原发性心电疾病患者的低血钾与室性心律失常

目的分析原发性心电疾病患者低血钾的原因,探讨其低血钾与室性心律失常的关系。方法2003年8月~2005年6月共确定原发性心电疾病患者12例,8例在晕厥后或晕厥发作间歇期存在低钾血症,其中特发性心室颤动(简称室颤)1例,长QT综合征(LQTS)3例,B rugada综合征(BS)3例,多形性室性心动过速(简称室速)1例。结果8例共检测到低血钾23次,血钾浓度为2.92±0.42(1.8~3.34)mmol/L。4例低血钾可能是室速/室颤的诱因,4例低血钾与室速/室颤的关系不太明确。补钾治疗对3例LQTS与1例多形性室速患者有效,使1例BS患者晕厥发作减少。结论低血钾是原发性心电疾病患者室性心律失常发生的危险因素,应常规监测血钾浓度并维持其正常。     

Long-term follow-up in patients with Brugada Syndrome in South China

ObjectiveTo evaluate the presence of Brugada electrocardiogram (ECG) pattern, clinical characteristics, treatment, and long‐term prognosis of Brugada syndrome in southern Chinese population.MethodsThis prospective study consisted of a consecutive series of patients with diagnostic coved type I Brugada ECG pattern at baseline between January 2007 and February 2020. Histories of symptoms including ventricular tachycardia (VT)/ventricular fibrillation (VF) episode, syncope, and family history of Brugada Syndrome (BrS) or unexplained sudden cardiac death were collected. Electrophysiological study and implantable cardioverter‐defibrillator (ICD) were performed. All patients included in this study were followed up in the outpatient department every 6 months after baseline evaluation. Occurrences of syncope, VF, and sudden death were independently analyzed by two cardiologists.Results45 (56.3%) patients were diagnosed with BrS. During a mean follow‐up of 7.9 ± 3.6 years, six patients had experienced documented VF/sudden cardiac death (SCD) or recurrent syncope. Two patients experienced episodes of syncope more than once. Two patients experienced onset of electrical storm with a total of 11 episodes of VF. There were 50% of these events occurring in fever status. One of patient with BrS died of SCD.ConclusionThere was a very low prevalence of Brugada syndrome in southern Chinese population. The risk of arrhythmic events was low in asymptomatic patients. ICD was high effective in preventing SCD without adverse device outcome in long‐term follow‐up. Fever can lead to predispose to malignant arrhythmia, and aggressive treatment of febrile state in Brugada syndrome was recommended.     

普罗帕酮试验引起Brugada综合征心室颤动(附一例报告)

探索普罗帕酮试验在Brugada综合征中的作用及安全性。患者有家族性夜间猝死史 ,近 1 9个月来反复发作性夜间晕厥5次 ,两次猝死未遂 ,一次记录到的心电图为心室颤动 (VF) ,其超声心动图、冠状动脉造影、左右心室造影正常 ,电生理检查心脏传导系统功能正常 ,右心室程序电刺激可诱发VF ,诊断不明。静脉用普罗帕酮 2mg/kg后 30min出现典型Brugada综合征心电图改变 ,并诱发VF。结论 :普罗帕酮可作为诊断Brugada综合征的工具 ,同时应警惕其致心律失常作用     

Primary aldosteronism with ventricular fibrillation: a case report

A 60-year-old female had sudden onset of syncope. The emergency service noticed that she suffered cardiopulmonary arrest (ventricular fibrillation: VF). After defibrillation in the ambulance, she was transported to our emergency department. Electrocardiography monitoring showed QT prolongation. Serum potassium level was extremely low at 1.8 mEq/l. Although potassium and lidocaine were administered, it was difficult to maintain appropriate electrolyte balance and prevent VF after admission, so temporary overdrive pacing was required. She was diagnosed as having primary aldosteronism after laboratory and imaging examinations. VF was otherwise uncontrollable so a cardioverter defibrillator was implanted on the 24th hospital day. Laparoscopic adrenalglandectomy was performed about 1 month later. After the surgery, serum potassium level remained at an appropriate level without medication. No severe neurological deficits were found at discharge from our hospital.     

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.     

Brugada syndrome occurring after autologous peripheral blood stem cell transplantation for acute myeloid leukemia

A 74-year-old male was found to be suffering from the Brugada syndrome after undergoing high dose chemotherapy followed by autologous peripheral blood stem cell transplantation (ABSCT) for acute myeloid leukemia. A specific ECG pattern of right bundle-branch block and a coved-type ST-segment elevation in leads V1 through V3, which is compatible with the Brugada syndrome, was unmasked by febrile neutropenia on the 8th day after ABSCT. He experienced syncope on the 11th day due to ventricular tachycardia, which was immediately improved with the administration of intravenous lidocaine. The Brugada syndorome should be considered in febrile patients if they have a episode of syncope or ECG change.     

The ajmaline challenge in Brugada syndrome: diagnostic impact, safety, and recommended protocol.

AIMS: The diagnostic ECG pattern in Brugada syndrome (BS) can transiently normalize and may be unmasked by sodium channel blockers such as ajmaline. Proarrhythmic effects of the drug have been well documented in the literature. A detailed protocol for the ajmaline challenge in Brugada syndrome has not yet been described. Therefore, we prospectively studied the risks of a standardized ajmaline test. METHODS AND RESULTS: During a period of 60 months, 158 patients underwent the ajmaline test in our institution. Ajmaline was given intravenously in fractions (10mg every two minutes) up to a target dose of 1mg/kg. In 37 patients (23%) the typical coved-type ECG pattern of BS was unmasked. During the test, symptomatic VT appeared in 2 patients (1.3%). In all other patients, the drug challenge did not induce VT if the target dose, QRS prolongation >30%, presence/appearance of the typical ECG, or the occurrence of premature ventricular ectopy were considered as end points of the test. A positive response to ajmaline was induced in 2 of 94 patients (2%) with a normal baseline ECG, who underwent evaluation solely for syncope of unknown origin. CONCLUSION: The ajmaline challenge using a protocol with fractionated drug administration is a safe method to diagnose BS. Because of the potential induction of VT, it should be performed under continuous medical surveillance with advanced life-support facilities. Due to the prognostic importance all patients with aborted sudden death or unexplained syncope without demonstrable structural heart disease and family members of affected individuals should presently undergo drug testing for unmasking BS.     

Acute and chronic management in patients with Brugada syndrome associated with electrical storm of ventricular fibrillation.

BACKGROUND: Some patients with Brugada syndrome experience an electrical storm of ventricular fibrillation (VF). OBJECTIVE: The purpose of this study was to investigate the clinical, laboratory, electrocardiographic, and electrophysiologic characteristics, acute and subsequent chronic treatment, and follow-up data of patients with Brugada syndrome associated with electrical storm of VF. METHODS: Sixty-seven patients with Brugada syndrome (65 men and 2 women, age 46 +/- 14 years) were divided into three groups: 7 patients with a history of electrical storm of VF (group I), 39 symptomatic patients with documented VF and/or syncope (group II), and 21 asymptomatic patients (group III). Electrical storm was defined as three or more episodes of VF per day recorded by the memory of an implantable cardioverter-defibrillator. RESULTS: No significant differences were observed among the three groups with regard to clinical (age at diagnosis, familial history of sudden cardiac death), laboratory (SCN5A mutation and serum potassium level), electrocardiographic and electrophysiologic characteristics, and follow-up duration after diagnosis. However, arrhythmic events during follow-up after diagnosis and number of arrhythmic events per patient were significantly higher in group I compared with groups II and III. Isoproterenol infusion (0.003 +/- 0.003 microg/kg/min for 24 +/- 13 days) completely suppressed electrical storm of VF in all five patients treated and was successfully replaced with oral medications, including denopamine, quinidine, isoproterenol, cilostazol, and bepridil alone or in combination. CONCLUSION: No specifically clinical, laboratory, electrocardiographic, and electrophysiologic characteristics were recognized in patients with Brugada syndrome associated with electrical storm of VF. Isoproterenol infusion was effective as an acute treatment in suppressing electrical storm of VF and was successfully replaced with chronic oral medications.     

Clinical significance of the dispersion of the activation--recovery interval and recovery time as markers for ventricular fibrillation susceptibility in patients with Brugada syndrome.

Brugada syndrome (BS) is associated with sudden cardiac death and the markers for ventricular fibrillation (VF) remain unclear, so the activation-recovery interval (ARI) dispersion and recovery time (RT) dispersion were investigated as possible markers in 20 subjects with BS (BS group) and 22 healthy individuals (H group). The 20 BS subjects were divided into 8 cases with documented VF (BS-VF group), 3 of which had recurrences, and 12 without (BS-N group). The corrected dispersion measurements from the standard 12-lead ECG of the QT interval (QTcd), ARI (ARIcd) and RT (RTcd) were compared among the groups. There were significant differences noted between the BS-VF and BS-N groups for the ARIcd and the RTcd, but not for the QTcd. Further, there were critical differences, 150 ms(1/2), observed for the ARIcd and RTcd, and these were associated with a prolongation of the maximum ARI or RT, shortening of the minimum ARI or RT, and prolongation only of the maximum QT for the QTcd. Susceptibility to VF may be predicted by the ARIcd or RTcd in BS.     

Amplification of spatial dispersion of repolarization underlies sudden cardiac death associated with catecholaminergic polymorphic VT, long QT, short QT and Brugada syndromes

This review examines the hypothesis that amplification of spatial dispersion of repolarization in the form of transmural dispersion of repolarization (TDR) underlies the development of life-threatening ventricular arrhythmias associated with inherited ion channelopathies including the long QT, short QT and Brugada syndromes as well as catecholaminergic polymorphic ventricular tachycardia. In the long QT syndrome, amplification of TDR is often secondary to preferential prolongation of the action potential duration (APD) of M cells, whereas in the Brugada syndrome, it is thought to be because of selective abbreviation of the APD of right ventricular epicardium. Preferential abbreviation of APD of either endocardium or epicardium appears to be responsible for amplification of TDR in the short QT syndrome. In catecholaminergic polymorphic VT, the reversal of the direction of activation of the ventricular wall is responsible for the increase in TDR. In conclusion, the long QT, short QT, Brugada and catecholaminergic VT syndromes are pathologies with very different phenotypes and aetiologies, but which share a common final pathway in causing sudden death.     

Long-QT-Syndrom und Brugada-Syndrom

Long QT syndrome and Brugada syndrome are potentially fatal inherited arrhythmogenic diseases. Thanks to the contribution of molecular genetics, the genetic bases, pathogenesis, and genotype-phenotype correlation of both diseases have been progressively unveiled and shown to have an extremely high degree of genetic heterogeneity. The clinical manifestation of the diseases is also highly variable. Symptomatic patients experience ventricular tachyarrhythmias which may lead to recurrent syncope and/or sudden cardiac death. In long QT syndrome patients with syncope, therapy with beta-blockers has proven effective. When, despite beta-blocker treatment, arrhythmia-related symptoms continue to occur, an implantable cardioverter defibrillator is indicated. Such a device should also be implanted in resuscitated patients. In symptomatic patients with Brugada syndrome, the implantable cardioverter defibrillator is the only life-saving option. In asymptomatic patients with a Brugada ECG pattern, risk stratification has become of utmost importance in order to discover which patients really need definitive treatment.     

Clinical characteristics and risk stratification in symptomatic and asymptomatic patients with brugada syndrome: multicenter study in Japan

Background: Neither the clinical characteristics nor risk stratification in Brugada syndrome have been clearly determined. We compared the clinical and ECG characteristics of symptomatic and asymptomatic patients with Brugada syndrome to identify new markers for high-risk patients.
Methods: A total of 188 consecutive individuals with Brugada syndrome (mean age 53 ± 14 years, 178 males) were enrolled in the Japan Idiopathic Ventricular Fibrillation Study (J-IVFS). Clinical and ECG characteristics were evaluated in three groups of patients: Ventricular fibrillation (VF) group: patients with documented VF (N = 33); Syncope (Sy) group: patients with syncope without documented VF (N = 57); and asymptomatic (As) group: subjects without symptoms (N = 98). Their prognostic parameters were evaluated over a 3-year follow-up period.
Results: (1) Clinical characteristics: incidence of past history of atrial fibrillation (AF) was significantly higher in the VF and Sy groups than in the AS group (P = 0.04). (2) On 12-lead ECG, r-J interval in lead V2 and QRS duration in lead V6 were longest in the VF group (P = 0.001, 0.002, respectively). (3) Clinical follow-up: during a mean follow-up period of 37 ± 16 months, incidences of cardiac events (sudden death and/or VF) were higher in the symptomatic (VF/Sy) groups than in the As group (P < 0.0001). The r-J interval in lead V2 ≥ 90 ms and QRS duration in lead V6 ≥ 90 ms were found to be possible predictors of recurrence of cardiac events in symptomatic patients.
Conclusions: Prolonged QRS duration in precordial leads was prominent in symptomatic patients. This ECG marker may be useful for distinguishing high- from low-risk patients with Brugada syndrome.     

Síncope e padrão de Brugada intermitente

Brugada syndrome is a rare syndrome, with an estimated prevalence in Europe of 1-5/10 000 population, whose initial clinical presentation can be sudden death. Although it has a characteristic electrocardiographic pattern, this can be intermittent. The authors present the case of a 32-year-old man, with no family history of syncope or sudden death, who went to the emergency department for syncope without prodromes. The initial electrocardiogram (ECG) in sinus rhythm documented an isolated and non-specific ST-segment elevation in V2. During further diagnostic studies, a repeat ECG revealed type 1 Brugada pattern. This pattern was later seen in a more marked form during a respiratory infection. The patient subsequently underwent electrophysiological study, followed by implantation of an implantable cardioverter-defibrillator (ICD), with an episode of ventricular fibrillation converted via ICD shock two months after implantation.     

先天性长QT综合征七例的临床研究

目的 对1例先天性长QT综合征（long QT syndrome,LQTS)患者（先证者）所在的家族进行普查，研究该家族的发病情况及临床和心电图特点，推测其相应的表现型和基因型。方法 按常规采集26例家庭成员的临床病史，进行体格检查，并采集同步12导联心电衅，测量QT间期和校正的QT间期，采用Schwartz提出的评分标准作为LQTS的诊断标准。结果 26例中有7例（28%）长QT综合征患者，6例可疑诊断。发生晕厥的诱因均为情绪激动或体力劳动，心电图表现为QT间期延长，在发病前后延长得更加明显，T波宽大有切迹，多可见U波，病情严重的患者心电图表现更加典型，该家族中患者的首次发病年龄较在，预后好，没有1例发生猝死或未成年夭折。结论 该家族中LQTS患者的临床和心电图表现符合LQTS1或者LQTS2。基因型有可能为KVLQT1或者HERG基因的突变。     

Neurally Mediated Syncope as a Cause of Syncope in Patients with Brugada Electrocardiogram

Neurally Mediated Syncope in Brugada Syndrome. Introduction: Patients with type 1 Brugada electrocardiogram (ECG) and an episode of syncope are diagnosed as symptomatic Brugada syndrome; however, all episodes of syncope may not be due to ventricular tachyarrhythmia. Methods and Results: Forty‐six patients with type 1 Brugada ECG (all males, 51 ± 13 years, 29 spontaneous, 17 Ic‐drug induced), 20 healthy control subjects (all males, 35 ± 11 years), and 15 patients with suspected neurally mediated syncope (NMS; 9 males, 54 ± 22 years) underwent the head‐up tilt (HUT) test. During the HUT test, 12‐lead ECGs were recorded in all patients, and the heart rate variability was investigated in some patients. Sixteen (35%) of 46 patients with Brugada ECG, 2 (10%) of 20 control subjects, and 10 (67%) of 15 patients with suspected NMS showed positive responses to the HUT test. Although no significant differences were observed in HUT‐positive rate among Brugada patients with documented VT (7/14; 50%), syncope (5/19; 26%) and asymptomatic patients (4/13; 31%), the HUT‐positive rate was significantly higher in patients with documented VT (50%) and those with VT or no symptoms (11/27, 41%) compared to that in control subjects (10%) (P < 0.05). Augmentation of ST‐segment amplitude (≥0.05 mV) in leads V1‐V3 was observed in 11 (69%) of 16 HUT‐positive patients with Brugada ECG during vasovagal responses, and was associated with augmentation of parasympathetic tone following sympathetic withdrawal. Conclusion: Thirty‐five percent of patients with Brugada ECG showed vasovagal responses during the HUT test, suggesting that some Brugada patients have impaired balance of autonomic nervous system, which may relate to their syncopal episodes. (J Cardiovasc Electrophysiol, Vol. 21, pp. 186‐192, February 2010)     

Electrocardiographic methods for diagnosis and risk stratification in the Brugada syndrome

The Brugada syndrome (BrS) is a malignant, genetically-determined, arrhythmic syndrome manifesting as syncope or sudden cardiac death (SCD) in individuals with structurally normal hearts. The diagnosis of the BrS is mainly based on the presence of a spontaneous or Na + channel blocker induced characteristic, electrocardiographic (ECG) pattern (type 1 or coved Brugada ECG pattern) typically seen in leads V1 and V2 recorded from the 4th to 2nd intercostal (i.c.) spaces. This pattern needs to be distinguished from similar ECG changes due to other causes (Brugada ECG phenocopies). This review focuses mainly on the ECG-based methods for diagnosis and arrhythmia risk assessment in the BrS. Presently, the main unresolved clinical problem is the identification of those patients at high risk of SCD who need implantable cardioverter-defibrillator (ICD), which is the only therapy with proven efficacy. Current guidelines recommend ICD implantation only in patients with spontaneous type 1 ECG pattern, and either history of aborted cardiac arrest or documented sustained VT (class I), or syncope of arrhythmic origin (class IIa) because they are at high risk of recurrent arrhythmic events (up to 10% or more annually for those with aborted cardiac arrest). The majority of BrS patients are asymptomatic when diagnosed and considered to have low risk (around 0.5% annually) and therefore not indicated for ICD. The majority of SCD victims in the BrS, however, had no symptoms prior to the fatal event and therefore were not protected with an ICD. While some ECG markers such as QRS fragmentation, infero-lateral early repolarisation, and abnormal late potentials on signal-averaged ECG are known to be linked to increased arrhythmic risk, they are not sufficiently sensitive or specific. Potential novel ECG-based strategies for risk stratification are discussed based on computerised methods for depolarisation and repolarisation analysis, a composite approach targeting several major components of ventricular arrhythmogenesis, and the collection of large digital ECG databases in genotyped BrS patients and their relatives.Abbreviations: AP, action potential; ARI, activation-recovery intervals; BrS, Brugada syndrome; ECG, electrocardiogram; EPS, electrophysiology study; ICD, implantable cardioverter-defibrillator; IHD, ischaemic heart disease; LBBB, left bundle branch block; MAP, monophasic action potential; MI, myocardial infarction; PCA, principal component analysis; RVOT, right ventricular outflow tract; SAECG, signal-averaged electrocardiogram; SCD, sudden cardiac death; SNP, single-nucleotide polymorphism; VF, ventricular fibrillation; VT, ventricular tachycardia; WT, wavelet transform     

A Case of Brugada Syndrome Presenting With Incessant Polymorphic Ventricular Tachycardia

Brugada syndrome, an inherited arrhythmogenic cardiac disease, manifests with ST‐segment changes in the right precordial leads, right bundle block pattern, and susceptibility to ventricular tachyarrhythmias and sudden death. The only established therapy for this disease is prevention of sudden death by implantation of a defibrillator. Herein we describe a case of a patient who presented with incessant ventricular tachycardia (VT) and syncope and who had a type 1 Brugada pattern on ECG. The patient was successfully treated with quinidine, after which the classically described type 2 and 3 patterns emerged. Copyright © 2009 Wiley Periodicals, Inc.     

Arrhythmogenic hereditary syndromes: Brugada Syndrome, long QT syndrome, short QT syndrome and CPVT

In approximately 10-20% of all sudden deaths no structural cardiac abnormalities can be identified. Important potential causes of sudden cardiac deaths in the absence of heart disease are primary electrical diseases such as Brugada syndrome, long QT syndrome (LQTS), short QT syndrome and catecholaminergic polymorphic ventricular tachyarrhythmias. Each of these cardiac channelopathies is charaterized by unique genetic and clinical features. The resting ECG and the ECG under exercise are pivotal for the diagnosis of ion channel diseases. Molecular genetic screening can reveal underlying mutations in a variable degree among the cardiac ion channel diseases in up to 70% (LQTS) and may identify individuals with incomplete penetration of the disease. In patients with primary electrical diseases specific clinical triggers for arrhythmic events such as syncope or sudden cardiac death have been identified including exercise, strenuous activity, auditory stimuli or increased vagal tone. The significance of programmed ventricular stimulation is at present unclear concerning risk stratification in patients with Brugada syndrome and short QT syndrome and of no significance in long QT syndrome and catecholaminergic polymorphic ventricular tachycardias. The success of medical therapy remains modest for prevention of sudden cardiac death and may necessitate the insertion of an implantable cardioverter. However, side effects with inappropriate therapies in this patient group with often young and active individuals have to be encountered. More insights into the arrhythmogenesis is critical for future development of effective medical treatment strategies.     

ECG Features that suggest a potentially life-threatening arrhythmia as the cause for syncope

Syncope is a risk factor for sudden cardiac death (SCD) in many conditions associated with structural heart disease as well as inherited heart disease. The ECG in patients with syncope should be examined carefully for signs of structural heart disease, such as myocardial infarction or cardiomyopathy; signs of conduction system disease, such as bundle branch block or atrioventricular block; and signs of primary electrical disease. Important forms of cardiomyopathy accompanied by ECG changes include hypertrophic cardiomyopathy (HCM), and arrhythmogenic right ventricular dysplasia (ARVD/C). Common ECG findings in HCM include left ventricular hypertrophy by voltage, repolarization abnormalities, QRS widening, pseudoinfarction patterns, and slurred QRS upstroke mimicking delta waves. Classical ECG findings of ARVD/C include T-wave inversions and epsilon waves in the right precordial leads (V₁–V₃). Important forms of primary electrical disease which may result in syncope include Wolff–Parkinson–White syndrome, long QT syndrome, and Brugada syndrome, which is characterized by coved ST-segments in the right precordial leads, associated with a history of syncope, ventricular arrhythmia, or sudden cardiac death in probands or family member. There are three Brugada ECG patterns; however, only type I (spontaneous or induced) is considered diagnostic. Recently, studies have suggested that patients with J-point elevation or early repolarization pattern on ECG are at elevated risk of SCD. The clinical significance of finding early repolarization in a patient with syncope is unknown and should be a subject of future research.     

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.