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儿茶酚胺介导的多形性室速是一种少见却严重的遗传性心律失常,表现为无器质性心脏病的个体在运动或激动时发生双向性、多形性室速导致发作性晕厥及进展为心室颤动导致猝死。心肌细胞肌浆网异常释放钙离子使细胞内钙离子超载引起的延迟后除极可能是儿茶酚胺介导的多形性室速发生的机制。目前已知的和儿茶酚胺介导的多形性室速相关的基因为常染色体显性遗传的RyR2(位于1q42.1-q43)和常染色体隐性遗传的CASQ2(位于1p13.3-p11)。治疗:β-阻断剂适用于所有临床症状的个体和可能有RyR2突变而没有心脏事件(晕厥)或运动试验诱发的室性心律失常等病史的个体。反复心脏骤停患者需植入式心律转复除颤器。每6至12个月随访以监测疗效。患者所有的一级亲属,都应予心脏评估。
Catecholamine-mediated pleiotropic ventricular tachycardia is a rare but severe hereditary arrhythmia that manifests itself in individuals who have no structural heart disease who develop bipolarity during exercise or excitement, pleomorphic VT result in episodic syncope and Progressive ventricular fibrillation leads to sudden death. Delayed post-depolarization caused by the abnormal release of intracellular calcium by intracellular Ca2 + in cardiomyocytes may be a mechanism of catecholamine-mediated pleiotropic ventricular tachycardia. Currently known catecholamine-mediated polymorphic VT-related genes are autosomal dominant inherited RyR2 (located at 1q42.1-q43) and autosomal recessive CASQ2 (located at 1p13.3-p11). Treatment: Beta-blockers are used in individuals with all clinical symptoms and individuals who may have a history of mutations in RyR2 without a heart attack (syncope) or exercise-induced ventricular arrhythmias. Patients with recurrent cardiac arrest require an implantable cardioverter-defibrillator. Follow up every 6 to 12 months to monitor efficacy. All first-degree relatives of patients, should be assessed by the heart.