The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhouWenzhouWenzhou Medical College Medical College Medical College Medical College温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心少见病因致尖端扭转室速一例少见病因致尖端扭转室速一例 温州医学院附属第一医院心内科温州医学院附属第一医院心内科 计光计光 胡开宇胡开宇The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhouWenzhouWenzhou Medical College Medical College Medical College Medical College温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心病例简介病例简介女性，女性，3737岁，无不良嗜好岁，无不良嗜好反复发作心悸、头痛、胸闷反复发作心悸、头痛、胸闷2 2年年晕厥晕厥2 2次次 伴腹痛、低热入院伴腹痛、低热入院一次发作时血压一次发作时血压 200/100mmHg200/100mmHg心电监护：频发室早、短阵室速（扭转室速）心电监护：频发室早、短阵室速（扭转室速）The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhouWenzhouWenzhou Medical College Medical College Medical College Medical College温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心特殊药物服用：拜复乐罗红，黛力新（停用三天）特殊药物服用：拜复乐罗红，黛力新（停用三天）辅检：辅检：UCGUCG、腹、腹B B超、超、EEGEEG、颈椎、颈椎MRIMRI，胸部，胸部CT CT 无异常无异常实验室检查：肝肾功能、电解质、血气正常，心肌坏死标实验室检查：肝肾功能、电解质、血气正常，心肌坏死标记物（记物（）入院前入院前DCGDCG：窦性心律，偶发房早、室早，长：窦性心律，偶发房早、室早，长QTQT间期，间期，T T波波改变改变QTc 0.54The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhouWenzhouWenzhou Medical College Medical College Medical College Medical College温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心诊诊 断断心源性晕厥（尖端扭转性室速）心源性晕厥（尖端扭转性室速）长长QT综合症综合症查因查因?The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhouWenzhouWenzhou Medical College Medical College Medical College Medical College温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心长长QT原因原因先天性先天性Q-T间期延长间期延长 LQT1-7(1-3最常见最常见)获得性获得性Q-T间期延长间期延长药物诱导药物诱导Q-T间期延长间期延长心血管药物心血管药物非心脏药物（抗组织胺药、大环内脂类药、奎诺酮非心脏药物（抗组织胺药、大环内脂类药、奎诺酮类药物、抗真菌药物、抗症疾药、抗病毒药、抗精类药物、抗真菌药物、抗症疾药、抗病毒药、抗精神病药、抗抑郁药、促进动力药）神病药、抗抑郁药、促进动力药）其他其他 心肌病变，电解质紊乱，内分泌疾病，颅内疾心肌病变，电解质紊乱，内分泌疾病，颅内疾病，营养疾病，起搏病，营养疾病，起搏截至2005年6月, ADRAC已经接到140例QT间期延长和(或)扭转型室性心动过速的报告,有7例死亡。索他洛尔( 2 5 例) 、西沙比利( 1 7例) 、氯氮平(1 4例) 、胺碘酮(1 2例)和红霉素(1 2例)The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhouWenzhouWenzhou Medical College Medical College Medical College Medical College温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心病前服用过拜复乐罗红，黛力新病前服用过拜复乐罗红，黛力新药物性长药物性长QT？The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhouWenzhouWenzhou Medical College Medical College Medical College Medical College温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心QTc 0.54The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhouWenzhouWenzhou Medical College Medical College Medical College Medical College温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心第三天第三天 QTc 0.61The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhouWenzhouWenzhou Medical College Medical College Medical College Medical College温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心入院第三天入院第三天 突发持续性右侧腹痛突发持续性右侧腹痛腹部腹部CT点击此处看图点击此处看图The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhouWenzhouWenzhou Medical College Medical College Medical College Medical College温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心问题：患者腹部占位与长问题：患者腹部占位与长QT，及尖端扭转，及尖端扭转性室速关系？性室速关系？The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhouWenzhouWenzhou Medical College Medical College Medical College Medical College温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心入院第三天入院第三天去甲肾上腺素去甲肾上腺素 37.32ng/ml (1.7)肾上腺素肾上腺素 0.1 多巴胺多巴胺 0.1入院第六天入院第六天去甲肾上腺素去甲肾上腺素 3.72ng/ml (1.7)肾上腺素肾上腺素 0.1 多巴胺多巴胺 0.1The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhouWenzhouWenzhou Medical College Medical College Medical College Medical College温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心第三天第三天 QTc 0.61去甲肾上腺素去甲肾上腺素 37.32ng/ml The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhouWenzhouWenzhou Medical College Medical College Medical College Medical College温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心第六天第六天 QTc 0.43去甲肾上腺素去甲肾上腺素 3.72ng/ml The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhouWenzhouWenzhou Medical College Medical College Medical College Medical College温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心肾上腺素能刺激肾上腺素能刺激常与遗传性长常与遗传性长QT综合征有关综合征有关 促进继发促进继发Tdp的发生的发生The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhouWenzhouWenzhou Medical College Medical College Medical College Medical College温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心 肾上腺素能刺激常与遗传性长肾上腺素能刺激常与遗传性长QT综合征有关综合征有关 促进继发促进继发Tdp的发生的发生 细胞内腺苷酰环化酶活性细胞内腺苷酰环化酶活性 激活蛋白激酶激活蛋白激酶A 肾上腺素能肾上腺素能 cAMP Ca 2通道及通道及K通道通道磷酸化磷酸化 离子内流增加离子内流增加 EAD的发生。的发生。 1肾上腺素能通过蛋白激酶肾上腺素能通过蛋白激酶C，使，使Ca 2通道磷酸通道磷酸化，使内向电流增加化，使内向电流增加 1肾上腺素能可阻断肾上腺素能可阻断Ik及及Ito，延缓复极，延长，延缓复极，延长AP时间时间 对对NaK泵刺激使细胞膜超极化，降低心率泵刺激使细胞膜超极化，降低心率The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhouWenzhouWenzhou Medical College Medical College Medical College Medical College温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心Whalen RK 等报道肾上腺外嗜咯细胞瘤占成人嗜咯等报道肾上腺外嗜咯细胞瘤占成人嗜咯细胞瘤细胞瘤13，儿童，儿童30Whalen RK, Althausen AF, Daniels GH. Extra-adrenal pheochromocytoma. J Urol 1992;147:110.Goldfarb DA等报道好发于等报道好发于2030Y ，高血压，高血压+三联三联症症Goldfarb DA, Novick AC, Bravo EL, et al. Experience with extra-adrenal pheochromocytoma. J Urol 1989;142:9316.Michaels RD ECG 异常异常75，首发室性心律失常，首发室性心律失常罕见罕见 （1990年）年）Michaels RD, Hays JH, OBrian JT, Shakir KM. Pheochromocytoma associated ventricular tachycardia blocked with atenolol. J Endocrinol Invest. 1990;13:943-7.The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhouWenzhouWenzhou Medical College Medical College Medical College Medical College温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心确诊：确诊： 嗜咯细胞瘤嗜咯细胞瘤 继发性长继发性长QT间期间期 尖端扭转性室速尖端扭转性室速转归：手术切除肿瘤，病理确诊嗜咯细胞瘤转归：手术切除肿瘤，病理确诊嗜咯细胞瘤 术后无再发作晕厥，术后无再发作晕厥，QTc 0.40The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhouWenzhouWenzhou Medical College Medical College Medical College Medical College温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心 谢 谢The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhouWenzhouWenzhou Medical College Medical College Medical College Medical College温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心第四天第四天 QTc 0.48The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhouWenzhouWenzhou Medical College Medical College Medical College Medical College温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心第六天第六天 QTc 0.43The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhouWenzhouWenzhou Medical College Medical College Medical College Medical College温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心温州市心脏介入治疗中心后腹膜巨大占位后腹膜巨大占位平平 扫扫增增 强强后腹膜巨大占位增强后后腹膜巨大占位增强后部分强化部分强化