少见病因致尖端扭转室速一例

The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhou 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 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 计光 胡开宇 The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhou 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 病例简介 女性，37岁，无不良嗜好 反复发作心悸、头痛、胸闷2年 晕厥2次 伴腹痛、低热入院 一次发作时血压 200/100mmHg 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 WenzhouWenzhou 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 特殊药物服用：拜复乐罗红，黛力新（停用三天） 辅检：UCG、腹B超、EEG、颈椎MRI，胸部CT 无异常 实验室检查：肝肾功能、电解质、血气正常，心肌坏死标 记物（） 入院前DCG：窦性心律，偶发房早、室早，长QT间期，T波 改变 QTc 0.54 The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhou 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 诊 断 心源性晕厥（尖端扭转性室速） 长QT综合症 查因? The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhou 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 长QT原因 先天性Q-T间期延长 LQT1-7(1-3最常见) 获得性Q-T间期延长 药物诱导Q-T间期延长 心血管药物 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 神病药、抗抑郁药、促进动力药） 其他 心肌病变，电解质紊乱，内分泌疾病，颅内疾 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 WenzhouWenzhou 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 先天性 VS 获得性 家族史 特殊药物使用 对异丙肾反应 先天性 LQTS 晕厥与交感神经活动突然增加 有关 ECG Li Zhang, Katherine W. Timothy, G. et al. Long-QT Syndrome : ECG Findings Identify Genotypes Spectrum of ST-TWave Patterns and Repolarization Parameters in Congenital.Circulation. 2000;102:2849- 2855. The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhou 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 病前服用过拜复乐罗红，黛力新 药物性长QT？ The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhou 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 QTc 0.54 The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhou 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 第三天 QTc 0.61 The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhou 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 入院第三天 突发持续性右侧腹痛 腹部CT点击此处看图 The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhou 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 问题：患者腹部占位与长QT，及尖端扭转 性室速关系？ The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhou 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 入院第三天 去甲肾上腺素 37.32ng/ml (1.7) 肾上腺素 0.1 多巴胺 0.1 入院第六天 去甲肾上腺素 3.72ng/ml (1.7) 肾上腺素 0.1 多巴胺 0.1 The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhou 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 第三天 QTc 0.61 去甲肾上腺素 37.32ng/ml The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhou 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 第六天 QTc 0.43 去甲肾上腺素 3.72ng/ml The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhou 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 肾上腺素能刺激 常与遗传性长QT综合征有关 促进继发Tdp的发生 The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhou 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 肾上腺素能刺激常与遗传性长QT综合征有关 促进继发Tdp的发生 细胞内腺苷酰环化酶活性 激活蛋白激酶A 肾上腺素能 cAMP Ca 2通道及K通道 磷酸化 离子内流增加 EAD的发生。 1肾上腺素能通过蛋白激酶C，使Ca 2通道磷酸 化，使内向电流增加 1肾上腺素能可阻断Ik及Ito，延缓复极，延长AP 时间 对NaK泵刺激使细胞膜超极化，降低心率 The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhou 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 Whalen RK 等报道肾上腺外嗜咯细胞瘤占成人嗜咯 细胞瘤13，儿童30 Whalen RK, Althausen AF, Daniels GH. Extra-adrenal pheochromocytoma. J Urol 1992;147:110. Goldfarb DA等报道好发于2030Y ，高血压+三联 症 Goldfarb DA, Novick AC, Bravo EL, et al. Experience with extra-adrenal pheochromocytoma. J Urol 1989;142:9316. Michaels RD ECG 异常75，首发室性心律失常 罕见 （1990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 WenzhouWenzhou 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 确诊： 嗜咯细胞瘤 继发性长QT间期 尖端扭转性室速 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，QTc 0.40 The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhou 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 谢 谢 The First Affiliated Hospital of The First Affiliated Hospital of WenzhouWenzhou 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 第四天 QTc 0.48 The First Affiliated Hospital of The First Affiliated Hospital of WenzhouW