Left-dominant arrhythmogenic cardiomyopathy: an under-recognized clinical entity.

摘要: Objectives We sought to investigate the clinical-genetic profile of left-dominant arrhythmogenic cardiomyopathy (LDAC). Background In absence coronary disease and left ventricular (LV) systolic dysfunction, lateral T-wave inversion arrhythmia LV origin are often considered benign. Similarly, chest pain with enzyme release might be attributed viral myocarditis. hypothesized that these abnormalities manifestations “left-dominant” subtype right cardiomyopathy. Methods The 42-patient cohort was established through clinical evaluation individuals unexplained (infero)lateral inversion, origin, and/or proven LDAC/idiopathic myocardial fibrosis in family. Results Patients presented from adolescence age >80 years or but not heart failure. Desmosomal mutations were identified 8 24 families (15 33 patients). Magnetic resonance findings included late-enhancement a subepicardial/midwall distribution, corresponding fibrofatty replacement on histopathology. Fifty percent had previously been misdiagnosed myocarditis, dilated (DCM), hypertrophic cardiomyopathy, idiopathic tachycardia. Arrhythmic events presentation fibrillatory arrest 1 patient 2 instances sudden cardiac death during follow-up. Conclusions Arrhythmogenic is distinguished DCM by propensity towards exceeding degree dysfunction. under-recognized owing misattribution other disorders lack specific diagnostic criteria. Clinicians alerted possibility LDAC patients any apparent (with arrhythmic presentation), myocarditis (chest rise unobstructed arteries).