家族性电紊乱性心脏病未植入心律转复除颤器患者的长期随访研究

Long-term follow-up of patients with familial electric disorder cardiomyopathy without ICD implantation

目的研究家族性电紊乱性心脏病高危患者，未植入心律转复除颤器（ICD）的长期预后。方法13例患者中11例长QT综合征（LQTS）、2例Brugada综合征，均有心脏性晕厥。男性4例，女性9例，平均年龄（44±19）岁。6例（46％）因心跳骤停住院治疗。4例LQTS植入起搏器，平均随访（7±4）年。结果11例（85％）患者仍然发作晕厥，1例心脏骤停首次入院，5例（39％）心脏骤停再入院，2例LQTS死亡，其中1例（0．8％）猝死。结论LQTS和Brugada综合征患者一旦出现晕厥，以后会反复发作，如果没有条件接受ICD治疗，其他的药物治疗、医生的密切监控随访、指导患者避免触发因素和针对家属的心肺复苏训练同样非常重要。

Objective To evaluate the long-term prophylactics of high-risk patients with hereditary arrhythmia at absence of implantable cardioverter defibrillator（ICD） treatment. Methods Eleven cases of congenital long QT syndrome （LQTS） and 2 cases Brugada syndrome were enrolled,4 male ,9 female with average age （44 ± 19 ）years. Routine clinical investigation and electrocardiography（ECG） were delivered. All patients experienced syncope or aborted cardiac arrest. Six cases（46% ） were hospitalized due to cardiac arrest. Four patients with LQTS were implanted pacemakers. Patients were educated to avoid trigger factors of cardiac events and families were trained with cardiac pulmonary resuscitation. Results LQTS patients still experienced syncope or cardiac arrest after propranolol and pacemaker therapy but attacks occurred less than before. One LQTS had her first cardiac arrest. Five LQTS（39% ） had cardiac arrest again during follow-up. Two LQTS cases died. One （0. 8% ） died of sudden cardiac attack. The other died of cerebral hemorrhage. Both Brugada syndrome patients had cardiac arrest and survived again. One case experienced 5 times cardiac arrest and was saved by his wife＇ s cardiac pulmonary resuscitations. Conclusion LQTS and Brugada syndrome patients are in high risk for sudden cardiac death. B-blocker and pacemaker may be effective to improve symptoms. With or without ICD implantation,patients should be educated to avoid trigger factors of cardiac evens and was families should be trained with cardiac pulmonary resuscitation.