The purpose of the present study is to provide guidelines regarding risk factors that may worsen the Long-QT Syndrome (LQTS), based on available literature. This review evaluates the current knowledge on these risk fa...The purpose of the present study is to provide guidelines regarding risk factors that may worsen the Long-QT Syndrome (LQTS), based on available literature. This review evaluates the current knowledge on these risk factors of acquired LQTS, with an emphasis on non genetic risk factors, including environmental factors. PubMed was searched for literature in English from 1999 to 2011 on the molecular and clinical studies of Long-QT syndrome. We agree, with recent investigations described in the literature, that variety of factors, inherited or environmental, can influence expression of ion channel proteins with impact on repolarization.展开更多
Objectives To explore the optimized program-control mode of a dual-chamber pacemaker combined withβ-blocker to treat congenital long QT syndrome(LQTS). Methods 12 LQTS patients in our hospital that still have symptom...Objectives To explore the optimized program-control mode of a dual-chamber pacemaker combined withβ-blocker to treat congenital long QT syndrome(LQTS). Methods 12 LQTS patients in our hospital that still have symptoms despite use of regular drug therapies or that can not endure the therapies were implanted with DDD cardiac pacemaker.The QT/QTc intervals of those patients were measured at different pacing rates respectively.Their cardiac pacemakers were all programmed to selectively turn on and turn off some related functions at the pacing rate of 80 beats/min.The dosage ofβ-blockers was adjusted according to the patients’ PR intervals and blood pressures.The MACE and the cardiac function of the patients were recorded after operation.Results The measured QT / QTc interval decreased with the pacing rate increasing.The pacing rate of 80 beats/min can make QT/QTc interval basically normal. The MACE of the patients were statistically declined(P = 0.003) and no negative effect on cardiac function was found during the follow-up.Conclusions The optimized program-control mode of a dual-chamber pacemaker combined withβ-blocker to treat congenital LQTS are:to pace at the rate of 80 beats/min and program to turn off lag,sleep,automatic preventing PMT and automatic threshold-capture feature and turn on the PVC,rate adaptation and atrioventricular node priority function.展开更多
Background Congenital long QT syndrome （LQTS） is an inherited ion channel disorder resulting in abnormal cardiac repolarization that can cause syncope and sudden death associated with a prolonged rate-corrected QT i...Background Congenital long QT syndrome （LQTS） is an inherited ion channel disorder resulting in abnormal cardiac repolarization that can cause syncope and sudden death associated with a prolonged rate-corrected QT interval and polymorphic ventricular tachycardia. Several studies in adults showed that LQTS patients have altered QT adaptation to heart rate changes compared with normal subjects which forming a ＂hysteresis loop＂ in the QT-circle length plot. This study was to observe the QT interval changing during exercise testing in children long QT syndrome （LQTS） patients, explore the new diagnosis methods of LQTS. Methods The subjects were divided into 3 groups according to 1993 LQTS diagnostic criteria. Group 1： LQTS group （n=17） who scored 〉 or = 4 points indicating definite LQTS. Group 2： Middle group （n=16）, patients who have prolonged QT interval but scored 1.5 to 3.5. Group 3： Normal control group （n=18）. The average age of all study population is （12.3±5.8） years. No case had beta-adrenergic antagonists administration before exercise testing. All subjects were underwent tread mill exercise testing and electrocardiograph in whole exercise testing and recovery were recorded. QT and heart rate changing during whole exercise testing period were recorded. /kQT, the QT interval at 1, 2, 4, 6 minutes into recovery subtract from the QT interval at a similar heart rate during exercise, were calculated. Results In all three groups, QT intervals were shortening with the increasing of heart rate, but QTc had no significant change. ΔQT at 1 minute （（45±11） ms）, 2 minutes （（37±15） ms）, 4 minutes （（23±12） ms） into recovery in LQTS group were significantly greater than that of the other two groups （P〈0.05, P〈0.01, P〈0.01, respectively）. There was no ΔQT significant difference between middle group and normal control group at recovery time. During the recovery phase in LQTS group, the QT interval remained shortened despite a decelerating heart rate, forming a hysteresis ＂loop＂ in the curve relating the QT interval to the cycle length. Conclusions In children LQTS patients, there is significant QT hysteresis loop in the relation of QT interval with heart rate during recovery of exercise testing, which could be useful to the early diagnosis for LQTS.展开更多
目的:国外文献提示运动平板试验QT间期延长是长QT综合征(LQTS)的表现之一,但国内常规运动平板试验检查未测量分析QT/校正QT(QTc)间期。本研究旨在探索运动平板试验作为国人LQTS辅助诊断操作流程的可行性和有效性。方法:前瞻性连续入选2...目的:国外文献提示运动平板试验QT间期延长是长QT综合征(LQTS)的表现之一,但国内常规运动平板试验检查未测量分析QT/校正QT(QTc)间期。本研究旨在探索运动平板试验作为国人LQTS辅助诊断操作流程的可行性和有效性。方法:前瞻性连续入选2023年7月至2024年7月在中国医学科学院阜外医院行运动平板试验者80例,包括健康对照37例、常见心血管疾病患者25例及基因和临床诊断或疑诊LQTS患者18例,即健康对照组、常见心血管疾病组和疑诊LQTS组。研究对象均行本研究设置的辅助诊断LQTS的运动平板试验检查方案,即在运动平板试验开始前卧位、坐位、立位及运动峰值、恢复期1 min和4 min共6个时间点记录12导联心电图并测量QTc间期。对比3组研究对象在上述6个时间点的QTc间期差异。结果:健康对照组和常见心血管疾病组在运动平板试验检查前后6个时间点的QTc间期均<480 ms,且两组差异均无统计学意义(P均>0.05)。疑诊LQTS组恢复期4 min QTc间期≥480 ms者占66.7%(12/18),其中5例基因检测有明确LQTS致病基因突变(包括1例基因诊断LQTS1型,卧位心电图QTc间期489 ms,恢复期4 min QTc间期636 ms);5例为Schwartz评分≥4分临床确诊患者(基因检测阴性或未检测),另2例Schwartz评分为3分。疑诊LQTS组恢复期4 min QTc间期在445~480 ms者占27.8%(5/18),包括2例临床Schwartz评分≥4分和3例临床疑诊(Schwartz评分2~3分)患者。还有1例临床疑诊(Schwartz评分3分)患者恢复期4 min QTc间期<445 ms,后期基因检测阴性。受试者工作特征(ROC)曲线分析显示,恢复期4 min QTc间期≥482 ms辅助诊断LQTS的灵敏度为83.3%、特异度为98.4%。结论:测量多个时间点QTc间期的运动平板试验检查方案能够有效识别LQTS,在临床实践中有较强的可行性和推广性。展开更多
人类ether-à-go-go相关基因(human ether-à-go-go related gene,hERG)亚家族H成员2(KCNH2)所编码的快速激活延迟整流钾离子通道,是许多药物心脏毒性的靶标。药物诱导性长QT间期综合征(drug-induced long QT syndrome,diLQTS)...人类ether-à-go-go相关基因(human ether-à-go-go related gene,hERG)亚家族H成员2(KCNH2)所编码的快速激活延迟整流钾离子通道,是许多药物心脏毒性的靶标。药物诱导性长QT间期综合征(drug-induced long QT syndrome,diLQTS)是由各类抗心律失常药、抗生素、抗组胺药、抗精神病药和血管扩张药等一个或多个脱靶相互作用而诱导的QT间期延长的病理状态。男性校正后的QT间期(QTc)>450 ms、女性QTc>460 ms是diLQTS心电图的临床表征之一。这种获得性长QT间期综合征容易诱发尖端扭转型室性心动过速,继而进展为心室颤动甚至心脏性猝死。本文从化学结构、心电图学、生物学、电生理学和分子生物学这几方面,综述hERG在diLQTS发生发展中的作用。展开更多
文摘The purpose of the present study is to provide guidelines regarding risk factors that may worsen the Long-QT Syndrome (LQTS), based on available literature. This review evaluates the current knowledge on these risk factors of acquired LQTS, with an emphasis on non genetic risk factors, including environmental factors. PubMed was searched for literature in English from 1999 to 2011 on the molecular and clinical studies of Long-QT syndrome. We agree, with recent investigations described in the literature, that variety of factors, inherited or environmental, can influence expression of ion channel proteins with impact on repolarization.
文摘Objectives To explore the optimized program-control mode of a dual-chamber pacemaker combined withβ-blocker to treat congenital long QT syndrome(LQTS). Methods 12 LQTS patients in our hospital that still have symptoms despite use of regular drug therapies or that can not endure the therapies were implanted with DDD cardiac pacemaker.The QT/QTc intervals of those patients were measured at different pacing rates respectively.Their cardiac pacemakers were all programmed to selectively turn on and turn off some related functions at the pacing rate of 80 beats/min.The dosage ofβ-blockers was adjusted according to the patients’ PR intervals and blood pressures.The MACE and the cardiac function of the patients were recorded after operation.Results The measured QT / QTc interval decreased with the pacing rate increasing.The pacing rate of 80 beats/min can make QT/QTc interval basically normal. The MACE of the patients were statistically declined(P = 0.003) and no negative effect on cardiac function was found during the follow-up.Conclusions The optimized program-control mode of a dual-chamber pacemaker combined withβ-blocker to treat congenital LQTS are:to pace at the rate of 80 beats/min and program to turn off lag,sleep,automatic preventing PMT and automatic threshold-capture feature and turn on the PVC,rate adaptation and atrioventricular node priority function.
文摘Background Congenital long QT syndrome （LQTS） is an inherited ion channel disorder resulting in abnormal cardiac repolarization that can cause syncope and sudden death associated with a prolonged rate-corrected QT interval and polymorphic ventricular tachycardia. Several studies in adults showed that LQTS patients have altered QT adaptation to heart rate changes compared with normal subjects which forming a ＂hysteresis loop＂ in the QT-circle length plot. This study was to observe the QT interval changing during exercise testing in children long QT syndrome （LQTS） patients, explore the new diagnosis methods of LQTS. Methods The subjects were divided into 3 groups according to 1993 LQTS diagnostic criteria. Group 1： LQTS group （n=17） who scored 〉 or = 4 points indicating definite LQTS. Group 2： Middle group （n=16）, patients who have prolonged QT interval but scored 1.5 to 3.5. Group 3： Normal control group （n=18）. The average age of all study population is （12.3±5.8） years. No case had beta-adrenergic antagonists administration before exercise testing. All subjects were underwent tread mill exercise testing and electrocardiograph in whole exercise testing and recovery were recorded. QT and heart rate changing during whole exercise testing period were recorded. /kQT, the QT interval at 1, 2, 4, 6 minutes into recovery subtract from the QT interval at a similar heart rate during exercise, were calculated. Results In all three groups, QT intervals were shortening with the increasing of heart rate, but QTc had no significant change. ΔQT at 1 minute （（45±11） ms）, 2 minutes （（37±15） ms）, 4 minutes （（23±12） ms） into recovery in LQTS group were significantly greater than that of the other two groups （P〈0.05, P〈0.01, P〈0.01, respectively）. There was no ΔQT significant difference between middle group and normal control group at recovery time. During the recovery phase in LQTS group, the QT interval remained shortened despite a decelerating heart rate, forming a hysteresis ＂loop＂ in the curve relating the QT interval to the cycle length. Conclusions In children LQTS patients, there is significant QT hysteresis loop in the relation of QT interval with heart rate during recovery of exercise testing, which could be useful to the early diagnosis for LQTS.
文摘目的:国外文献提示运动平板试验QT间期延长是长QT综合征(LQTS)的表现之一,但国内常规运动平板试验检查未测量分析QT/校正QT(QTc)间期。本研究旨在探索运动平板试验作为国人LQTS辅助诊断操作流程的可行性和有效性。方法:前瞻性连续入选2023年7月至2024年7月在中国医学科学院阜外医院行运动平板试验者80例,包括健康对照37例、常见心血管疾病患者25例及基因和临床诊断或疑诊LQTS患者18例,即健康对照组、常见心血管疾病组和疑诊LQTS组。研究对象均行本研究设置的辅助诊断LQTS的运动平板试验检查方案,即在运动平板试验开始前卧位、坐位、立位及运动峰值、恢复期1 min和4 min共6个时间点记录12导联心电图并测量QTc间期。对比3组研究对象在上述6个时间点的QTc间期差异。结果:健康对照组和常见心血管疾病组在运动平板试验检查前后6个时间点的QTc间期均<480 ms,且两组差异均无统计学意义(P均>0.05)。疑诊LQTS组恢复期4 min QTc间期≥480 ms者占66.7%(12/18),其中5例基因检测有明确LQTS致病基因突变(包括1例基因诊断LQTS1型,卧位心电图QTc间期489 ms,恢复期4 min QTc间期636 ms);5例为Schwartz评分≥4分临床确诊患者(基因检测阴性或未检测),另2例Schwartz评分为3分。疑诊LQTS组恢复期4 min QTc间期在445~480 ms者占27.8%(5/18),包括2例临床Schwartz评分≥4分和3例临床疑诊(Schwartz评分2~3分)患者。还有1例临床疑诊(Schwartz评分3分)患者恢复期4 min QTc间期<445 ms,后期基因检测阴性。受试者工作特征(ROC)曲线分析显示,恢复期4 min QTc间期≥482 ms辅助诊断LQTS的灵敏度为83.3%、特异度为98.4%。结论:测量多个时间点QTc间期的运动平板试验检查方案能够有效识别LQTS,在临床实践中有较强的可行性和推广性。
文摘人类ether-à-go-go相关基因(human ether-à-go-go related gene,hERG)亚家族H成员2(KCNH2)所编码的快速激活延迟整流钾离子通道,是许多药物心脏毒性的靶标。药物诱导性长QT间期综合征(drug-induced long QT syndrome,diLQTS)是由各类抗心律失常药、抗生素、抗组胺药、抗精神病药和血管扩张药等一个或多个脱靶相互作用而诱导的QT间期延长的病理状态。男性校正后的QT间期(QTc)>450 ms、女性QTc>460 ms是diLQTS心电图的临床表征之一。这种获得性长QT间期综合征容易诱发尖端扭转型室性心动过速,继而进展为心室颤动甚至心脏性猝死。本文从化学结构、心电图学、生物学、电生理学和分子生物学这几方面,综述hERG在diLQTS发生发展中的作用。
