Diagnosis and management for Primary Inherited Arrhythmia and Cardiomyopathy
遺伝性不整脈と心筋症の診断と治療

Sudden cardiac death (SCD) is caused by lethal arrhythmias such as ventricular fibrillation and ventricular tachycardia. Althoug ischemic heart diseases (IHD) is a leading cause of SCD, cardiac myopathies (CM) such as dilated CM (DCM), hypertrophic CM (HCM), and arrhythmogenic right ventricular cardiomyopathy (ARVC) are a 2nd leading cause of SCD. Primary inherited arrhythmia such as congenital long QT syndrome, Brugada syndrome, catecholaminergic polymorphic ventricular tachycardia, and early repolarization syndrome account for 1～2 % of SCD in Western countries, and more in Japan, approximately 10% of SCD. Lethal arrhythmias leading to SCD often develop in young to middle age in ptients with inherited arrhythmias, HCM, or ARVC, therefore, it is of particular importance in health policy to elucidate their pathogenesis leading to the prediction and prevention of SCD. Recent genetic studies have established a link between a number of CM or inherited arrhythmias and mutations in genes encoding for sarcomeric, cytoskeletal and desmosomal proteins, or cardiac ion channels. In this symposium, expert physicians and researchers will present their own experience or research products and provide a comprehensive discussion for the diagnosis, management, and the elucidation of pathogenesis of primary inherited arrhythmia and cardiomyopathy.