Hydroquinidine therapy in Brugada syndrome

OBJECTIVES: We sought to assess hydroquinidine (HQ) efficacy in selected patients with Brugada syndrome (BrS). BACKGROUND: Management of asymptomatic patients with BrS and inducible arrhythmias remains a key issue. Effectiveness of class Ia antiarrhythmic drugs, which inhibit the potassium transient outward current of the action potential, has been suggested in BrS. METHODS: From a cohort of 106 BrS patients, we studied 35 who received HQ (32 men; mean age 48 +/- 11 years). Patients had asymptomatic BrS and inducible arrhythmia (n = 31) or multiple appropriate shocks from an implantable cardioverter-defibrillator (ICD) (n = 4). Asymptomatic patients with inducible arrhythmia underwent electrophysiologic (EP)-guided therapy. When ventricular tachycardia (VT)/ventricular fibrillation (VF) inducibility was not prevented, or in case of HQ intolerance, an ICD was placed. RESULTS: Hydroquinidine prevented VT/VF inducibility in 76% of asymptomatic patients who underwent EP-guided therapy. Syncope occurred in two of the 21 patients who received long-term (17 +/- 13 months) HQ therapy (1 syncope associated with QT interval prolongation and 1 unexplained syncope associated with probable noncompliance). In asymptomatic patients who received an ICD (n = 10), one appropriate shock occurred during a follow-up period of 13 +/- 8 months. In patients with multiple ICD shocks, HQ prevented VT/VF recurrence in all cases during a mean follow-up of 14 +/- 8 months. CONCLUSIONS: Hydroquinidine therapy prevented VT/VF inducibility in 76% of asymptomatic patients with BrS and inducible arrhythmia, as well as VT/VF recurrence in all BrS patients with multiple ICD shocks. These preliminary data suggest that preventive treatment by HQ may be an alternative strategy to ICD placement in asymptomatic patients with BrS and inducible arrhythmia.     

Implantable defibrillator event rates in patients with unexplained syncope and inducible sustained ventricular tachyarrhythmias: a comparison with patients known to have sustained ventricular tachycardia

OBJECTIVES

To assess the clinical significance of inducible ventricular tachyarrhythmias among patients with unexplained syncope.

BACKGROUND

Induction of sustained ventricular arrhythmias at electrophysiology study in patients with unexplained syncope and structural heart disease is usually assigned diagnostic significance. However, the true frequency of subsequent spontaneous ventricular tachyarrhythmias in the absence of antiarrhythmic medications is unknown.

METHODS

In a retrospective case-control study, the incidence of implantable cardiac defibrillator (ICD) therapies for sustained ventricular arrhythmias among patients with unexplained syncope or near syncope (syncope group, n = 22) was compared with that of a control group of patients (n = 32) with clinically documented sustained ventricular tachycardia (VT). Sustained ventricular arrhythmias were inducible in both groups and neither group received antiarrhythmic medications. All ICDs had stored electrograms or RR intervals. Clinical variables were similar between groups except that congestive cardiac failure was more common in the syncope group.

RESULTS

Kaplan-Meier analysis of the time to first appropriate ICD therapy for syncope and control groups produced overlapping curves (p = 0.9), with 57 ± 11% and 50 ± 9%, respectively, receiving ICD therapy by one year. In both groups, the induced arrhythmia was significantly faster than spontaneous arrhythmias, but the cycle lengths of induced and spontaneous arrhythmias were positively correlated (R = 0.6, p < 0.0001). During follow-up, three cardiac transplantations and seven deaths occurred in the syncope group, and two transplantations and five deaths occurred in the control group (36-month survival without transplant 52 ± 11% and 83 ± 7%, respectively, p = 0.03).

CONCLUSIONS

In patients with unexplained syncope, structural heart disease and inducible sustained ventricular arrhythmias, spontaneous sustained ventricular arrhythmias occur commonly and at a similar rate to patients with documented sustained VT. Thus, electrophysiologic testing in unexplained syncope can identify those at risk of potentially life-threatening tachyarrhythmias, and aggressive treatment of these patients is warranted.     

Usefulness of programmed ventricular stimulation in predicting future arrhythmic events in patients with cardiac sarcoidosis

The utility of programmed ventricular stimulation to predict future arrhythmic events in patients with cardiac sarcoidosis is unknown. Similarly, the long-term benefit of implantable cardioverter-defibrillators (ICDs) in cardiac sarcoidosis has not been established. Thirty-two consecutive patients with cardiac sarcoidosis underwent programmed ventricular stimulation. Patients with spontaneous or inducible sustained ventricular arrhythmias (n = 12) underwent ICD insertion. All study patients were followed for the combined arrhythmic event end point of appropriate ICD therapies or sudden death. Mean length of follow-up to sustained ventricular arrhythmia or sudden death was 32 +/- 30 months. Five of 6 patients (83%) with spontaneous sustained ventricular arrhythmias and 4 of 6 patients (67%) without spontaneous but with inducible sustained ventricular arrhythmias received appropriate ICD therapy. Two of 20 patients (10%) with neither spontaneous nor inducible sustained ventricular arrhythmias experienced sustained ventricular arrhythmias or sudden death. Programmed ventricular stimulation predicted subsequent arrhythmic events in the entire population (relative hazard 4.47, 95% confidence interval [CI] 1.30 to 15.39) and in patients who presented without spontaneous sustained ventricular arrhythmias (relative hazard 6.97, 95% CI 1.27 to 38.27). No patient with an ICD died of a primary arrhythmic event. In patients with spontaneous or inducible sustained ventricular arrhythmias, mean survival from first appropriate ICD therapy to death or cardiac transplant was 60 +/- 46 months, with only 2 patients dying or reaching transplant at study end. In conclusion, programmed ventricular stimulation identifies patients with cardiac sarcoidosis at high risk for future arrhythmic events. ICDs effectively terminate life-threatening arrhythmias in high-risk patients, with significant survival after first appropriate therapy.     

Comparison of event rates and survival in patients with unexplained syncope without documented ventricular tachyarrhythmias versus patients with documented sustained ventricular tachyarrhythmias both treated with implantable cardioverter-defibrillators

Patients with unexplained syncope and inducible ventricular tachyarrhythmias during electrophysiologic testing have an increased cardiac mortality rate. We compared event rates and survival of 178 patients with unexplained syncope and no documented ventricular arrhythmias (syncope group) versus 568 patients with documented sustained ventricular tachycardia (VT or fibrillation (VF) (VT/VF group) treated, as part of a lead (Ventritex TVL) investigation, with similar implantable cardioverter-defibrillators (ICDs) capable of extensive data storage. The 2 groups shared similar clinical characteristics. The mean follow-up was 11 months for the syncope group and 14 months for the VT/VF group. The mean time from device implantation to first appropriate therapy was similar in the 2 groups (109 +/- 140 vs 93 +/- 131 days, p = 0.40). Actuarial probability of appropriate ICD therapy was 49% and 55% at 1 and 2 years, respectively, in syncope group and 49% and 58% in VT/VF group (p = 0.57). Recurrent syncope was associated with ventricular tachyarrhythmias in 85% and 92% of the syncope group and VT/VF group, respectively (p = 0.54). At 2 years, actuarial survival was 91% in the syncope group and 93% in VT/VF group (p = 0.85). We conclude that patients treated with ICD with unexplained syncope and induced VT/VF have an equally high incidence of appropriate ICD therapy and low mortality compared with similar patients with documented VT/VF. These findings, plus the high association between recurrent syncope and ventricular arrhythmias, indicate that VT/VF are likely etiologies in selected patients with unexplained syncope and support ICD therapy in such cases.     

Long-term outcome of patients with unexplained syncope treated with an electrophysiologic-guided approach in the implantable cardioverter-defibrillator era.

OBJECTIVES: We evaluated the long-term outcome of patients with coronary artery disease and unexplained syncope who were treated with an electrophysiologic (EP)-guided approach. BACKGROUND: Electrophysiologic studies are frequently performed to evaluate unexplained syncope in patients with coronary artery disease. Patients with this profile who have inducible ventricular tachycardia are considered at high risk for sudden death and increased overall mortality, and therefore are often treated with an implantable cardioverter-defibrillator (ICD). The impact of this EP-guided strategy is unknown because there are no data comparing the long-term outcome of ICD recipients with that of noninducible patients. METHODS: We evaluated 67 consecutive patients with coronary artery disease and unexplained syncope. All patients were treated with an EP-guided approach that included ICD implantation in patients with inducible ventricular tachycardia. RESULTS: Electrophysiologic testing suggested a plausible diagnosis in 32 (48%) of these patients. Inducible monomorphic ventricular tachycardia was the most common abnormality. Despite frequent appropriate therapy with ICDs, the total mortality for patients with inducible monomorphic ventricular tachycardia was significantly higher than for noninducible patients. The respective one- and two-year survival rates were 94% and 84% in noninducible patients and 77% and 45% in inducible patients (p = 0.02). CONCLUSIONS: Electrophysiologic testing suggests an etiology for unexplained syncope in approximately 50% of patients and risk stratifies these patients with regard to long-term outcome. Patients who receive an ICD for the management of inducible ventricular tachycardia have a high incidence of spontaneous ventricular arrhythmias requiring ICD therapy. However, despite ICD implantation and frequent appropriate delivery of ICD therapies, patients with inducible ventricular tachycardia have a significantly worse prognosis than do those who are noninducible.     

Antiarrhythmic drug use in the implantable defibrillator arm of the Antiarrhythmics Versus Implantable Defibrillators (AVID) Study

BACKGROUND: Previous retrospective or observational series suggest that many patients with an implantable cardioverter-defibrillator (ICD) will be treated with antiarrhythmic drugs (AADs) to modify the frequency or manifestation of recurrent ventricular arrhythmias. The relative clinical benefit, however, is uncertain, and deleterious interactions can occur. The objective of this clinical investigation was to study the need for, and effects of, concomitant AAD use with the ICD in a prospectively defined cohort. METHODS: All patients randomly assigned to the ICD arm of the Antiarrhythmics Versus Implantable Defibrillators (AVID) study were followed for the addition of class I or III AADs ("crossover") after hospital discharge. Addition of AADs was strictly regulated by AVID protocol. The timing and reasons for crossover and the effects on ventricular arrhythmia recurrence were analyzed. Patients were excluded if they required AADs before hospital discharge after index arrhythmias or if they had no ventricular arrhythmia before initiation of AADs. RESULTS: After a median follow-up of 135 days, 81 (18%) of the 461 eligible patients required AADs and formed the crossover group. The primary reason for crossover was frequent ICD shocks in 64% of patients. The most common AAD selected was amiodarone (in 42%). Independent predictors of crossover were lower ejection fraction, absence of ventricular fibrillation, or presence of nonsyncopal ventricular tachycardia at presentation, prior unexplained syncope, female sex, and history of cigarette smoking. Before AAD use, the 1-year arrhythmia event rate was 90%; after AAD, the event rate was only 64% (P =.0001). The time to first event was extended from 3.9 +/- 0.7 months to 11.2 +/- 1.8 months. There were 1.4 +/- 3.7 fewer ICD therapy events (P =.005) after crossover, predominantly accounted for by reduction in shocks rather than antitachycardia pacing therapies. CONCLUSIONS: The majority of patients who receive ICDs for sustained ventricular tachycardia or ventricular fibrillation can be treated without AADs. Most commonly, AADs are added to combat frequent ICD shocks, which are successfully reduced by AAD therapy.     

Appropriate ICD therapy in patients with idiopathic dilated cardiomyopathy: long term follow-up

The implantable cardioverter defibrillator (ICD) has proved effective in preventing sudden death and decreasing mortality in randomised secondary prevention trials. Some nonrandomized studies have reported different incidences and predictors of appropriate ICD therapy in patients with idiopathic dilated cardiomyopathy (DCM). The antiarrhythmic and other medical therapies were different between the published studies and it was reported that not using beta-blockers was a predictor of appropriate ICD therapy. In the present study, we report on our long-term experience with ICD therapy in patients with DCM, the majority of whom were treated with beta-blockers and amiodarone. The study population consisted of 25 patients with DCM who underwent initial transvenous ICD implantation between December 1995 and May 2005. Indications for ICD implantation were monomorphic sustained ventricular tachycardia (VT) in 16 patients (64%), cardiac arrest in 8 patients (32%), and syncope plus inducible VT in one patient. Twenty-four patients underwent an electrophysiologic study (EPS). In 18 patients, the ICDs were programmed to only shocks and in 7 patients an additional antitachycardia pacing program was performed. One patient was lost to follow-up and 24 patients were followed-up primarily in our ICD pacemaker outpatient clinic. Appropriate ICD therapy was defined as antitachycardia pacing therapy or shock for tachyarrhythmia determined by evaluation of the clinical information and by device diagnostics to be either ventricular fibrillation or ventricular tachycardia. The mean follow-up was 39.29 +/- 30.59 months after ICD implantation. At follow-up, 17 patients were using a beta-blocker and 16 patients amiodarone. Appropriate ICD therapy was observed in 14 patients (58%). The detected arrhythmias were VT in 12 patients, ventricular fibrillation (VF) in one, and VT and VF in one patient. The time to first ICD therapy was 15.93 +/- 18.45 (range, 1-74) months. Using the Kaplan-Meier method, the percent survival free of appropriate ICD therapy was 82%, 72%, 66%, and 55% at 1, 2, 3, and 4 years follow-up, respectively. The clinical, echocardiographic, and electrophysiologic characteristics did not differ between those who did and did not receive appropriate ICD therapy. However, the mean QRS duration was significantly longer in patients who received appropriate ICD therapies. Cox regression analysis did not reveal any factors that predicted appropriate ICD therapy. Five patients (21%) died during follow-up. Four deaths were classified as cardiac and one as noncardiac. The cumulative survival from total death was 94%, 82%, 82%, and 69%, and the cumulative survival from cardiac death was 94%, 82%, 82%, and 76% during 1, 2, 3, and 4 years of follow-up, respectively. In summary, in this selected patient population with DCM, the majority of patients were unresponsive to beta-blocker and antiarrhythmic therapy. Most of these patients received appropriate ICD therapy during follow-up. Cox regression analysis did not identify any factors that predicted appropriate ICD therapy. Additional trials with larger patient populations are needed to detect the predictors of appropriate ICD therapy in patients with DCM.     

Implications of implantable cardioverter defibrillator therapy in congenital heart disease and pediatrics

INTRODUCTION: The incidence of appropriate and inappropriate discharges, indicators of system failure, and clinical implications of implantable cardioverter defibrillator (ICD) therapy in children and young adults with heart disease is poorly defined. METHODS AND RESULTS: In a retrospective study at a single medical center, a total of 90 ICD procedures were performed in 76 patients younger than age 30 years (median 16 years, range 1-30): 42% with congenital heart disease, 33% with primary electrical disease, 17% with hypertrophic cardiomyopathy, and 8% with idiopathic dilated cardiomyopathy. Indications for ICD included arrest or sustained ventricular tachycardia (n = 27), and combinations of syncope (n = 32), palpitations (n = 17), spontaneous ventricular arrhythmia (n = 40), inducible ventricular tachycardia (n = 36), or severe hypertrophic cardiomyopathy. Transvenous dual-chamber ICDs were implanted in 29 patients. Subcutaneous arrays or epicardial patches were used in 9 patients. Over a median 2-year follow-up, 28% of patients received appropriate shocks for ventricular tachycardia (median 13 months to first shock) and 25% experienced inappropriate shocks for multiple causes (median 16 months). With multivariate analysis, growth strongly correlated with lead failure (odds ratio 73, 3.5-1530, P = 0.006). Complications occurred in 29 patients, including lead failure in 16 (21%), ICD "storm" with sequential shocks in 5, and infection in 2 patients. No deaths were attributable to ICD placement or subsequent device failure. CONCLUSION: ICD therapy can effectively manage malignant arrhythmias in selected pediatric and congenital heart patients. Spurious shocks or ICD storm may increase morbidity and emphasize the need for concomitant medical and ablative therapy. ICD lead failure was relatively frequent in this population.     

Implantable cardioverter defibrillator discharge rates in patients with unexplained syncope, structural heart disease, and inducible ventricular tachycardia at electrophysiologic study

BACKGROUND AND HYPOTHESIS: The implantable cardioverter defibrillator (ICD) is the best available strategy to protect patients from life-threatening ventricular arrhythmia. Although unproven, it is commonly utilized to treat subjects with syncope, a negative clinical workup, structural heart disease, and inducible sustained monomorphic ventricular tachycardia (VT) on programmed electrophysiologic stimulation (EPS). The purpose of this paper was to validate this approach. METHODS: We retrospectively identified 36 subjects who received primary ICD therapy for syncope in the setting of structural heart disease with inducible sustained monomorphic VT on EPS. The cohort was predominantly male (32/36) with underlying coronary artery disease (29/36). The mean left ventricular ejection fraction was 31 +/- 12%, and a third of the patients (12/36) had undergone bypass surgery. RESULTS: The study group was followed for a mean of 23 +/- 15 months (range 3-81 months) and experienced an ICD event rate of 22% at 3 months, which increased to 55% at 36 months. This event rate was comparable with the 66% event rate seen in a group of patients with primary ICD therapy for spontaneous life-threatening VT treated during the same time period. No future predictors of ICD events in the study group could be identified. CONCLUSION: Syncope patients with negative workup, structural heart disease, and sustained monomorphic VT at EPS are at high risk for future tachyarrhythmic events. Based on present evidence, primary ICD therapy in this group appears warranted and justified.     

Implantable cardioverter-defibrillator therapy improves long-term survival in patients with unexplained syncope, cardiomyopathy, and a negative electrophysiologic study

OBJECTIVES: The purpose of this study was to evaluate the long-term outcomes of patients with unexplained syncope, ischemic or nonischemic cardiomyopathy, and a negative electrophysiologic study (EPS). BACKGROUND: EPS is frequently performed to evaluate syncope in patients with left ventricular dysfunction. Limited long-term data evaluating all-cause mortality in patients with no inducible arrhythmia or examining the potential benefits from implantable cardioverter-defibrillator (ICD) therapy are available. METHODS: We evaluated 102 consecutive patients with unexplained syncope, cardiomyopathy, and a negative EPS from September 1996 to December 2000. A blinded matched case-control analysis utilized 51 of these patients (19 treated with an ICD and 32 matched controls treated with conventional therapy). We compared primary endpoint of death and documented cardiac arrest of patients treated with ICD therapy to matched controls. RESULTS: Baseline characteristics were similar between groups. There were 14 primary events among the study population during a follow-up period of 44.3 +/- 20 months: 2 in the ICD group and 12 in the conventional therapy group. The hazard ratio for the risk of event in the ICD group compared with the conventional therapy group was 0.18 (95% confidence interval, 0.04-0.85; P = .04). Other comorbid conditions, including age, sex, ischemic etiology of heart failure, ejection fraction, and antiarrhythmic use, did not predict outcome. Appropriate ICD shocks occurred in 26% of patients at 2 years. CONCLUSIONS: This study suggests that empiric ICD therapy improves long-term outcomes in patients with unexplained syncope, ischemic or nonischemic cardiomyopathy, and negative EPS.     

Reduction in defibrillator shocks with an implantable device combining antitachycardia pacing and shock therapy

Implantable defibrillators reduce the risk of sudden death in patients with malignant ventricular arrhythmias, but significant restriction in quality of life can occur as a result of frequent device activation. To determine if a device that provides both antitachycardia pacing and shock therapy can safely reduce the frequency of shocks after implantation, 46 consecutive patients undergoing initial implantation of a defibrillator were studied. In all patients, the implanted device provided antitachycardia pacing and shock therapy. Detected tachycardia characteristics and the results of therapy were stored in the device's memory. There were 42 men and 4 women, aged 26 to 71 years (mean 58.7 +/- 13.5). Left ventricular ejection fraction ranged from 13% to 67% (mean 32.2 +/- 13.4%) and 31 patients had experienced one or more episodes of cardiac arrest. Induced arrhythmias included sustained monomorphic ventricular tachycardia in 38 patients, nonsustained polymorphic ventricular tachycardia in 2 and ventricular fibrillation in 4. Over a total follow-up period of 255 patient-months (range 1 to 13, mean 6.1), 25 patients experienced spontaneous arrhythmic events. In 22 patients, 909 episodes of tachycardia were treated by antitachycardia pacing, which was successful on 840 occasions (92.4%). Acceleration of ventricular tachycardia by pacing therapy was estimated to have occurred 39 times. Syncope occurred once during pacing-induced acceleration of ventricular tachycardia. Forty-four episodes of tachycardia in seven patients were treated directly by shocks because of short tachycardia cycle length; 88% of all detected tachycardias were treated without the need for shocks. Four patients died from cardiorespiratory failure and one patient died suddenly without any detected tachyarrhythmia.(ABSTRACT TRUNCATED AT 250 WORDS)     

埋藏式心脏复律除颤器治疗恶性室性心律失常的随访观察

对２２例埋置了埋藏式心脏复律除颤器（ＩＣＤ）的有晕厥史的恶性室性心律失常患者进行了随访，观察ＩＣＤ的治疗效果。２２例中扩张型心肌病８例、肥厚型心肌病２例、冠心病７例、ＱＴ延长综合症１例，４例未发现器质性心脏病。所有患者均经临床证实有室性心动过速或（和）心室颤动（ＶＴ／ＶＦ）发作。ＩＣＤ具有多项治疗及信息储存记忆功能（即第三代）。随访时通过体外程控仪调出ＩＣＤ储存的资料进行分析。平均随访７．１（１～２３）个月，１１例患者（５０．０％）ＶＴ／ＶＦ发作１１８次，其中５４次为非持续性ＶＴ，均自行终止；接受ＩＣＤ治疗的６４次为ＶＴ／ＶＦ发作，４５次为ＶＴ，２２次由ＩＣＤ的抗心动过速起搏（ＡＴＰ）终止、１６次由低能量（２～１５Ｊ）转复终止、２次发作为窦性心动过速（误判为ＶＴ），其余５次ＶＴ在ＡＴＰ治疗过程中加速转为ＶＦ，由高能量除颤终止。ＩＣＤ诊断的ＶＦ发作共有１９次，发生于４例患者，有１例患者接受除颤１６次，４次系为频率超过ＶＦ感知频率的ＶＴ。１９次发作均被ＩＣＤ有效除颤终止。２例扩张型心肌病患者，１例术后３个月死于心功能衰竭，另１例术后２个月反复发作ＶＦ，多次接受ＩＣＤ除颤治疗，最后死于心功能衰竭。结果表明常规进行?     

Follow-up of patients with unexplained syncope and inducible ventricular tachyarrhythmias: analysis of the AVID registry and an AVID substudy. Antiarrhythmics Versus Implantable Defibrillators

INTRODUCTION: A prospective registry and substudy were conducted in the Antiarrhythmics Versus Implantable Defibrillators (AVID) Study to clarify the prognosis and recurrent event rate, risk factors, and impact of implantable cardioverter defibrillator (ICD) therapy in patients with unexplained syncope, structural heart disease, and inducible ventricular tachyarrhythmias. METHODS AND RESULTS: Included in the AVID registry were patients from all participating sites who had "out of hospital syncope with structural heart disease and EP-inducible VT/VF with symptoms." In addition, 13 collaborating sites provided more in-depth clinical and electrophysiologic data as part of a formal prospective substudy. Patients in the substudy were followed by local investigators for recurrent arrhythmic events and mortality. Registry patients were tracked for fatal outcomes by the National Death Index. A total of 429 patients with syncope were entered in the AVID registry, of whom 80 participated in the substudy. Of the substudy patients, 21 patients (26%) had inducible polymorphic ventricular tachycardia/ventricular fibrillation (VT/VF), 11 patients (14%) had sustained monomorphic VT <200 beats/min, and 48 patients (60%) had sustained monomorphic VT > or = 200 beats/min. The ICD was used as sole therapy in 75% of the syncope substudy patients (and with antiarrhythmic drug in an additional 9%) and in 59% of the syncope registry patients. Survival rates at 1 and 3 years were 93% and 74% for the substudy patients and 90% and 74% for the registry patients, respectively. Survival of the syncope substudy patients (predominantly treated by ICD) was similar to the VT patients treated by ICD and superior to the VT patients treated by an antiarrhythmic drug (P = 0.05) in the randomized main trial. Mortality events in the substudy were marginally predicted by ejection fraction (P = 0.06) but not by electrophysiologic study-induced arrhythmia. The significant predictor of increased mortality in the registry was age (P = 0.003) and of reduced mortality was treatment with ICD (P = 0.006). CONCLUSION: The results of these analyses support the role of the ICD as primary antiarrhythmic therapy in patients with unexplained syncope, structural heart disease, and inducible VT/VF at electrophysiologic study.     

Brugada综合征的电生理检查和置入性心脏复律除颤器治疗

目的　探讨Brugada综合征的电生理检查和置入性心脏复律除颤器 (ICD)治疗临床研究。方法　心电图自发性或普罗帕酮药物试验表现为Brugada波的 10例患者行电生理检查 ,均为男性 ,平均年龄 (41± 10 )岁 ,经超声心动图和冠状动脉造影检查未发现器质性心脏病。电生理检查诱发心室颤动 (室颤 )并对有条件者置入ICD治疗。结果　 3例有家族性心脏猝死史 ,4例有反复晕厥史 ,其中 2例晕厥发作时心电图记录到室颤。电生理检查 ,AH和HV间期分别为 5 0～ 12 4 (86± 2 1)ms和 4 1～ 84 (5 8± 15 )ms ,4例晕厥者诱发室颤 ,1例心悸者诱发房室折返性心动过速 ,3例有自发性或诱发心房颤动。 4例诱发室颤者中 ,3例置入ICD ;另 1例因经济原因未置入ICD ,随访中发生猝死。1例诱发房室折返性心动过速者作左侧房室旁路射频导管消融治疗。结论　有晕厥症状的Brugada综合征患者 ,经电生理检查 ,室颤有很高的诱发率 ,是猝死的高危人群 ,为了防止猝死应置入ICD治疗。     

Outcome after implantable cardioverter-defibrillator in patients with Brugada syndrome: the Gulf Brugada syndrome registry

Background and ObjectiveAmong patients with Brugada syndrome (BS) and aborted cardiac arrest, syncope, or inducible ventricular fibrillation at electrophysiologic study (EPS), the only currently recommended therapy is an implantable cardioverter-defibrillator (ICD), but these are not without complications. We assessed the total number of shocks (appropriate and inappropriate) and complications related to ICD in patients with BS.Methods and ResultsTwenty-five patients implanted with ICD for BS in 6 Gulf centers between January 1, 2002, and December 31, 2010, were reviewed. Implantable cardioverter-defibrillator indication was based on aborted cardiac arrest (24%), syncope (56%), or in asymptomatic patients with positive EPS (20%). During a follow-up of 41.2 ± 17.6 months, 3 patients (all with prior cardiac arrest) had appropriate device therapy. Four patients developed complications; 3 of them had inappropriate shocks.ConclusionIn our cohort, appropriate device therapy was limited to cardiac arrest survivors, whereas none of those with syncope and/or positive EPS had arrhythmias. Overall complication rate was relatively high, including inappropriate ICD shocks.     

69例植入型心律转复除颤器治疗患者的随访

目的 对全国50家医院69例植入型心律转复除颤器（implantable cardioverter defibrilla-tor,ICD)的患者通过较长期随访获得的实际经验,以期促进和提高我国ICD的应用水平。方法 资料来自全国50家医院的门诊随访、电话询问或厂家随访,通过常规心电图、动态心电图及ICD程控分析仪调出的资料,对串屠 情况及ICD工作情况进行分析,结果 平均随访2.3年中有30例患者接受成功的ICD治理,占全组患者43.5%,总共发作快速心律失常276次,其中VT236次占85.5%,VF40次占14.5%,VT由ATP终止135次,占57.2%,由CV终止101次,占42.8%,VF经DF（除颤）40次,全部一次成功,2例VT加速转成VF,2例无休止发作VT,电击成功,但瞬间又转为VT,2例服胺碘酮后VT频率减慢（140-100次/min),69例中有6例死亡,其中半数因心力衰竭加重所致,结论 ICD的治疗效果肯定,需加强随访及时修改参数,更准确地识别和治疗快速室性心律失常,同时需注意药物的辅助治疗,积极改善心功能,还应加强对患者的心理教育。     

Arrhythmia recurrence in patients with an old myocardial infarct treated by implantable defibrillator: an analysis according to the initial clinical presentation

INTRODUCTION AND OBJECTIVES: The importance of the clinical presentation in the frequency and type of recurrences of ventricular arrhythmias in patients that received an automatic implantable defibrillator is not well known. The purpose of this study was to analyze the frequency and type of recurrences in patients with an old myocardial infarction that received an automatic implantable defibrillator with electrogram recording. METHODS AND RESULTS: We analyzed 100 patients classified in 3 groups according to their clinical presentation: Sustained Monomorphic Ventricular Tachycardia (VT Group n = 65), Cardiac Arrest (CA Group = 19), and Syncope (Syncope Group n = 16). There were no significant differences in the clinical variables among the different groups, nor in the inducibility of arrhythmia at the electrophysiologic study. In a follow-up 27 +/- 14 months, 54% of patients presented at last one episode of sustained ventricular arrhythmia. All recurrences except one were as sustained monomorphic ventricular tachycardia (776 episodes). 81% of episodes of sustained monomorphic ventricular tachycardia (630) were treated with antitachycardia pacing with an effectiveness of 89%. There were no differences in the probability of arrhythmic recurrence among groups but death probability was higher in the ventricular fibrillation group at 36 follow-up months (38% vs 7% and 12% in the sustained monomorphic ventricular tachycardia and syncope groups respectively, p = 0.0113). CONCLUSIONS: In the patients with an old myocardial infarction and malignant ventricular arrhythmias, most of recurrences are due to sustained monomorphic ventricular tachycardia independently of the clinical presentation. The antitachycardia pacing is not only effective in patients with documented sustained monomorphic ventricular tachycardia but also in those that are presented as cardiac arrest or syncope.     

Use of telemetry functions in the assessment of implanted antitachycardia device efficacy.

Twenty patients (aged 50 +/- 21 years and mean left ventricular ejection fraction 37 +/- 17%) with recurrent ventricular arrhythmias were treated with an investigational, implantable combined antitachycardia-pacing cardioverter defibrillator. The device's telemetry capabilities include both stored (1-second snapshots) and real-time display of endocardial and device-circuit signals. The device can store these before, during and after up to 50 tachycardia and antitachycardia pacing episodes. All stored events are indexed to a 24-hour internal clock. During 10.1 +/- 5.1 months of follow-up, the device was used in 11 of 20 patients. In the entire group, antitachycardia pacing was activated on 44 +/- 14 occasions per patient (total 874) and shock delivery occurred on 8 +/- 14 occasions per patient (total 156). Reconstruction by stored telemetry of all device-therapy episodes was possible. Twenty-six percent of all shocks delivered were not appropriate and were due to atrial arrhythmias in 2 patients and dysfunction of the sensing lead in 3. The absence of a relation between symptoms and appropriate shock delivery was documented in 1 patient. Antitachycardia pace acceleration occurred in 5.3% of cases; 7% of attempts at pacing were unsuccessful and needed shock therapy. It is concluded that the enhanced telemetry available in newer antitachycardia devices enables more accurate assessment of device use and enhances diagnosis of inappropriate therapy delivery.     

Effect of cardiac resynchronization therapy on the incidence of ventricular arrhythmias in patients with an implantable cardioverter-defibrillator.

BACKGROUND: Cardiac resynchronization therapy (CRT) reduces mortality in selected patients with heart failure. However, this result may not be entirely related to the beneficial hemodynamic effects of CRT. OBJECTIVES: The purpose of this study was to assess retrospectively the effect of CRT on the incidence of appropriate therapy in patients with an implantable cardioverter-defibrillator (ICD). METHODS: Sixty-five patients (48 men and 17 women; mean age 58 +/- 13 years) with an ICD (31 biventricular, 34 dual-chamber) were included in the study. Clinical, ECG, and ICD stored data and electrograms were collected. RESULTS: Biventricular and dual-chamber ICDs were implanted in 31 and 34 patients, respectively, who had either ischemic (n = 36) or dilated cardiomyopathy (n = 29). Thirty-two (49%) patients received > or =1 appropriate ICD therapy during follow-up of 11 +/- 8 months. Thirty-five percent and 62% of patients with biventricular (n = 11) and dual-chamber ICDs (n = 21), respectively, received appropriate ICD therapy during the follow-up period (odds ratio = 0.340, P = .048). Stratifying the patients according to underlying heart disease and ejection fraction resulted in an adjusted odds ratio = 0.239 (P = .029). Comparing the rate of > or =1 appropriate ICD therapy between the two groups by Kaplan-Meier analysis and the log rank test resulted in P = .027. CONCLUSION: In this retrospective analysis, biventricular pacing was associated with a decreased incidence of sustained ventricular arrhythmias requiring ICD therapy. The antiarrhythmic effect of biventricular pacing could contribute to the reduction in mortality reported in recent large-scale clinical trials on CRT. However, further prospective studies are warranted to clarify this issue.     

Prevalence and prognostic impact of comorbidities in heart failure patients with implantable cardioverter defibrillator.

AIMS: This study assessed the prevalence and the prognostic impact of comorbidities in heart failure patients with implantatable cardioverter-defibrillator (ICD). METHODS AND RESULTS: We prospectively enrolled 146 patients with chronic heart failure, an ICD, and systolic dysfunction (mean ejection fraction 29 +/- 10%). Cardiac death was chosen as the primary endpoint. Death or appropriate ICD therapy, i.e. antitachycardia pacing/shock due to sustained ventricular tachycardia or ventricular fibrillation, was chosen as the secondary endpoint. Seventy-five patients (52%) had chronic kidney disease (defined as an estimated glomerular filtration rate <60 mL/min/1.73 m(2)), 39 patients (27%) were anaemic, and 34 patients (23%) had diabetes mellitus. During a follow-up of 663 +/- 400 days, 22 patients (15%) died, and 41 patients (28%) received an appropriate ICD therapy. By multivariate Cox analysis, independent predictors of cardiac death were chronic kidney disease, age, and NYHA functional class. Death/appropriate ICD therapy were independently predicted by chronic kidney disease and QRS duration. In the presence of chronic kidney disease, outcome was significantly worse when compared with the absence (event-free survival rate 51 vs. 76%, P < 0.001). CONCLUSION: In heart failure patients with an ICD, comorbidities are frequent but only the presence of chronic kidney disease is independently associated with increased morbidity and mortality.