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1.
The LQTS is a prime example of how molecular biology, ion channel, cellular, and organ physiology, coupled with clinical observations, promise to be the future paradigm for advancement of medical knowledge. Both the congenital and acquired LQTS are due to abnormalities (intrinsic and/or acquired) of the ionic currents underlying cardiac repolarization. In this review, the continually unraveling molecular biology of congenital LQTS is discussed. The various pharmacological agents associated with the acquired LQTS are listed. Although it is difficult to predict which patients are at risk for TdP, careful assessment of the risk-benefit ratio is important before prescribing drugs known to be able to cause QT prolongation. The in vivo electrophysiological mechanism of TdP in the LQTS is described using, as a paradigm, the anthopleurin-A canine model, a surrogate for LQT3. In the LQTS, prolonged repolarization is associated with increased spatial dispersion of repolarization. Prolongation of repolarization also acts as a primary step for the generation of EADs. The focal EAD induced triggered beat(s) can infringe on the underlying substrate of inhomogeneous repolarization to initiate polymorphic reentrant VT, sometimes having the characteristic twisting QHS configuration known as TdP. The review concludes by discussion of the clinical manifestations and current management of both the congenital and acquired LQTS. The initial therapy of choice for the large majority of patients with the congenital LQTS is a beta-blocking drug. This therapy seems to be effective in LQT1 and LQT2 patients, but may not be as effective in LQT3 patients. Other therapeutic options include pacemakers, cervicothoracic sympathectomy, and the implantable cardioverter defibrillator. Recent molecular genetic studies have suggested several genotype specific therapies; however, long-term efficacy data are not available. 相似文献
2.
RICHARD F. MILLER MICHAEL W. HALEY LASZLO LITTMANN 《Pacing and clinical electrophysiology : PACE》2003,26(1P1):103-104
MILLER, R.F., et al .: Long QT Syndrome: First and Fatal Events Provoked by Hemodialysis. Long QT syndrome (LQTS) involves both congenital and acquired predispositions toward the characteristic torsades de pointes (TP) ventricular arrhythmia. Congenital long QT syndrome generally manifests with TP, syncope, or sudden death early in life. This is a documented case of previously undiagnosed congenital LQTS in a 48-year-old woman where the first and fatal episodes of TP were provoked by hemodialysis. (PACE 2003; 26[Pt. I]:103–104) 相似文献
3.
Cycle Length-Associated Modulation of the Regional Dispersion of Ventricular Repolarization in a Canine Model of Long QT Syndrome 总被引:1,自引:0,他引:1
MASAOMI CHINUSHI EDWARD B. CAREF MARK RESTIVO GIANDOMENICO NOLL YOSHIFUSA AIZAWA NABIL EL-SHERIF 《Pacing and clinical electrophysiology : PACE》2001,24(8):1247-1257
CHINUSHI, M., et al. : Cycle Length‐Associated Modulation of the Regional Dispersion of Ventricular Repolarization in a Canine Model of Long QT Syndrome. Previous tridimensional activation mapping showed that the development of functional conduction block at the onset of torsades de pointes was regionally heterogeneous; conduction block was frequently observed in the LV and the interventricular septum (IVS) but not in the RV, in the canine anthopleurin‐A (AP‐A) model of long QT syndrome (LQTS). This may be related to the distribution of myocytes with M celllike electrophysiological characteristics. To better understand the regional difference of arrhythmogenicity in LQTS, the authors investigated cycle length related modulation of ventricular repolarization among three different layers: the endocardium (End), mid‐myocardium (Mid), and epicardium (Epi) of the LV and RV and at two different areas: the Epi and septum (Sep) in the IVS. The LQT3 model was produced by AP‐A in dogs. Using constant pacing and single premature stimulation (S1S2), the ventricular repolarization pattern was analyzed from 256 unipo2 lar electrograms. Activation‐recovery intervals (ARIs) were used to estimate local repolarization. In seven experiments, AP‐A increased regional ARI dispersion to 88.1 ± 36.0 ms in the LV, to 72.9 ± 35.7 ms in the IVS, and to 23.0 ± 8.7 ms in the RV at the pacing cycle length (CL) of 1,000 ms. Development of the large ARI dispersion was due to greater ARI prolongation at the Mid site in the LV and at Sep site in the IVS. As the S1S2 interval was shortened, regional ARI dispersion decreased gradually, and finally, ARI dispersion showed a reversal gradient of repolarization between the Mid and Epi sites in the LV and between the Sep and Epi sites in the IVS. Two factors contributed to create the reversal gradient of repolarization: (1) a difference in restitution kinetics at the Mid site in the LV and at the Sep site in the IVS, characterized by a larger Δ ARI and slower time constant (τ), and (2) a difference in diastolic intervals at each site resulting in different input to restitution at the same CL. However, the RV showed small alteration in the transmural dispersion of repolarization in the S1S2 protocol. S2 created heterogeneous functional conduction block in the LV and IVS but not in the RV. In the LQT3 model, the arrhythmogenicity of torsades de pointes is primarily due to dispersion of repolarization in the LV and IVS because of prominent distribution of M cells. The RV seems to participate passively in reentrant excitation during torsades de pointes. 相似文献
4.
ALPAY ÇELIKER KÜRÇAD TOKEL ERGUN ÇIL SÜHEYLA ÖZKUTLU ÇENCAN ÖZME 《Pacing and clinical electrophysiology : PACE》1994,17(11):1814-1817
Torsades de pointes is a rare arrhythmia characterized by its bradycardia dependence and increased adrenergic discharge, whether it occurs as a congenital anomaly or as an acquired problem resuiting from drug intoxication or other conditions. There are no reliable tests to assess the propensity toward torsades de pointes or evaluate the efficacy of treatment in these patients. Adenosine can result in marked slowing of sinus and ventricular rate and leads to increased sympathic discharge when given intravenously. We induced torsades de pointes in a child with congenital long QT syndrome (Jervell-Lange-Nielsen syndrome) using 200 μg/kg IV adenosine bolus. Higher dosage of adenosine (600 μg/kg) did not lead to torsades de pointes after β blockade. Adenosine may induce torsades de pointes in patients with the long QT syndrome and may be used as a test to reproduce the clinical arrhythmia. Whether adenosine proves to be useful for assessing the efficacy of treatment will require extensive investigation in larger series of patients. 相似文献
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6.
A 60-year-old woman who had been successfully treated with atenolol and cardiac pacing for hypertension and long QT syndrome developed recurrent episodes of palpitations and syncope. Several days prior to these episodes, indapamide 2.5 mg/day was taken for better control of hypertension. The episodes were associated with prolongation of QT interval and mild hypokalemia (3.1 MEQ/L). Indapamide was immediately stopped, but the QT remained prolonged 2 days later, although the potassium level was normalized. This case suggests that indapamide can cause potassium independent prolongation of the QT interval, resulting in arrhythmia induced syncope. 相似文献
7.
FUMITO MIYOSHI M.D. KAORU TANNO M.D. YOUICHI KOBAYASHI M.D. 《Pacing and clinical electrophysiology : PACE》2013,36(3):e67-e69
This report describes a case of a patient with long QT syndrome (LQTS) with recurrent episodes of torsades de pointes (TdP). Use of biventricular pacing (BiVP) resulted in a shorter QT interval and a shorter T‐peak‐end interval and prevented further episodes of TdP. These findings suggest that BiVP may be helpful in patients with LQTS and refractory TdP. 相似文献
8.
ANDREW E. EPSTEIN MICHAEL J. ROSNER GILBERT R. HAGEMAN JAMES H. BAKER II VANCE J. PLUMB G. NEAL KAY 《Pacing and clinical electrophysiology : PACE》1996,19(7):1095-1104
Although high thoracic left Sympathectomy via art anterior surgical approach is a highly efficacious treatment for refractory ventricular arrhythmias in patients with the long QT syndrome, the degree of sympathetic denervation has been variable, success of the operation is influenced by anatomical differences between patients, and Horner's syndrome may result. We hypothesized that interruption of sympathetic input to the heart could be accomplished using a posterior thoracic approach to this variable and often complex anatomy by division of the sympathetic chain rather than by direct destruction of the stellate and superior thoracic ganglia with the more conventional anterior, supraclavicular approach. In addition, the posterior approach should decrease the risk of Horner's syndrome by avoiding the ocular sympathetic efferent nerves. This posterior approach is described in five patients with the long QT syndrome and recurrent ventricular arrhythmias. After a mean follow-up of 18 ± 12 months, all are alive without Homer's syndrome. 相似文献
9.
ANTONIO E. ALDARIZ HORACIO ROMERO MARTA BARONI HUGO BAGLIVO RICARDO J. ESPER 《Pacing and clinical electrophysiology : PACE》1986,9(6):836-841
A 65-year-old man with arterial hypertension received oral treatment with Ketanserin, a new drug, during a period of five months. He developed marked QT interval prolongation and have several Stokes-Adams attacks. A Holter recording obtained during one of these episodes showed torsade de pointes ventricular tachycardia. The arrhythmias occurred during maximum QT interval prolongation, The correlation between Ketanserin and QT interval prolongation was evaluated by using several Holter studies during administration and withdrawal of the drug. The effect of Ketanserin on the QTc interval was analyzed retrospectively in six patients who had been taking the drug orally. Following a period of four to eight months, the QTc interval was prolonged by the drug (5 to 31%, mean 17%) in five patients. We conclude that torsade de pointes is a potential hazard of long-term treatment with Ketanserin. 相似文献
10.
Nemec J Ackerman MJ Tester DJ Hejlik J Shen WK 《Pacing and clinical electrophysiology : PACE》2003,26(8):1660-1667
Macrovoltage T wave alternans (TWA) has been described in congenital long QT syndrome (LQTS). Microvoltage T wave alternans (microV-TWA) at low heart rate (HR) is a marker of arrhythmogenic risk in many conditions, but its significance in LQTS has not been established. Twenty-three genotypically heterogeneous patients with LQTS and 16 control subjects were studied at rest and during phenylephrine and dobutamine provocation. Genotyping was established by PCR amplification and DNA sequencing of the three most common LQTS genes; KCNQ1/KVLQT1 (LQT1), KCNH2/HERG (LQT2), and SCN5A (LQT3). microV-TWA was determined using Fast Fourier transform. Precluded by ectopy, microV-TWA could not be assessed in 8 of 23 patients with LQTS. In the remaining 15 patients with LQTS, microV-TWA occurred at lower HR in LQTS than in controls (117 +/- 49 vs 153 +/- 37 beats/min; P < 0.05). Patients with LQTS developed microV-TWA at HR < 150 beats/min more often than controls (10/15 vs 2/16; P = 0.003). However, microV-TWA was not detected in the 3 individuals with a history of out-of-hospital cardiac arrest including a 14-year-old male with an F339del-KVLQT1 mutation (LQT1) who had dobutamine-provoked polymorphic ventricular tachycardia requiring external defibrillation. Catecholamine-provoked microV-TWA occurs at lower HR in patients with LQTS than in healthy people but does not identify high risk subjects. 相似文献
11.
Azithromycin-induced torsade de pointes 总被引:1,自引:0,他引:1
Huang BH Wu CH Hsia CP Yin Chen C 《Pacing and clinical electrophysiology : PACE》2007,30(12):1579-1582
Although erythromycin frequently induces long QT interval and torsade de pointes, the newer drug, azithromycin, has rarely been reported to be associated with torsade de pointes. We report here the occurrence of a significant typical QT prolongation within a few hours after taking azithromycin which lead to torsade de pointes. 相似文献
12.
JAN NMEC M.D. MARIE BUNCOVÁ M.D. † VLADIMIR SHUSTERMAN M.D. Ph .D. BRUCE WINTER B.E.T. B.Sc . M.B.A.¶ WIN-KUANG SHEN M.D. ‡ MICHAEL J. ACKERMAN M.D. Ph .D.§ 《Pacing and clinical electrophysiology : PACE》2009,32(1):72-81
Background: Increased QT variability (QTV) has been reported in conditions associated with ventricular arrhythmias. Data on QTV in patients with congenital long QT syndrome (LQTS) are limited.
Methods: Ambulatory electrocardiogram recordings were analyzed in 23 genotyped LQTS patients and in 16 healthy subjects (C). Short-term QTV was compared between C and LQTS. The dependence of QT duration on heart rate was evaluated with three different linear models, based either on the RR interval preceding the QT interval (RR0 ), the RR interval preceding RR0 (RR-1 ), or the average RR interval in the 60-second period before QT interval (mRR).
Results: Short-term QTV was significantly higher in LQTS than in C subjects (14.94 ± 9.33 vs 7.31 ± 1.29 ms; P < 0.001). It was also higher in the non-LQT1 than in LQT1 patients (23.00 ± 9.05 vs 8.74 ± 1.56 ms; P < 0.001) and correlated positively with QTc in LQTS (r = 0.623, P < 0.002). In the C subjects, the linear model based on mRR predicted QT duration significantly better than models based on RR0 and RR-1 . It also provided better fit than any nonlinear model based on RR0 . This was also true for LQT1 patients. For non-LQT1 patients, all models provided poor prediction of QT interval.
Conclusions: QTV is elevated in LQTS patients and is correlated with QTc in LQTS. Significant differences with respect to QTV exist among different genotypes. QT interval duration is strongly affected by noninstantaneous heart rate in both C and LQT1 subjects. These findings could improve formulas for QT interval correction and provide insight on cellular mechanisms of QT adaptation. 相似文献
Methods: Ambulatory electrocardiogram recordings were analyzed in 23 genotyped LQTS patients and in 16 healthy subjects (C). Short-term QTV was compared between C and LQTS. The dependence of QT duration on heart rate was evaluated with three different linear models, based either on the RR interval preceding the QT interval (RR
Results: Short-term QTV was significantly higher in LQTS than in C subjects (14.94 ± 9.33 vs 7.31 ± 1.29 ms; P < 0.001). It was also higher in the non-LQT1 than in LQT1 patients (23.00 ± 9.05 vs 8.74 ± 1.56 ms; P < 0.001) and correlated positively with QTc in LQTS (r = 0.623, P < 0.002). In the C subjects, the linear model based on mRR predicted QT duration significantly better than models based on RR
Conclusions: QTV is elevated in LQTS patients and is correlated with QTc in LQTS. Significant differences with respect to QTV exist among different genotypes. QT interval duration is strongly affected by noninstantaneous heart rate in both C and LQT1 subjects. These findings could improve formulas for QT interval correction and provide insight on cellular mechanisms of QT adaptation. 相似文献
13.
MASAOMI CHINUSHI IWAO NAKAGAWA TOMOYUKI HORI FUMIO YAMASHITA TAKASHI WASHIZUKA YOSHIFUSA AIZAWA 《Pacing and clinical electrophysiology : PACE》2001,24(10):1561-1562
Intracoronary acetylcholine administration, which was performed to exclude vasospasms, unmasked an abnormal QT interval prolongation and initiated torsades de pointes in a patient with normal QT interval at rest. 相似文献
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15.
Mancuso EM Brady WJ Harrigan RA Pollack M Chan T 《The Journal of emergency medicine》2004,27(4):385-393
Long QT Syndrome is a cardiac disorder caused by an abnormal prolongation of the ventricular repolarization phase. The primary concern in this syndrome is the propensity towards polymorphic ventricular tachycardia and sudden cardiac death. This article presents several cases, highlighting the pathophysiology, clinical presentation, and management of this disorder. 相似文献
16.
WALTER W. CHIEN ELYSE FOSTER BARRY PHILLIPS NELSON SCHILLER JERRY C. GRIFFIN 《Pacing and clinical electrophysiology : PACE》1991,14(8):1209-1212
A patient with long QT syndrome was treated with beta blockers and had a permanent DDD pacemaker implanted. The lower rate was set to 85 beats/min because this provided the best shortening of QT interval at the lowest paced heart rate. The atrioventricular (AV) delay was programmed to 250 msec to allow native AV conduction. Patient returned complaining of symptoms suggestive of pacemaker syndrome. ECG during one of these episodes showed AV sequential pacing. Doppler echocardiography of hepatic vein flow suggested atrial contraction against a closed tricuspid valve. Endocardial electrogram telemetry demonstrated ventriculoatrial (VA) conduction with the retrograde atrial electrogram falling within the atrial refractory period and thus was not sensed. The following atrial stimulus did not capture because of the atrial refractoriness. Ventricular pacing proceeded after the programmed AV delay. Reprogramming the AV delay to 200 msec restored AV synchrony by allowing the atrial stimulus to capture by placing it outside of the refractory period of the atrium. No further symptoms reported during six months of follow-up. 相似文献
17.
《Journal of PeriAnesthesia Nursing》2020,35(2):104-111
Prolongation of the QT interval can predispose patients to fatal arrhythmias such as torsade de pointes. While arrhythmias can occur spontaneously in patients with a genetic predisposition, drugs such as ondansetron and droperidol, which are frequently used in the perioperative period, have been implicated in the prolongation of the QT interval. As the list of medications that cause QT prolongation grows, anesthesia providers and perioperative nurses must be informed regarding the importance of the QT interval. This article reviews the physiology and measurement of the QT interval, the risk factors of QT prolongation, the mechanism of drug-induced QT prolongation, and perioperative considerations for patient care. 相似文献
18.
Konstantinos P. Letsas Michalis Efremidis Stavros P. Kounas Loukas K. Pappas Gerasimos Gavrielatos Ioannis P. Alexanian Nikolaos P. Dimopoulos Gerasimos S. Filippatos Antonios Sideris Fotis Kardaras 《Clinical research in cardiology》2009,98(4):208-212
The present study aimed to investigate the causative medications and underlying risk factors that predispose to drug-induced
QT interval prolongation. Twenty-one patients with drug-induced long QT (90% females, mean age 64.3 ± 14.1 years) were included
in the study. Transthoracic echocardiography as well as continuous or ambulatory 48-h electrocardiographic monitoring was
carried out in all patients during their hospitalization. The mean corrected QT (QTc) interval was 542 ± 56.8 ms. Known cardiac
agents (mainly class III antiarrhythmics) were implicated in 13/21 (62%), antipsychotics in 8/21 (38%), and antibiotics in
5/21 patients (24%). Potential drug-interactions through inhibition of cytochrome P450 isoenzymes were considered responsible
in 5/21 cases (24%). The underlying cardiovascular diseases included hypertension (57%) with left ventricular hypertrophy
(29%), paroxysmal atrial tachyarrhytmias (48%), heart failure (14%), valvular heart disease (10%), and coronary artery disease
(5%). Torsade de pointes (TdP) was recorded in 6/21 of patients, and cardiac arrest necessitating resuscitation occurred in
five of them. A significant correlation was observed between administration of cardiac agents and TdP events (P < 0.05). TdP and cardiac arrest events were both associated with a QTc interval >510 ms (P < 0.05). Advanced age (>60 years), female gender, hypertension and paroxysmal atrial tachyarrhytmias were the most common
identifiable pre-existing factors for drug-induced long QT in our patient cohort. Marked QTc interval prolongation should
be considered of prognostic significance for TdP and cardiac arrest events. 相似文献
19.
JIN-TAI ZHOU LIANG-RONG ZHENG WEI-YU LIU 《Pacing and clinical electrophysiology : PACE》1992,15(11):2164-2168
Torsade de pointes (TdP) syncopal episodes were almost invariably precipitated by emotional stress or menstruation in a 17-year-old girl. V wave accentuation occurred during sinus rhythm without pauses in periods of heightened sympathetic tone. To examine the role of early afterdepolarization (EAD), monophasic action potentials were recorded during ventricular extrasystoles and TdP occurring spontaneously and induced by ventricular pacing. The effects of lidocaine, verapamil, propranolol, and epinephrine were assessed. Our data show that: (1) EAD plays a significant role in the genesis of familial long QTU syndrome and TdP; (2) rapid ventricular pacing causes postpause-dependent EADs, U waves, and TdP; and (3) EAD is enhanced by epinephrine infusion in the absence of pause, whereas EAD-triggered firing is inhibited by verapamil and propranolol but not by lidocaine. 相似文献
20.
探讨安置起搏器患者中发生QT间期延长伴尖端扭转型室速 (LPTs -TdP)的早期诊断和治疗。方法 :对本文报道的 6例患者进行综合分析其病因 ,机制 ,临床特点及心电图变化 ,结果 :被抢救的 6例患者 ,3例为典型性LPTs -TdP ,3例为非典型性LPTs-TdP ,6例中 5例抢救成功 ,1例失败。结论 :有晕厥发作史的起搏患者应警惕合并存在LQTs -TdP。 相似文献