CAJ | 학술논문

目的探讨短QT综合征(SQTs)先证者及其家系的临床电生理特点和评估心脏性猝死风险并观察疗效.方法对包括先证者在内的14例家系成员进行常规临床检查、血电解质和心肌酶化验、心电图、24 h动态心电图、心电图运动试验、超声心动图、X线胸片等检查,并对高危患者进行心内电生理检查.结果调查的14例家系成员中有4例早年心脏性猝死,4例确诊为SQTs(包括先证者及其两个女儿和一个儿子).阳性患者体表12导联心电图表现为窦性心律下持续性QT间期缩短,QTc≤320 ms,QT/QTp<80%,T波高尖对称,伴ST段缩短.心脏结构均无明显异常.先证者进行了心内电生理检查,经右室流出道S1S2S3(400、250、140ms)刺激后诱发多形性室性心动过速、心室颤动并发生晕厥,后给予埋藏式自动复律除颤器置入治疗,随访6个月无明显异常.结论 SQTs是一种少见的易发生恶性心律失常的遗传性疾病,高危患者置入埋藏式自动复律除颤器能有效预防猝死.
목적 탐토단QT종합정(SQTs)선증자급기가계적림상전생리특점화평고심장성졸사풍험병관찰료효.방법 대포괄선증자재내적14례가계성원진행상규림상검사、혈전해질화심기매화험、심전도、24 h동태심전도、심전도운동시험、초성심동도、X선흉편등검사,병대고위환자진행심내전생리검사.결과 조사적14례가계성원중유4례조년심장성졸사,4례학진위SQTs(포괄선증자급기량개녀인화일개인자).양성환자체표12도련심전도표현위두성심률하지속성QT간기축단,QTc≤320 ms,QT/QTp<80%,T파고첨대칭,반ST단축단.심장결구균무명현이상.선증자진행료심내전생리검사,경우실류출도S1S2S3(400、250、140ms)자격후유발다형성실성심동과속、심실전동병발생훈궐,후급여매장식자동복률제전기치입치료,수방6개월무명현이상.결론 SQTs시일충소견적역발생악성심률실상적유전성질병,고위환자치입매장식자동복률제전기능유효예방졸사.
Objective To observe the clinical characteristics and therapeutic efficacy of a Chinese family with congenital short QT Syndrome (SQTs). Methods Fourteen family members including the proband were screened with routine clinical examination, serum electrolyte, serum myocardial enzymes, electrocardiography (ECG), Holter recording, treadmill exercise test, echocardiography and chest radiograph. High risk patient received intracardiac electrophysiological study (EPS). Results Among the fourteen family members, 4 members died of sudden cardiac death at young ages, 4 members including the preband and his two daughters and one son were diagnosed as SQTs with structurally intact hearts. Initial ECG in these subjects revealed sinus rhythm with a high peaked T wave, QTc interval ≤ 320 ms and QT/QTp <80%. The proband was a 45-year-old man presented with occasional dizziness and family history of sudden cardiac death. Polymorphic ventricular tachyarrhythmias and ventricular fibrillation as well as syncope were induced in this patient during programmed stimulation (S1=400 ms, S2=250 ms, S30=140 ms) from right ventricular outflow tract and a dual chamber Implantable Cardioverter Defibrillator (ICD) was implanted and the patient remained asymptomatic during the 6 months follow up. Conclusions SQTs could be presented as an inherited disorder with increased risk for arrhythmias and sudden cardiac death. ICD implantation is the first-choice therapy for high risk patients.