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     

The usefulness of the consensus clinical diagnostic criteria in Brugada syndrome

Background

Consensus statements were proposed for the diagnosis of Brugada syndrome (BS). The clinical diagnostic criteria were defined as documented ventricular fibrillation or ventricular tachycardia (VT), family history of sudden cardiac death at < 45 years, diagnostic ECGs of family members, inducibility of VT during electrophysiological study, syncope or nocturnal agonal respiration. The clinical validation of these criteria is still missing.Methods and results280 patients (41 ± 18 years, male: 168 pts) with diagnostic coved type I ECG were included. Consensus clinical diagnostic criteria were present in 244 (87%) patients (40 ± 18 y, 142 males). In 36 pts (13% of the 280 pts, 51 ± 12 years, 27 males) consensus clinical diagnostic criteria were not met. Nine patients (25%) presented with spontaneous type I ECG. Ten of the 36 patients (28%) had a history of atrial fibrillation and 13 (36%) had conduction disease on the baseline ECG. In 23 patients (64%) family screening was not performed. Two of the 36 patients had undocumented syncope during follow-up. Univariate analysis showed no significant difference in event free survival between patients with or without consensus clinical diagnostic criteria.

Conclusions

In a significant number of patients with diagnostic ECG pattern the current diagnostic criteria for BS are not met. These patients have frequently spontaneous type I ECG and clinical signs of Brugada syndrome as paroxysmal atrial fibrillation or conduction disturbances. Our results suggest that in patients with a diagnostic type I ECG pattern the current clinical consensus diagnostic criteria have limited added diagnostic value.     

Brugada综合征患者的心电图及临床特点分析

目的分析Brugada综合征患者的心电图及临床特点。方法对我院近5年诊断的8例Brugada综合征住院患者的心电图及临床情况进行长期随访观察。结果8例Brugada综合征患者均为男性，年龄平均（40±13）岁。心电图Ⅰ型Brugada波者3例，Ⅱ型4例，Ⅲ型1例；Brugada波具有多变性，提高肋间描记右胸导联心电图可显现Brugada波或使其更明显。8例中4例有猝死家族史，5例有晕厥史，3例在住院期间发生室速／室颤，随访期间2例猝死。结论心电图Brugada波（尤其Ⅰ型）是诊断Brugada综合征的必要条件，明确诊断Brugada综合征尚需联合其他几项临床指标；Brugada综合征患者猝死的风险高，消除晕厥或室速／室颤的诱因是预防的关键。     

四种代谢综合征诊断标准在贵阳市成人流行病学调查中的应用比较

目的 了解不同诊断标准下,贵阳市居民代谢综合征（MS）的患病率及四种诊断标准间的一致性,并探讨2009年国际多学会联合声明（JIS）标准的应用价值.方法 分析2009至2010年贵阳市糖尿病和代谢性疾病研究数据.分别用2004年中华医学会糖尿病学分会（CDS）、美国国家胆固醇教育计划成人治疗方案第三次报告（ATPⅢ）、2005年国际糖尿病联盟（IDF）及2009年JIS的MS标准,分析贵阳市社区居民总体及不同性别MS的患病率,并使用k值比较诊断结果的一致性.结果 贵阳市社区20岁及以上成人共1 512人（男性634人,女性878人）中,MS的标化患病率分别为21.7％（CDS标准）、29.1％（IDF标准）、40.3％（ATPⅢ标准）和43.6％ （JIS标准）.IDF标准下女性MS患病率高于男性（31.0％比26.2％,P＜0.01）,而JIS标准下男性患病率高于女性（45.1％比38.8％,P＜0.01）.ATPⅢ标准和JIS标准的诊断一致性最好（k=0.94,P＜0.05）,其次为IDF标准与ATPⅢ标准k值为0.78（P＜0.05）.结论 不同的诊断标准下,贵阳市成人MS的患病率均较高,JIS标准与ATPⅢ标准的诊断一致性最好.     

心室晚电位在Brugada综合征患者心律失常事件的预测价值

目的 探讨心室晚电位(LP)预测Brugada综合征患者发生心律失常事件的价值.方法 43例Brugada综合征患者分为有症状组(24例)和无症状组(19例).采用信号平均心电图(SAECG)检测所有患者的心室晚电位;动态随访(33.8±9.0)个月,观察研究人群中心律失常事件的发生情况. 结果 有症状组中有22例(91.7％)患者晚电位阳性,无症状组中有7例(36.8％)晚电位为阳性.LP阳性的Brugada综合征患者心律失常事件发生率为72.4％,LP阴性的Brugada综合征患者心律失常事件发生率为14.3％,LP预测心律失常事件发生的相对危险度(RR)为5.1,95％可信区间(CI)为[1.4,18.6](P=0.002). 结论 心室晚电位可以作为Brugada综合征患者发生心律失常事件的有效预测因子之一.     

肠易激综合征诊断标准评价

目的评价Manning标准、罗马Ⅰ标准和罗马Ⅱ标准诊断IBS的真实性，并对适合我国人群的IBS诊断标准进行探讨。方法采用自行设计的调查表对724名来自上海第二医科大学附属新华医院消化内科的门诊患者和健康体检中心的体检人员进行问卷调查、体格检查和选择性辅助检查。其中402例为IBS患者，134例为非溃疡性消化不良患者，90例为溃疡性结肠炎患者，98名为健康者。计算Manning标准、罗马Ⅰ标准、罗马Ⅱ标准诊断IBS的灵敏度和特异度及其95％CI、阳性预测值和阴性预测值、阳性似然比和阴性似然比。结果Manning标准、罗马Ⅰ标准和罗马Ⅱ标准的灵敏度和特异度分别为96．02％(95％CI：95．06％～100．0％)、77．36％(95％CI：75．31％～79．41％)、68．41％(95％CI：66．14％～70．68％)和47．51％(95％CI：44．78％～50．23％)、73．29％(95％CI：70．87％～75．70％)、83．23％(95％CI：81．19％～85．27％)；阳性预测值和阴性预测值分别为69．55％、78．33％、83．59％和90．53％、72．17％、67．85％，阳性似然比和阴性似然比分别为1．83、2．90、4．07和0．08、0．31、0．38。结论通过仔细采集病史的方法可作出IBS的正确诊断，罗马Ⅱ标准诊断IBS有较高的真实性。     

1 065例健康汉族人Brugada心电图征发生率的初步调查

目的通过调查健康体检者Brugada心电图征的发生率,获得中国人Brugada综合征的流行病学资料。方法对连续1069例参加年度健康体检的公务员进行病史、家庭史询问、体检、X线胸片和标准12导联心电图检查,按照欧洲心脏病学会推荐标准筛选出Brugada心电图征。结果排除4例器质性心脏病患者后,共1065例(男性805例,女性260例)入选,年龄18～83平均(38.58±15.26)岁。其中39例有黑史,36例有晕厥史,7例有猝死家族史。共8份心电图符合阳性标准,占总例数7.5‰。所有携带者均为男性,心电图均呈鞍型,其中符合Ⅱ型和Ⅲ型者各4例,1例(N7)有黑和晕厥史。结论Brugada心电图征在中国健康汉族人中并不少见,男性多见,其临床意义有待于进一步随访研究证实。     

Long-term prognosis of asymptomatic individuals with spontaneous or drug-induced type 1 electrocardiographic phenotype of Brugada syndrome

Background

Risk stratification of asymptomatic individuals with type 1 electrocardiogram (ECG) phenotype of Brugada syndrome (BS) still remains controversial. This study investigated the long-term prognosis of asymptomatic subjects with spontaneous or drug-induced type 1 ECG pattern of BS.

Methods and results

Data from 33 apparently healthy individuals (30 males; age, 43.6 ± 13.4 years) with spontaneous (n = 12) or drug-induced (n = 21) type 1 ECG pattern of BS were retrospectively analyzed. Thirteen subjects (39.4%) displayed a positive family history of BS and/or sudden cardiac death. Electrophysiologic study was performed in 16 subjects, and programed right ventricular stimulation induced polymorphic ventricular tachycardia in 9 (56.3%) of them. A cardioverter defibrillator was implanted in 6 cases. During a mean follow-up period of 5.3 ± 2.8 years, all subjects remained asymptomatic. None of them had syncope or cardioverter defibrillator discharges due to ventricular arrhythmias.

Conclusions

Asymptomatic individuals with spontaneous or drug-induced type 1 ECG phenotype of BS display a benign clinical course during long-term follow-up.     

Role of subcutaneous implantable loop recorder for the diagnosis of arrhythmias in Brugada syndrome: A United Kingdom single-center experience

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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.     

Right ventricular electrocardiographic leads for detection of Brugada syndrome in sudden unexplained death syndrome survivors and their relatives

BACKGROUND: Sudden unexplained death syndrome (SUDS) is a sudden death syndrome in previously healthy Southeast Asian young adults without any structural causes of death. Many SUDS survivors show electrocardiographic (ECG) evidence of RSR' and ST elevation in leads V1 to V3, which is similar to the ECG pattern in Brugada syndrome. However, in many cases transient normalization of the ECG does not make diagnosis with standard 12-lead ECG possible. HYPOTHESIS: To overcome this problem, we utilized the new right ventricular ECG leads to detect the Brugada syndrome in SUDS survivors. METHODS: The subject was a Thai male patient who presented with a SUDS-like syncopal attack. He had cardiac arrest due to idiopathic ventricular fibrillation. RESULTS: Post-resuscitation standard 12-lead ECG showed no diagnostic features of Brugada syndrome. However, ECG patterns of RSR' and ST elevations typical for Brugada syndrome could be detected at the higher intercostal space leads V1 to V3. We observed similar findings in 2 of the other 10 SUDS survivors and 4 of 23 healthy family members. CONCLUSIONS: Our data suggest that these new right ventricular leads ECG may be helpful in detecting Brugada syndrome in SUDS survivors and their relatives.     

两种诊断标准诊断原发性胆汁性肝硬化-自身免疫性肝炎重叠综合征的比较

原发性胆汁性肝硬化(PBC)-自身免疫性肝炎(AIH)重叠综合征是自身免疫性肝病中最常见的一种类型,但它并非是单纯的PBC与AIH在生物化学和病理等方面的叠加.在临床治疗中,如诊断过严,会存在治疗不足,不能有效阻止病变的发展;诊断过宽,则会存在治疗过度,增加药物对肝脏的损害,故正确地认识并诊断PBC-AIH重叠综合征非常必要[1].     

Why the current diagnostic criteria of Takotsubo syndrome are outmoded: A proposal for new criteria

Diagnosis of Takotsubo syndrome (TTS), the reversible, acute heart failure pathological entity, precipitated by stress, is based on the fulfillment of sets of criteria, developed by careful characterization of the precipitants, symptoms, results of imaging testing, clinical course, and follow-up of many patients presented with this affliction. As understanding of TTS, increase in its awareness, and the diversion in its presentation have evolved, the various proposed diagnostic criteria, naturally have started to appear outmoded. The author argues that the initially proposed Mayo Clinic criteria, the subsequently revised Mayo Clinic criteria, the Japanese Circulation Society guidelines, the Johns Hopkins criteria, and the Gothenburg criteria for the diagnosis of TTS have been outpaced by the rapidly accumulating clinical experience, and thus need to be replaced by more realistic sets of diagnostic rules. To this effect the author proposes a set of diagnostic criteria for TTS, which include 2 plausible, albeit speculative, notions, that of the milder forms or formes frustes of TTS, and the existence of “TTS comorbidity” in patients with various other illnesses, which either precipitate TTS, or are being brought about by TTS.     

应用普罗帕酮激发试验对Brugada综合征七例患者的诊断价值

目的　评价应用普罗帕酮激发试验诊断Brugada综合征 (BS)的有效性和特异性。方法7例怀疑BS者和 10例对照者 ,经体格检查、X线胸片和超声心动图初步排除器质性心脏病 ,BS组行冠状动脉以及左、右心室造影排除冠心病。做好电复律和心肺复苏准备后按分级推注方案行普罗帕酮激发试验 ,观察标准 12导联心电图并监护心律失常发作。结果　 7例疑似BS者均达到欧洲心脏病学会建议诊断标准。 3例晕厥患者被确诊为BS ,另 4例被诊断为BS样心电图改变。 3例晕厥患者中 2例植入ICD ,其中 1例随访 10个月有 14次夜间心室颤动发作。另 1例拒绝植入ICD ,随访 4个月后夜间猝死。另 5例随访 (8± 6 )个月无心律失常事件发生。除 1例试验中出现室性早搏、心室颤动外 ,余无不良反应出现。结论　普罗帕酮激发试验可有效、特异地用于诊断Brugada综合征。     

Symptom-based diagnostic criteria for irritable bowel syndrome: the more things change, the more they stay the same

Medical students are taught that 90% of all diagnoses are made through careful assessment of the patients' symptoms. Clinicians now rely heavily on techniques such as endoscopy or radiology before making a definitive diagnosis of organic disease. Most gastroenterologists would require endoscopic confirmation before labeling a patient as having peptic ulcer disease and would make a diagnosis of Crohn disease based on small bowel radiology or colonoscopy. However, the most common causes of symptoms of the gastrointestinal tract are functional. It is important that clinicians obtain a thorough history so that the disorder of the patient can be accurately defined.     

健康体检人群Brugada心电图征发生率的初步调查和随访

目的通过调查健康体检者Brugada心电图征的发生率,获得中国人Brugada心电图征的流行病学资料,并通过随访,了解Brugada心电图征人群的短期预后。方法收集了2003年6月至2005年1月我们医院查体中心健康体检人群45152份心电图资料,按照2002年欧洲心脏学会（ECS）专家共识报告提出的标准[1]筛选出Brugada心电图征。随访分两组:A组确诊为Brugada综合征患者（9人）,B组为无症状Brugada心电图阳性人群（73人）。结果共82人具有Brugada心电图征,占总数的1.82‰,其中男性73人,女性9人。Ⅰ型心电图征的18人,Ⅱ型38人,Ⅲ型26人,其中符合Brugada综合征共9人。随访结果:A组（24±12）个月的随访期内室性心动过速发生率为2/9,B组（27±11）个月的随访期内室性心动过速发生率为3%（2/73）,两组相比具有显著性差异（P<0.05）,两组人群均无人猝死。结论Brugada心电图征在广东省健康成年人发生率为1.82‰,男女比8∶1。在随访中发现,Brugada综合征患者要比无症状Brugada心电图征人群预后差,容易发生致命性的室性心律失常。     

New electrocardiographic leads and the procainamide test for the detection of the Brugada sign in sudden unexplained death syndrome survivors and their relatives.

AIMS: Sudden unexplained death syndrome occurs in previously healthy South-east Asian young adults without any structural cause of death. The common electrocardiographic (ECG) change in sudden unexplained death syndrome survivors is right bundle branch block and ST elevations in leads V(1) to V(3), which are similar to the ECG pattern in the Brugada syndrome (Brugada sign). It is difficult to diagnose the Brugada sign with the 12-lead ECG in sudden unexplained death syndrome survivors and their family members because the ECG could be transiently normalized. We proposed using the higher intercostal space V(1) to V(3) lead ECG, together with procainamide to detect the Brugada sign. METHODS AND RESULTS: Among 20 ventricular fibrillation cardiac arrest patients, 13 sudden unexplained death syndrome survivors and their relatives (n=88) were studied using the single standard 12-lead ECG and the new six higher intercostal space V(1) to V(3) lead ECG (-V(1) to -V(3) and -2V(1) to -2V(3)). Ten sudden unexplained death syndrome survivors and relatives (n=48) who had a normalized ECG were also infused with procainamide (10 mg x kg(-1)i.v.) to unmask the Brugada sign and both ECG methods were recorded. Forty healthy individuals and 13 spouses served as the control group. Prior to the procainamide infusion, the Brugada sign could be detected in nine sudden unexplained death syndrome survivors (69.2%) and three (3.4%) relatives with the standard ECG and in 12 (92.3%) and nine (10.2%) with the new six-lead ECG. After the procainamide infusion, the Brugada sign could be demonstrated in seven sudden unexplained death syndrome survivors (70%) and seven (14.6%) relatives with the standard ECG and in nine (90%) (P=0.26) and 23 (47.9%) (P=0.0004) with the new six-lead ECG, respectively. All the controls were negative for the Brugada sign. CONCLUSIONS: Our data suggest that the new higher intercostal space lead ECG, with or without the procainamide test is helpful in detecting the Brugada sign in sudden unexplained death syndrome survivors and their relatives.