心外膜基质消融治疗Brugada综合征患者1例

1病例资料患者,男,52岁,发生心源性猝死,当地医院抢救成功,体表心电图显示I型Brugada波,动态心电图捕捉到心室颤动,确诊为Brugada综合征。转我院行ICD植入术,术后1个月内又反复出现心室颤动及ICD放电,给予药物治疗效果差,遂在我院行射频消融术。术前心电图提示为典型的I型Brugada波。24h动态心电图无室性期前收缩,无室性心动过速。     

一个Brugada综合征合并先天性长QT综合征的家系及临床研究

目的 1例Brugada综合征合并先天性长QT综合征的家系的临床研究。方法 对夜间反复发作性晕厥的先证者进行冠状动脉，左、右心室造影和电生理检查及药物试验(异丙肾上腺素和普罗帕酮)；对其家族成员进行病史询问、体格检查、超声心动图、动态心电图和药物试验，同时记录右侧胸前导联(V1～V3)上两个肋间的心电图。结果 家族中有两例猝死，均发生在睡眠中。家族成员未被发现器质性心脏病。先证者心电图表现为右侧胸前和下壁导联ST段抬高，住院期再次发生夜间晕厥记录到心电图为多形室性心动过速(室速)。冠状动脉及左、右心室造影正常，电生理检查诱发多形室速。异丙肾上腺素试验时ST段正常，QTc间期明显延长；普罗帕酮试验阳性。另两例家族成员，右侧胸前导联上一或二肋间心电图呈典型Brugada综合征改变，异丙肾上腺素试验QTc间期亦明显延长，1例普罗帕酮试验阳性。结论 结果表明可能是由于一种新的钠通道基因(SCN5A)突变类型同时引起Brugada综合征和先天性长QT综合征。     

药物激发试验在Brugada综合征中的应用

目的探讨药物激发试验在隐匿性Brugada综合征中的应用。方法将高度怀疑为Brugada综合征的9例患者分成两组,分别用缓脉灵(ajmaline)1mg/kg和氟卡尼(flecainide)2mg/kg进行激发试验。以2002年欧洲心脏病协会心律失常组提出的阳性标准为判断标准。结果两组中各有1例患者诱发呈阳性反应。结论药物激发试验有助于隐匿性Brugada综合征的发现。     

Brugada综合征合并先天性长QT综合征一例

目的报告1例反复发作晕厥伴胸前导联J—ST—T显著抬高患者的临床过程。方法1例15岁男孩反复于夜间卧床休息时发生晕厥，对该患者及其父母进行病史询问、体格检查、心电图及超声心动图检查，并行普罗帕酮激发试验。对患者进行冠状动脉，左、右心室造影和心内电生理检查。结果患者及其父母无器质性心脏病依据，无阳性猝死家族史。患者直立倾斜试验阴性，冠状动脉和左、右心室造影正常，心内电生理检查未发现异常，未诱发室性心律失常。患者基础心电图胸前导联J-ST—T显著抬高，晕厥后窦性心动过速时J—ST—T降低伴QTc延长。静脉注射普罗帕酮70mg后胸前导联J—ST与T波第二峰进一步抬高。患者母亲基础心电图ST—T类似于LQT3，但QTc正常。患者父母在静脉注射普罗帕酮70mg后胸前导联ST—T均进一步抬高。结论该患者心电图不同于已报道的Brugada综合征合并LQT3，可能为新的SCN5A基因突变导致的一种新的表型。     

Drug-induced pericarditis mimicking Brugada syndrome.

Brugada syndrome (BS) is associated with sudden cardiac death in patients with a structurally normal heart. The ECG pattern of BS has also been described in patients with myocardial abnormalities. Cardiac hypersensitivity and myopericarditis have been reported during long-term treatment with mesalazine. We report the case of a man, treated with mesalazine for Crohn's disease who developed drug-induced pericarditis. The ECG showed a coved ST-segment elevation in the right precordial leads V1-V3, a pattern mimicking BS. The ECG normalized in a few days after mesalazine withdrawal and the follow-up was uneventful. The ECG remained normal. Two ajmaline tests were both negative and ruled out the diagnosis of BS. This observation illustrates that a coved ST-segment elevation in the right precordial leads should not be, systematically, regarded as a marker of a specific syndrome, but may also reflect a common electrical manifestation of abnormalities in the right ventricle or pericardium.     

An atypical case of Brugada syndrome

Polymorphic ventricular tachycardia and ventricular fibrillation are the most common arrhythmias in Brugada syndrome causing syncope or sudden death. Sustained monomorphic ventricular tachycardias are rare in this context. We report of a patient with syncopal episodes due to episodes of sustained ventricular tachycardia, where a Type-I Brugada pattern was revealed after pharmacological provocation with procainamide.     

Computer simulation of atypical Brugada syndrome

Recently, the so-called atypical Brugada syndrome (BS) has been reported in few cases in literature. The typical BS is characterized by ST-segment elevation in the right precordial leads, whereas atypical forms of the disease are distinguished by electrocardiogram abnormalities of the J wave, and ST-segment elevation appeared in the inferior and lateral leads. In this work, we report a simulation of atypical BS based on a 3-dimensional whole-heart model. By setting the action potentials of Brugada model cells in different epicardial regions, we calculated 12-lead electrocardiogram and body surface potentials that are in good agreement with clinical data. Applying additional electrical stimuli, we obtained the induction of ventricular fibrillation in both typical and atypical BS forms. The calculated results confirm possibility of similar electrophysiological basis in both cases and suggest that BS can also be observed in inferior and lateral precordial leads.     

Cycle length-dependent repolarization changes during atrial fibrillation in the Brugada syndrome

This is a case report of a patient with Brugada syndrome who developed paroxysmal atrial fibrillation. During the episode, beat-to-beat changes in ventricular repolarization were observed. These changes were a paradoxical ST-segment alteration after a short-coupled ventricular beat. These findings, not reported before, may be helpful for the diagnosis of this syndrome.     

新胸导联在诊断Brugada综合征中的应用

目的 评价自行设计的新胸导联在Brugada综合征诊断中的应用价值。方法 4例Brugada综合征先证者和9例家族成员接受新胸导联和普罗帕酮药物试验。11例无晕厥史和无猝死家族史的房室结折返性室上性心动过速患者作为新胸导联检查对照组。自行设计的心电图新胸导联包括A-G(7)列、0-5(6)行,共42个导联,记录方法同标准胸导联。普罗帕酮试验:普罗帕酮70 mg(体重>70kg者用105 mg)加入生理盐水10 ml于5 min内静脉注射,用药前、后记录标准12导联心电图;用药后标准V1-V3导联J点或ST段抬高超过2 mm(或ST段抬高由BrugadaⅡ或Ⅲ型转变成Ⅰ型),称为药物试验阳性。结果 先证者1～3均有晕厥史,先证者1和3有家族猝死史,先证者2和3晕厥发作时记录到心室颤动,先证者3猝死;除先证者4标准胸导联心电图呈Brugada典型下斜型改变外,先证者1-3标准胸导联心电图不能明确诊断为Brugada综合征。新胸导联发现先证者1-3呈典型Brugada综合征心电图改变,位于标准胸导联以外区域。新胸导联同时发现,5例家族成员在标准胸导联以外区域有典型的Brugada心电图改变。新胸导联阳性者亦被普罗帕酮试验证实。对照组11例新胸导联检查均为阴性。结论 新胸导联有助于发现Brugada综合征典型心电图改变位于标准V1-V3导联以外的病例,且应用安全,方法较简单     

Type 1 electrocardiographic burden is increased in symptomatic patients with Brugada syndrome

Background

Spontaneous type 1 electrocardiographic (ECG) is a risk factor for arrhythmic events in Brugada patients but the importance of the proportion of time with a type 1 ECG is unknown.

Patients and Methods

Thirty-four Brugada patients (15 symptomatic) underwent a 24-hour 12-lead ECG recording. One-minute averaged waveforms displaying ST-segment elevation above 200 μV, with descending ST-segment and negative T-wave polarity on leads V₁-V₃ were considered as type 1 Brugada ECG. The burden was defined as the percentage of type 1 Brugada waveforms.

Results

Type 1 ECG on lead V2 was more frequent in symptomatic patients (median 80.6% [15.7-96.7] vs 12.4% [0.0-69.7], P = .05). Patients with a permanent type 1 pattern on lead V₂ were more likely to be symptomatic (5/6) than patients without type 1 ECG during a 24-hour period (2/9) (P < .05).

Conclusion

Type 1 pattern is more prevalent across a 24-hour period in symptomatic Brugada patients.     

Brugada syndrome in pure black Africans

Aims: Although all races are concerned with the Brugada syndrome, no case has ever been reported among black Africans. We describe five different cases in this specific group of populations.
Methods and Results: In all patients, Brugada syndrome was identified after detailed noninvasive and invasive evaluations. Sex ratio was four males for one female. Convulsive syncope was noticed in 1 patient with a family history of sudden death. Diagnostic coved-type pattern was observed spontaneously in the normal position of right precordial leads in 3 patients and in a higher position of leads in 3 patients. Sixty percent had first-degree atrioventricular block. An ajmaline test was performed in 4 patients and was positive either in normal position of leads or in superior position in all of them. Sustained ventricular tachycardia (VT) or ventricular fibrillation (VF) was inducible during programmed ventricular stimulation in 3 patients. Right ventricular cineangiography found localized apical hypokinesia with preserved systolic function in 1 patient. Automatic cardioverter defibrillator was implanted in 2 patients. SCN5A was not found in any of the patients.
Conclusion: These observations demonstrate that Brugada syndrome is also present in black African populations, and increasingly reported cases of apparent sudden death in the sub-Saharan part of the world need to rule out cardiac electrical disturbance such as Brugada syndrome.     

Manifestation of Brugada syndrome after pacemaker implantation in a patient with sick sinus syndrome

A 49-year-old woman experienced syncope 10 months after DDD pacemaker implantation for sick sinus syndrome. ECG revealed abnormal ST elevation in leads V1 to V3 during a paced rhythm. Multifocal premature ventricular contractions followed by ventricular fibrillation were documented. Saddleback-type ST elevation was confirmed after a mode change to AAI. The diagnosis of Brugada syndrome was made, and the DDD pacemaker was upgraded to an implantable cardioverter defibrillator. Brugada syndrome can be easily overlooked if the classic ECG findings are not initially noted but may be observed even during pacing therapy.     

Clinical predictors of atrial fibrillation in Brugada syndrome.

AIMS: Atrial arrhythmias have been reported in patients with Brugada syndrome. The aim of this study was to evaluate clinical predictors of atrial fibrillation (AF) in Brugada syndrome. METHODS AND RESULTS: Patients diagnosed with Brugada ECG pattern were enrolled in the study. Type 1, 2, and 3 Brugada ECG pattern was found in 28, 56, and 31 patients, respectively. A total of 85 healthy age and gender-matched subjects were selected as a control group. Mean age, maximum P-wave duration (P(max)), P-wave dispersion (P(disp)), and left atrial dimension were not significantly different between patients and controls. There were no differences between P(max), P(disp), and left atrial dimension of the type 1, 2, and 3 Brugada patients. Spontaneous paroxysmal AF was detected in 15 of 28 type 1 Brugada patients (53%) and none of the type 2 and 3 Brugada patients. All 15 patients with AF had at least one episode of paroxysmal AF and none of the patients showed persistent or chronic AF. All 15 patients who had paroxysmal AF had previous life threatening cardiac events. In contrast, paroxysmal AF did not occur in type 1 Brugada patients without previous life threatening cardiac events. In multiple regression analysis, only the occurrence of previous life threatening cardiac events was a risk factor for paroxysmal AF (P = 0.0001). CONCLUSION: It is concluded that the most important predictor of AF in Brugada syndrome is the occurrence of previous life threatening cardiac events.     

Electrical storm in Brugada syndrome successfully treated using isoprenaline.

A case of an electrical storm occurring in a patient implanted with a cardioverter-defibrillator for Brugada syndrome is reported. Recurrent ventricular fibrillation was initiated by short-coupled isolated monomorphic ventricular premature beats probably originating from the right ventricular outflow tract, associated with a manifest electrocardiographic pattern of Brugada syndrome. Infusion of atropine accelerated the heart rate but did not prevent ventricular fibrillation, however, low doses of isoprenaline quickly obviated any recurrence of ventricular fibrillation. This was associated with the disappearance of the short-coupled premature beats together with a normalization of the electrocardiographic pattern. Possible mechanisms are discussed according to the accepted pathophysiological hypothesis.     

Brugada syndrome and its relevance in the perioperative period

Brugada syndrome is an autosomal dominant genetic disorder associated with an increased risk of sudden cardiac death, as well as ventricular tachyarrhythmias. The defective cardiac sodium channels result in usual electrocardiographic findings of a coved-type ST elevation in precordial leads V1 to V3. The majority of patients have uncomplicated courses with anesthesia, surgery, and invasive procedures. However there is risk of worsening ST elevation and ventricular arrhythmias due to perioperative medications, surgical insult, electrolyte abnormalities, fever, autonomic nervous system tone, as well as other perturbations. Given the increasing numbers of patients with inherited conduction disorders presenting for non-cardiac surgery that are at risk of sudden cardiac death, safe anesthetic management depends upon a detailed knowledge of these conditions.