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目的观察儿茶酚胺敏感性多形性室性心动过速(CPVT)患者的临床特征,总结临床经验,为今后该患者的诊疗方法提供理论依据。方法选取2005年1月—2013年1月收治的7例CPVT患者,对其临床资料进行回顾性分析。结果 7例CPVT患者均为青少年,平均年龄(12.1±3.7)岁。首发症状均为运动、情绪激动后晕厥。心电图特征为:静息状态下,无明显异常,可有轻度窦性心动过缓,T波改变,U波突出;运动试验时,7例患者的心电图均记录到双向性室性心动过速特征或多形性室性心动过速特征,有RYR2或CASQ2基因突变。口服β受体阻滞剂治疗后,患者的症状均有所改善。且3个月后的随访结果表明,患者无复发晕厥的记录。结论 CPVT是一种好发于青少年人群的遗传性疾病。临床表现为运动、情绪激动所致心动过速。可有晕厥甚至猝死发生。发病时,患者心电图表现为典型的双向性、多形性室性心动过速特征。β受体阻滞剂对该病的治疗切实有效,患者应长期服用。也可根据患者的个人情况,行埋藏式心脏转复除颤器(ICD)治疗。
Objective To observe the clinical characteristics of patients with catecholamine-sensitive pleomorphic ventricular tachycardia (CPVT) and to summarize the clinical experience and provide a theoretical basis for the future diagnosis and treatment of this patient. Methods Seven patients with CPVT who were admitted from January 2005 to January 2013 were retrospectively analyzed. Results All 7 CPVT patients were adolescents with an average age of (12.1 ± 3.7) years. The first symptom is exercise, syncope after agitation. Electrocardiogram characteristics: resting state, no obvious abnormalities may have mild sinus bradycardia, T wave changes, U wave prominent; exercise test, the ECG of 7 patients were recorded bidirectional ventricular tachycardia Features or pleomorphic ventricular tachycardia characteristics, RYR2 or CASQ2 gene mutations. Oral beta-blocker treatment, the patient’s symptoms have improved. Follow-up after 3 months showed no recurrent syncope in patients. Conclusions CPVT is a genetic disease that occurs in adolescents. Clinical manifestations of exercise, emotional excited tachycardia. May have syncope or even sudden death. When the disease, the patient’s ECG showed a typical two-way, pleomorphic ventricular tachycardia characteristics. β-blockers for the treatment of the disease effective, patients should be long-term use. Buried cardioverter defibrillator (ICD) treatment can also be performed according to the patient’s personal circumstances.