Figure.