Prognostic significance of syncope in patients with Wolff-Parkinson-White syndrome

The prognostic value of syncope in symptomatic patients with Wolff-Parkinson-White syndrome (WPW) is unknown. Therefore, in order to evaluate the sensibility, specificity, positive and negative predictive value of syncope and compare those values with the one obtained for the shortest RR interval (less than or equal to 250 msec) as well as for the anterograde refractory period of the accessory pathway (less than 270 msec), we reviewed the clinical and electrophysiological data of 158 symptomatic patients with WPW. Fourty-eight patients (30%) reported at least one episode of syncope, and 24 out of 158 patients experienced an aborted sudden death, probably due to rapid conduction via the accessory pathway during atrial fibrillation. Syncope has poor sensibility but high specificity in recognizing an aborted sudden death. However, the syncope demonstrated it had a lower prognostic value when compared with other electrophysiological parameters in correctly identifying patients with a history of ventricular tachycardia and/or fibrillation. In conclusion, the data of this study propose the symptom "syncope" as a frequent event in the history of symptomatic patients with WPW referred to electrophysiological study. Generally its presence does not correctly identify patients who experienced an aborted sudden death. Furthermore, its prognostic value is significantly lower than a shorter RR interval (less than or equal to 250 msec) during atrial fibrillation and an anterograde effective refractory period less than 270 msec.     

Prediction of a fatal atrial fibrillation in patients with asymptomatic Wolff-Parkinson-White pattern

Paroxysmal atrial fibrillation (PAF) in patients with manifest WPW syndrome can be a life-threatening arrhythmia by deterioration into ventricular fibrillation. In patients with asymptomatic WPW pattern, the first PAF may lead to ventricular fibrillation or sudden death. Therefore, the purpose of this study was to predict a fatal PAF in patients with asymptomatic WPW pattern. The patient population was divided into two groups: (1) 145 patients with manifest WPW syndrome, excluding intermittent ones (32 with an episode of PAF, 49 with AV reciprocating tachycardia alone, and 64 without any episode of paroxysmal tachyarrhythmia), and (2) mixed group of patients with and without WPW syndrome (36 with an episode of PAF and 66 without PAF). The results were as follows: (1) (a) out of 32 patients with WPW syndrome and PAF, 8 patients were observed to have both the shortest preexcited R-R interval of less than 200 msec during PAF and the shortest antegrade effective refractory period of the accessory pathway (ERP-AP) of less than 250 msec, 7 of whom had dizziness or syncope during PAF and 2 died suddenly during the follow-up period; (b) 21 (32.8%) out of 64 patients with asymptomatic WPW pattern showed the shortest antegrade ERP-AP less than 250 msec; (2) patients with PAF had a higher tendency to develop repetitive atrial firing (RAF), as well as fragmented atrial activity (FAA), which were induced using programmed atrial stimulation.(ABSTRACT TRUNCATED AT 250 WORDS)     

Relation of syncope in young patients with Wolff-Parkinson-White syndrome to rapid ventricular response during atrial fibrillation

Syncope in patients due to Wolff-Parkinson-White (WPW) syndrome may be related either to a rapid rate of supraventricular tachycardia or to rapid ventricular response over the accessory pathway during atrial fibrillation (AF). From 1982 to 1987, 74 patients less than or equal to 25 years old (mean age 12.6 years) with WPW syndrome on electrocardiogram underwent electrophysiologic study. Of the 74 patients, 14 (19%) had a history of syncope. During electrophysiologic study 9 of 14 patients with syncope had sustained (greater than 5 minutes or requiring termination due to hypotension) AF. Of the remaining 5 patients, 3 had inducible nonsustained AF and 2 had no AF. None of the 60 patients without syncope developed sustained AF; 34 had nonsustained and 26 had no AF. Occurrence of sustained AF had a sensitivity of 64% and specificity of 100% for history of syncope. All patients with syncope and AF (12) had a short RR interval between 2 consecutive preexcited QRS complexes during AF at less than or equal to 220 ms, in contrast to 9 of 34 patients without syncope (p less than 0.001, sensitivity 100%, specificity 74%). No patient with a short RR interval between 2 consecutive preexcited QRS complexes during AF of greater than 220 ms had a history of syncope. Thus, in these young patients with WPW syndrome, occurrence of AF with a rapid ventricular response during electrophysiologic study correlated well with a history of syncope and may be the cause of syncope in most patients. Electrophysiologic study may be helpful in identification of young patients with WPW at risk for syncope.     

Sudden death in the Wolff-Parkinson-White syndrome

Clinical electrophysiologic studies in patients with Wolff-Parkinson-White syndrome (WPW) suffering from ventricular fibrillation have shown a high prevalence of short anterograde refractory period of the accessory pathway (less than or equal to 250 ms), short preexcited RR intervals during atrial fibrillation (less than or equal to 250 ms), and multiple accessory pathways. Unfortunately the specificity of these findings is low, as they are present in almost 50% of patients with WPW without a history of ventricular fibrillation, and in 17% of patients with asymptomatic WPW. Pharmacologic and exercise testing detect a population of WPW with a low probability of having a short anterograde refractory period of the accessory pathway, but don't rule-out the ability of these patients to develop very short RR intervals during atrial fibrillation. Natural history studies show that sudden death in WPW occurs with an incidence less than or equal to 1:1,000 per year. The low predictive value of electrophysiologic and noninvasive studies for sudden death, makes then a poor means for screening patients at risk. Some clinical factors, such as the frequency of tachycardias and/or the detection of episodes of atrial flutter or fibrillation are markers of higher sudden death risk, and indications for aggressive electrophysiologic evaluation.     

Sinus bradycardia and atrial fibrillation associated with the Wolff-Parkinson-White syndrome

Thirty-three patients with atrial fibrillation associated with the Wolff-Parkinson-White (WPW) syndrome were studied to determine the relation of sinus bradycardia and atrial fibrillation. In seven patients the sinus rate was less than 40 beats/min and sinus nodal disease was considered a cause of the periods of bradycardia. Ventricular fibrillation or functional cardiac arrest was documented in four instances. Twenty-six patients demonstrated a type A and seven a type B WPW pattern during periods of sinus rhythm. Male patients predominated. The average age was 38.5 years among patients with a type A pattern compared with 25.3 years among those with a type B pattern. The shortest R-R cycle length in this group was 130 msec during a period of atrial fibrillation. Five thousand serial microscopic sections were studied in one patient who demonstrated ventricular fibrillation. Three anomalous pathways were located in this patient with the widest tract, 380 μ, containing about 400 muscle cells. Most of the sinoatrial node was replaced by collagen elastic fibers, and there was widespread destruction of the atria with a marked increase in fibrous connective tissue.Ventricular fibrillation or functional cardiac arrest is not a rare arrhythmia in patients with atrial fibrillation associated with the WPW syndrome and may be responsible for sudden death in patients with these arrhythmias. Hence, precise electrophysiologic studies and pharmacologic or surgical management, or both, are suggested to prevent sudden death in patients with short refractory periods associated with atrial fibrillation and the WPW syndrome.     

Automated external defibrillator documented degeneration of pre-excited atrial fibrillation into ventricular fibrillation

Wolff–Parkinson–White (WPW) syndrome can be the cause of syncope or sudden cardiac death, which results from ventricular fibrillation (VF) degenerated from rapid anterograde conduction of atrial fibrillation (AF) to the ventricles through the accessory pathway. We present a case of WPW syndrome in which recording the actual moment of onset of the degeneration of pre-excited AF into VF. This was fortuitous and also lucky for this patient.     

Prevalence of Supraventricular Tachyarrhythmias in a Cohort of 115 Patients with Brugada Syndrome

Background : The Brugada syndrome is characterized by ST segment elevation in leads V₁ to V₃ and a right bundle branch block like pattern. It is associated with an increased risk of syncope and sudden cardiac death. Initial reports in small numbers of patients suggest an association between supraventricular tachycardias and Brugada syndrome with a prevalence varying between 13% and 40%. Objective : Aim of this study was to evaluate the prevalence of AV nodal reentrant tachycardia, AV reentry tachycardia, and/or atrial fibrillation in a large cohort of patients diagnosed as Brugada syndrome. Methods and Results : From three different European centers 115 consecutive patients with a Brugada syndrome were evaluated noninvasively and invasively (mean age 45 ± 12 years, n = 82 men, n = 33 women). Nineteen of 115 patients (17%) had a history of previous cardiac arrest. Syncope was reported by 58 patients (50%), 33 patients had a positive family history of sudden cardiac death (29%). Supraventricular tachycardias were documented in 26 of the patients (23%): Eight patients (7%) had AV‐nodal reentrant tachycardias and two patients had AV‐reentry tachycardias; atrial tachycardias were documented in three patients, and another 13 patients (11%) suffered from atrial fibrillation/atrial flutter. Additionally, atrial fibrillation was inducible by programmed atrial stimulation in nine patients (8%). Conclusions : Supraventricular tachycardias occur in 23% of patients with Brugada syndrome. Documentation of atrial fibrillation especially in the young or supraventricular tachycardias associated with syncope should give reason to screen for Brugada syndrome.     

Natural history of patients with unexplained syncope and a nondiagnostic electrophysiologic study

The purpose of this study was to define the natural history of 99 patients with unexplained syncope who underwent an electrophysiologic test that either was entirely normal or demonstrated nonspecific abnormalities that were nondiagnostic (inducible polymorphic ventricular tachycardia or ventricular fibrillation, a mildly prolonged sinus node recovery time of less than 2 s, a His-ventricular interval of 55 to 99 ms or supraventricular tachycardia not associated with hypotension). The mean age (+/- SD) of the patients was 56 +/- 19 years; structural heart disease was present in 47 patients and absent in 52. Complete follow-up was available in 95 patients. During 20 +/- 11 months of follow-up, 2 patients (2%) died suddenly, 19 patients (20%) had recurrent syncope and 74 patients (78%) had no further episodes of syncope. Among the 19 patients who continued to have syncope after the electrophysiologic testing, the cause of syncope was established clinically in 4 and was found to be high degree atrioventricular (AV) block (2 patients) or sinus node dysfunction (2 patients). No clinical or laboratory findings distinguished patients who had sudden death or syncope during follow-up from patients who did not. In conclusion, in patients with unexplained syncope who undergo an electrophysiologic test that is nondiagnostic 1) the incidence of sudden death is low (2%); 2) the remission rate of syncope is high (80%); 3) the electrophysiologic test may be documented to have been falsely negative in greater than or equal to 20% of patients who continue to have syncope, syncope in these patients being caused by AV block or sinus node dysfunction; and 4) patients at risk of sudden death or recurrent syncope, or both, cannot be readily identified prospectively.     

Lack of prognostic value of syncope in patients with Wolff-Parkinson-White syndrome

Syncope in patients with Wolff-Parkinson-White syndrome may be considered a premonitory event heralding the future development of sudden death. Therefore, the clinical and electrophysiologic data of 101 patients with Wolff-Parkinson-White syndrome referred for invasive evaluation of known arrhythmias were reviewed to assess the incidence and clinical relevance of syncope. Thirty-six patients reported the occurrence of one or more syncopal episodes (group 1) and 65 patients had no syncope (group 2). These two groups did not differ significantly with regard to age, gender, incidence and characteristics of arrhythmia, clinical history, frequency of arrhythmic events and presence of associated cardiac disease. There were 10 patients in group 1 and 12 in group 2 who had ventricular fibrillation. There were no statistical differences between the two groups with respect to the effective refractory period of the right atrium, atrioventricular node, accessory pathway and right ventricle. Furthermore, no differences between the two groups were noted with respect to cycle length of circus movement tachycardia, mean heart rate during atrial fibrillation, and minimum RR interval during atrial fibrillation. In addition, the accessory pathway location was not significantly different between group 1 and group 2. The occurrence of syncope could not be predicted from any electrophysiologic finding and this symptom had a low sensitivity and specificity for recognition of dangerous rapid heart rates. Furthermore, the prognostic value of syncope was less accurate and predictive than the shortest RR interval during atrial fibrillation and the anterograde effective refractory period of the accessory pathway for aborted sudden death occurrence.(ABSTRACT TRUNCATED AT 250 WORDS)     

Susceptibility to atrial fibrillation and ventricular tachyarrhythmia in the Wolff-Parkinson-White syndrome: role of the accessory pathway

Clinical and electrophysiologic characteristics associated with spontaneous and inducible atrial fibrillation and ventricular tachyarrhythmia were assessed in 20 consecutive patients with Wolff-Parkinson-White (WPW) syndrome undergoing surgical division (n = 12) or transcatheter electrical ablation (n = 8) of accessory pathways. Patients with spontaneous atrial fibrillation were characterized by the trend (not significant) of a shorter antegrade accessory pathway effective refractory period (256 +/- 26 vs 303 +/- 109 msec). However, patients with and without spontaneous atrial fibrillation did not differ with respect to prevalence of structural heart disease (3 of 11 vs 2 of 9), intra-atrial conduction time (34 +/- 10 vs 32 +/- 10 msec), or interatrial conduction time (86 +/- 21 vs 88 +/- 17 msec). Thus, atrial and accessory pathway electrophysiologic properties (per se) were not clear determinants of susceptibility to atrial fibrillation. Among the 20 patients, 10 to 35 beats of nonsustained ventricular tachycardia (seven patients) or ventricular fibrillation (three patients) were induced at electrophysiologic study with one to three programmed extrastimuli. Clinically, a ventricular arrhythmia (ventricular fibrillation during atrial fibrillation) had occurred in only one of these patients. The discordance of these observations was significant (p less than 0.01). Patients with and without inducible ventricular arrhythmias were not distinguished by clinical factors or by electrophysiologic properties of the accessory pathway or ventricles. Accessory pathway conduction was completely or partially eliminated by ablation procedures in 14 of 20 patients. During a mean follow-up of 27 months, atrial fibrillation recurred in two patients with failed ablation procedures and in one patient with left atrial enlargement (despite accessory pathway division) (p = 0.019 vs pre-ablation). Ventricular arrhythmias remained inducible in two patients in whom accessory pathway ablation failed (p = 0.01 vs initial study). However, spontaneous ventricular tachyarrhythmias did not occur during follow-up. We conclude that susceptibility to spontaneous or inducible atrial fibrillation and ventricular tachyarrhythmia in patients with WPW syndrome and no organic heart disease depends primarily on the existence of a functional accessory pathway. These susceptibilities are eliminated by interruption of accessory pathway conduction. Ventricular tachyarrhythmias remain infrequent spontaneous events in the WPW syndrome. Their more frequent induction at electrophysiologic study is not predictive of clinical occurrence.     

Arrhythmias in patients with Brugada-type electrocardiographic findings.

Brugada syndrome is characterized by marked ST-segment elevation in the right precordial leads (Bru-ECG) and is associated with a high risk for sudden death. However, it is unclear whether the arrhythmogenesis is caused by the mechanisms responsible for Bru-ECG. The present study investigated the risk of arrhythmias in patients with Bru-ECG by retrospectively analyzing 30 patients (28 men; mean age, 51+/-14 years) with Bru-ECG. Aborted sudden cardiac death (ventricular fibrillation or syncope) occurred in 9 patients (30%); paroxysmal atrial fibrillation was present in 9 (30%) patients in addition to malignant ventricular arrhythmias, and some type of arrhythmic event (aborted sudden cardiac death or paroxysmal atrial fibrillation) occurred in 15 patients (50%). Of all the arrhythmic events, 93% occurred at night or early in the morning, and 92% had pronounced ST-segment elevation. These results suggest that Bru-ECG may be associated not only with an increased risk of ventricular tachyarrhythmias but also with an increased risk of paroxysmal atrial fibrillation, and that the arrhythmogenesis may be related to the pronounced ST-segment elevation.     

Influence of age on the presumed cause of syncope in patients with the Wolff-Parkinson-White syndrome

The aim of this study was to assess the causes of syncope in patients with the Wolff-Parkinson-White syndrome (WPW) and to determine whether the age of the patients was a significant factor. Forty-seven patients with a WPW, aged 11 to 72 years, underwent electrophysiological study by the oesophageal approach because of an unexplained syncope. Nineteen patients were under 20 years of age (16 +/- 3 years: group I) and 28 were over 20 years of age (40 +/- 13 years: group II). Junctional tachycardia was induced in 8 patients of group I (42%) and in 13 of group II (46%) (NS); atrial fibrillation was induced in 8 patients of group I (42%) and in 9 of group II (35%) (NS). A potentially malignant form of WPW was identified in 8 patients of group I (42%) and in 11 of group II (39%) (NS); Syncope was directly attributed to the WPW in 14 patients of group I (74%) and in 19 of group II (78%), either after identification of a serious form or induction of junctional tachycardia (6 patients of group I and 8 of group II). The rest of the syncopal episodes had various causes. There were no deaths. The authors conclude that oesophageal electrophysiological investigations enable rapid identification of a high incidence of tachycardias probably responsible for syncope in WPW. The causes of syncope and incidence of potentially severe forms of WPW were not significantly influenced by the age of the patients.     

Risk of sudden death after successful accessory atrioventricular pathway ablation in resuscitated patients with Wolff-Parkinson-White syndrome

INTRODUCTION: Cardiac arrest in patients with Wolff-Parkinson-White (WPW) syndrome can be due to ventricular fibrillation mediated by fast conduction over the accessory pathway during atrial fibrillation. However, if primary ventricular fibrillation is the reason for resuscitation, placement of an implantable cardioverter defibrillator (ICD) would be indicated. The aim of this study was to test the hypothesis that in resuscitated patients with WPW syndrome, recurrences can be prevented by sole ablation of their accessory pathways. METHODS AND RESULTS: We performed a long-term follow-up study of 48 resuscitated patients with WPW syndrome who underwent successful accessory pathway ablation as their sole primary treatment. Cardiac arrest had occurred either spontaneously in 32 patients (group A) or after intravenous administration of antiarrhythmic drugs in 16 patients (group B) and was never associated with an acute myocardial infarction or other concomitant factors. All patients had normal left ventricular function at echocardiography. A total of 56 accessory AV pathways were ablated successfully with radiofrequency current (n = 55) or during surgery (n = 1) and were located at the left free wall (n = 35), right free wall (n = 8), or septal-paraseptal region (n = 13). Follow-up 5.0+/-1.9 years after ablation (range 0.2 to 7.9) was obtained in all 48 patients. All of the patients were alive, and none had a life-threatening arrhythmia or syncope after successful ablation of their accessory pathways. CONCLUSION: In resuscitated patients with WPW syndrome who have normal left ventricular function at echocardiography and no ECG abnormalities suggesting additional electrical disease, ablation of their overt accessory pathways prevented cardiac arrest recurrences; therefore, ICD placement is generally not indicated.     

Ventricular fibrillation in the Wolff-Parkinson-White syndrome

Ventricular fibrillation (VF) is a well-known but rare complication of the Wolff-Parkinson-White syndrome (WPW). Clinical and electrophysiological data of 23 patients with spontaneous VF were compared with data from 100 consecutive patients with WPW without VF but with symptomatic supraventricular tachycardia. The 23 patients were collected in a multicentre retrospective study in seven European centres. VF occurred in only one patient who was receiving antiarrhythmic drugs, and was the first manifestation of the syndrome in six. No significant differences were found between those with VF and without VF in age, complaints of palpitations, syncope, and presence of structural heart disease. The retrograde effective refractory period of the accessory pathway, the atrial refractory period and the fastest atrial pacing rate with 1:1 anterograde conduction over the accessory pathway were similar in both groups. Significant differences were found for sex, permanent pre-excitation on the electrocardiogram, type of documented supraventricular tachyarrhythmias, shortest RR interval less than or equal to 220 ms during spontaneous atrial fibrillation (AF), inducibility of supraventricular tachycardias, ventricular effective refractory period less than or equal to 190 ms, mean shortest RR interval during induced AF less than or equal to 180 ms and presence of multiple accessory pathways.(ABSTRACT TRUNCATED AT 250 WORDS)     

Flecainide acetate in the treatment of supraventricular tachycardias: value of programmed electrical stimulation for long-term prognosis

Twenty patients with recurrent symptomatic supraventricular tachycardia were studied to estimate the efficacy of flecainide in the long-term treatment of these arrhythmias and to evaluate the prognostic value of programmed electrophysiologic stimulation. All patients had their arrhythmia inducible at baseline evaluation. Nine patients had a Wolff-Parkinson-White (WPW) syndrome, five had a concealed bypass tract, and two had dual atrioventricular (AV) nodal pathways. In the remaining patients there was an intraatrial reentry circuit. Previous medication was no to five antiarrhythmic drugs (mean 2.4 drugs). At baseline, a circus movement tachycardia was induced in 12, AV nodal tachycardia was induced in two, atrial tachycardia was induced in three, atrial fibrillation was induced in five, and a flutter was induced in two patients. After flecainide, 2 mg/kg intravenously in 10 minutes, six patients no longer had their arrhythmia inducible. In the WPW patients, atrial fibrillation was no more inducible. In 65% of the patients there was no recurrence during a follow-up period of 11 +/- 10 months. None of the six patients who no longer had their arrhythmia inducible had a recurrence of the tachycardia over a period of up to 3 years. Seven of the other 14 patients (who still had their arrhythmia inducible) had a recurrence of the tachycardia. Positive and negative predictive values are 50% and 100%, respectively. We conclude that flecainide prevents recurrences of supraventricular tachycardias in 65% of patients with inducible supraventricular tachycardias during a mean follow-up of 11 months. Programmed electrical stimulation has a high negative predictive value in this setting. Flecainide is especially effective in preventing atrial fibrillation in patients with WPW syndrome.     

The long QT syndrome: a prospective international study

During the past 4 years 196 patients with the idiopathic long QT syndrome were enrolled in a prospective international study conducted to obtain a better understanding of the clinical course of this unusual repolarization disorder. The mean patient age was 24 years, 64% were female, and 88% had family members with QT prolongation. During an average follow-up of 26 months per patient, four patients died suddenly (1.3% per year) and 27 patients had one or more syncopal episodes (8.6% per year). Multivariate analysis identified congenital deafness, history of syncope, female gender, and a documented episode of torsades de pointes or ventricular fibrillation as independent risk factors for postenrollment syncope or sudden death. Two types of treatment (left stellate ganglionectomy and beta-blocker therapy) were associated with a significant reduction in the occurrence of cardiac events during follow-up.     

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。     

Relatively benign clinical course in asymptomatic patients with brugada-type electrocardiogram without family history of sudden death

INTRODUCTION: The incidence of sudden death or ventricular fibrillation (VF) in asymptomatic Brugada syndrome patients with a family history of sudden death is reported to be very high. However, there are few reports on the prognosis of asymptomatic Brugada syndrome patients without a family history of sudden death. METHODS AND RESULTS: Eleven patients (all male; mean age 40.5 +/- 9.6 years, range 26 to 56) with asymptomatic Brugada-type ECG who had no family history of sudden death were evaluated. The degrees of ST segment elevation and conduction delay on signal-averaged ECG (SAECG) before and after pilsicainide were evaluated in all 11 patients. VF inducibility by ventricular electrical stimulation also was evaluated in 8 of 11 patients. Patients were followed for a period of 9 to 84 months (mean 42.5 +/- 21.6). The J point level was increased (V1: 0.19 +/- 0.09 mV to 0.36 +/- 0.23 mV; V2: 0.31 +/- 0.12 mV to 0.67 +/- 0.35 mV) by pilsicainide. Conduction delay was increased (total QRS: 112.2 +/- 6.3 msec to 131.7 +/- 6.3 msec; under 40 microV: 42.0 +/- 8.5 msec to 52.7 +/- 12.7 msec; last 40 msec: 17.4 +/- 5.9 microV to 10.4 +/- 6.1 microV) on SAECG by pilsicainide. VF was induced in only 1 of 8 patients. None of the patients had syncope or sudden death during a mean follow-up of 42.5 +/- 21.6 months. CONCLUSION: This study suggests that asymptomatic patients with Brugada-type ECG who have no family history of sudden death have a relatively benign clinical course.     

Long-term follow-up of primary prophylactic implantable cardioverter-defibrillator therapy in Brugada syndrome.

AIMS: To analyse the follow-up data of implantable cardioverter-defibrillator (ICD) therapy in Brugada syndrome (BS). METHODS AND RESULTS: We conducted a retrospective, single centre study of 47 patients (mean age: 44.5 +/- 15 years) with BS, who underwent primary prophylactic ICD implantation. All patients had baseline spontaneous (23 patients) or drug-induced (24 patients) coved type I ECG pattern. All patients were judged to be at high risk because of syncope (26 patients) and/or a positive family history of sudden death (26 patients). During a median follow-up of 47.5 months, seven patients had appropriate shocks. The presence of spontaneous type I ECG and non-sustained ventricular tachyarrhythmia in the ICD datalog suggested a trend towards shorter appropriate shock-free survival by Kaplan-Meier analysis (P = 0.037 and P = 0.012, respectively). Seventeen patients received inappropriate shocks (IS); eight patients for sinus tachycardia; six patients for new onset atrial arrhythmias; and five patients for noise oversensing. In multivariable Cox-regression analysis, new onset atrial fibrillation (AF) and less than 50 years of age were independent predictors of significantly shorter IS-free survival (P = 0.04 and P = 0.036, respectively). CONCLUSION: In high-risk patients with BS, primary prophylactic ICD therapy is an effective treatment. In this, young and otherwise healthy patient population, the IS rate is high.