儿茶酚胺敏感性多形性室性心动过速的诊治进展

儿茶酚胺敏感性多形性室性心动过速(catecholaminergic polymorphic ventricular tachycardia,CPVT)是一种少见的遗传性离子通道病,主要影响儿童和年轻人,以多态和双向性室性心动过速为特征,症状包括头晕、心悸,严重者可出现晕厥发作,甚至心跳骤停、心源性猝死。临床已证实青少...     

普罗帕酮联合普萘洛尔治疗儿茶酚胺敏感性多形性室性心动过速1 例报告并文献复习

目的探讨儿茶酚胺敏感性多形性室性心动过速(CPVT)的诊断和治疗。方法回顾分析1例CPVT患儿的临床资料,并复习相关文献。结果男性患儿,5岁2个月,间歇性心前区不适8月余,运动时晕厥发作2次。24小时动态心电图以及运动试验,窦性心率110 次/min即出现室性心律失常,随着心率增加室性心律失常频数增加,可见双向及多形性室速。基因检测示RYR2基因变异c.6886GA(p.E2296K)。予大剂量普萘洛尔治疗,症状未缓解且心电检查无明显改善,后加用普罗帕酮获得改善。随访1年,患儿未再有运动胸闷不适,动态心电图及运动试验显示无室性心律失常。结论 CPVT以运动或情绪激动诱发双向型和/或多形性室性心动过速为主要临床特征,晕厥、猝死为主要临床表现,普罗帕酮可能可以替代或联合用药治疗CPVT。     

TECRL基因与儿茶酚胺敏感性室性心动过速

儿茶酚胺敏感性室性心动过速(catecholaminergic polymorphic ventricular tachycardia,CPVT)是由情绪应激或运动诱发的一种高致死性遗传性心律失常,可突发快速多态性室性心动过速和室颤,并可能导致晕厥或猝死,预后不良。基因检测是确诊该疾病的方法。目前已发现该病与RyR2、...     

运动平板试验诱发的儿童儿茶酚胺敏感性多形性室性心动过速1例报告

<正>1病例资料患儿男,年龄6岁8个月,因"发作性晕厥4个月"于2014年11月21日至28日在安徽省儿童医院心内科住院治疗。入院前有晕厥发作2次,均为运动后出现,发作时有意识丧失、倒地,持续约10 s后可自行缓解,晕厥发作前无抽搐、呕吐,非饥饿状态。患儿入院前4个月爬坡时出现首次晕厥发作,意识丧失后倒地,头部皮肤磕破,持续10 s左右后自行恢复,未特殊处理。入院前2 d,发生第2次     

CASQ2基因变异致儿童儿茶酚胺敏感性多形性室性心动过速的临床及遗传学特点分析

目的:总结 CASQ2基因变异致儿童儿茶酚胺敏感性多形性室性心动过速(CPVT)的临床特征及遗传学特点。 方法:回顾性分析2017年1月至2018年11月在首都医科大学附属北京儿童医院诊治的8例(男女各4例) CASQ2基因变异阳性CPVT患儿的临床资料,基因检测采用靶向高通量二代测序...     

小儿室性心动过速19例分析

小儿室速是儿科急重症。本文总结我院1985～1995年收治的19例,着重分析其病因、临床表现、心     

28例小儿室性心动过速的诊治体会

室性心动过速(简称室速)在小儿虽然相对少见,偶可见于健康儿童,但大多发生于有严重器质性心脏病患儿,常引起阿斯氏综合征的发作,如处理不当甚至可发生猝死。本文总结近十年收治28例的诊治体会如下。     

特发性室性心动过速36例

目的探讨儿童特发性室性心动过速（IVT）的临床特点及诊治方法。方法对2003年11月～2006年12月在北京儿童医院住院确诊为IVT的36例患儿的临床表现、实验室检查及治疗方法进行回顾性分析,并进行统计学处理。结果IVT以年长儿童多见,平均年龄7岁8个月,男女比例为1.11,根据起源部位分为左室间隔部室速（ILVT）和右室流出道室速（RVOTVT）,心电图表现以右束支阻滞形态为主23例（63.8%）,为左室型室速;呈左束支阻滞图形13例（36%）,为右室型室速。左室型23例中胸闷、心悸15例（41.6%）,晕厥发作2例（5.5%）;右室型13例中心悸3例（8.3%）,乏力1例（2.7%）,余9例（25%）无明显临床症状。左室型室速临床表现较右室型室速重,二者比较差异有显著意义（P〈0.01）。左室型室速6例（15次）用维拉帕米有效,10例（17次）使用普罗帕酮有效,1例（1次）使用胺碘酮有效,2例（3次）行体外同步直流电击复律有效。右室型1例（1次）使用艾司洛尔有效,3例（2次）使用普罗帕酮有效。23例（63%）行射频消融治疗,室速消失。行射频消融治疗病例较长期服药的复发率低,二者比较差异有显著意义（P〈0.01）。结论IVT临床表现大多较轻,但亦有心力衰竭和晕厥发作,准确分型有利于准确用药,射频消融为较好治疗方法。     

Inherited catecholaminergic polymorphic ventricular tachycardia due to RYR2 mutation

We report the case of an 11‐year‐old boy who was diagnosed with catecholaminergic polymorphic ventricular tachycardia (CPVT). The patient had a medical history of three episodes of syncope. The last episode was cardiac arrest while swimming. After resuscitation using automated external defibrillator, he was placed under cerebral hypothermia, examined for long QT syndrome, and underwent insertion of implantable cardioverter defibrillator. He was subsequently discharged from hospital without any adverse sequelae. The patient was diagnosed with CPVT after detection of ryanodine receptor 2 mutation. His father also carried the same mutation, although he did not have any symptoms nor did he have a history of syncope. We propose that CPVT should be included in the differential diagnosis in children with recurrent episodes of syncope.     

Catecholaminergic polymorphic ventricular tachycardia in a child: a case report

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare arrythmogenic disease characterized by exercise--or stress--induced ventricular tachyarrythmias, syncope, or sudden death, usually in the pediatric age group. Familial occurrence has been noted in about 30% of cases. Inheritance may be autosomal dominant or recessive, usually with high penetrance. The causative genes have been mapped to chromosome 1. Mutations of the cardiac ryanodine receptor gene (RyR2) have been identified in autosomal dominant pedigrees, while calsequestrin gene (CASQ2) mutations are seen in recessive cases. CONCLUSION: Due to its potential lethal outcome, exclusion or confirmation of catecholaminergic polymorphic ventricular tachycardia in children with physical and emotional syncope is mandatory. We report a case of catecholaminergic polymorphic ventricular tachycardia in a three-year-old child only diagnosed by genetic mapping.     

Histopathologic aspects of endomyocardial biopsy in pediatric patients with idiopathic ventricular tachycardia

PURPOSE: The present study aimed to investigate the clinicopathologic findings and histopathologic characteristics of endomyocardial biopsy in pediatric patients with idiopathic ventricular tachycardia. METHODS: Histopathological findings of endomyocardial biopsy from 17 patients aged 7-15 years with idiopathic ventricular tachycardia (VT) but no organic heart disease were examined. Patients considered to have cardiomyopathy of the dilated, hypertrophic or specific form or arrhythmogenic right ventricular cardiomyopathy were excluded from this study. RESULTS: Advanced histopathologic findings, including myocyte hypertrophy, degeneration, interstitial fibrosis and disarrangement of muscle bundles, were disclosed in three cases (17.6%). One of these cases exhibited sustained VT with left bundle branch block configuration and showed increased frequency of VT during exercise testing. The remaining two cases had non-sustained VT with multifocal origin and had syncope episodes. Another 14 cases showed mild or no significant findings in the biopsy. CONCLUSIONS: These results indicate that advanced histopathology in endomyocardial biopsy is occasionally disclosed in cases of idiopathic VT, especially those of exercise-related VT or multifocal VT, and that these patients may be considered as having heart muscle disease.     

Idiopathic sustained left ventricular tachycardia in pediatric patients

BACKGROUND: Idiopathic sustained ventricular tachycardia originating from the left ventricle (ILVT) has been an indication for catheter ablation. The present study evaluated the clinical features, long-term prognosis and indications for treatment in pediatric patients with ILVT. METHODS: The subjects of the present study were eight patients (four males and four females) with a mean age at onset of 11.0 years (range 3-15 years). The mean follow-up period was 7.7 years (range 2.1-11.3 years). RESULTS: In electrophysiologic studies, intravenously administered verapamil was effective for the termination of tachycardia in all six patients who received this treatment and for the prevention of tachycardia in four of five patients. Oral administration of verapamil was effective in five of seven patients. Propranolol or flecainide was added to the treatment protocol for two patients who did not respond to verapamil alone. Tachycardia disappeared without drugs in four patients during the follow-up period and became non-sustained in another patient. Two of three patients with persistent tachycardia underwent catheter ablation. Pharmacologic treatment was very effective for ILVT among these patients. CONCLUSIONS: Pharmacologic therapy, such as with verapamil, is still the treatment of choice for ILVT because of a good long-term prognosis and potential risks and complications by manipulation of catheter ablation.     

室性心动过速的诊断治疗

目的了解小儿室性心动过速（VT）的诊治进展。方法分析临床资料，复习有关文献。结果33例小儿VT，有原发疾病者27例，包括心脏病22例，心外疾病5例，8／33例不需抗心律失常药物治疗。死亡10例均与严重基础疾病相关。结论对静脉用胺碘酮治疗致命的快速心律失常的效果和安全性重新作了很好的评价。射频消融是当今治疗顽固快速心律失常最大的进展。     

儿童特发性室性心动过速经改进的导管标测消融方法治疗体会

总结应用改进的标测方法和新技术治疗儿童特发性室性心动过速的经验。左室特发性室性心动过速(ILVT)共7例 ,其中ILVT时体表12导联心电图表现为完全右束支阻滞伴左前分支阻滞6例和伴左后分支阻滞1例 ;右室特发性室性心动过速(IRVT)3例 ,其中右室流出道2例和右室流入道1例。年龄8.4±3.2岁 ,体重28.5±13.4kg。①在窦性心律下浦肯野电位法标测ILVT消融靶点 ;②应用二根分别放置在右室流出道和右室流入道电极 ,采用“蛙跳”方法标测较早的心室激动点 ,然后用射频导管在该点附近标测IRVT时最早心室激动点 ;③应用Carto系统标测ILVT。结果显示 ,10例均成功消融 ,放电和透视时间分别为7.0±3.8次和20±8.4分钟 ;应用Carto系统标测2例ILVT ,1例ILVT在靶点附近标测过程中易出现室性早搏 ,难与ILVT鉴别而形成错误的电解剖图 ,导致消融失败。提示改进的标测方法可减少放电次数 ,缩短X线透视时间 ;Carto系统在ILVT(VT时体表12导联心电图表现为完全右束支阻滞伴左前分支阻滞或左后分支阻滞)标测过程中易受室性早搏干扰 ,形成错误的电解剖图而消融失败 ,且费用昂贵和手术时间长     

Long-term efficacy and safety of atenolol for supraventricular tachycardia in children

Propranolol, a first-generation nonselective β-adrenoceptor blocking agent, is commonly used to treat pediatric arrhythmias. Atenolol, relatively longacting, cardioselective β-adrenoceptor blocking agent, has been successfully used in adults with supraventricular tachycardia (SVT). There is only one report on the use of atenolol in children with SVT, and our report is on the first long-term prospective study to evaluate the use of atenolol in children. A group of 22 children <18 years of age with clinical SVT were enrolled in the study. The tachycardia was documented on electrocardiograms in each case and was confirmed by electrophysiologic studies in some. Once-a-day oral atenolol was started as a monotherapy. Of the 22 children with various types of SVT, 13 (59%) were well controlled on long-term oral atenolol therapy. The effective dose of atenolol ranged between 0.3 and 1.3 mg/kg/day (median effective dose 0.7 mg/kg/day). Five children had some adverse effects. However, none in the successful group of 13 patients required drug discontinuation because of such effects. Once-a-day oral atenolol as a monotherapy is effective and relatively safe for long-term management of SVT during childhood. It is an attractive alternative β-adrenoceptor blocking agent for the management of pediatric arrhythmias.     

射频消融分支电位治疗儿童左后分支性室性心动过速

目的 简化经导管标测和消融儿童左后分支性室性心动过速的方法.方法 窦性心律下,在后间隔(冠状静脉窦口下缘1～2 cm)的区域内,标测分支电位,其表现为心室波之前的双向波,两者之间存在明确的等电位线;当消融导管标测该电位较希氏柬电位晚20ms以上时试放电;放电前双角度(LAO 45°和30°)观察消融导管的位置,确定不在希氏束处;心电图出现左后分支阻滞,说明消融有效.消融术后心电监测24～48 h,注意室速终止后复极变化;服阿司匹林2～3 mg/kg 3个,月,停服抗心律失常药物,术后1d复查体表心电图、胸片、超声心动图,出院后1个月、3个月各随访一次,此后每半年门诊定期复查或电话随访.结果 15例患儿成功消融,术后心电图均出现左后分支阻滞图形;随访3～12个月,所有出现左后分支阻滞的患儿均无复发.其中1例术中靶点位置好的患儿,试放电后,心电图无改变,仍出现室速,后重新标测,试放电后出现左后分支阻滞,巩固90 s,成功消融,随访6个月,无复发.结论 射频消融分支电位治疗儿童左后分支性室性心动过速,简化了标测,减低了手术的难度,消融终点更为可靠.