致心律失常性右室心肌病一例报告

1　病例资料女, 43岁。因活动后心悸、气短 9个月,加重 2个月第 1次入院。查体:体温 36 4℃,脉搏 90 /min,呼吸 20 /min,血压100 /60mmHg。精神差,被迫高枕卧位。全身高度水肿,颈静脉怒张,肝 颈静脉回流征阳性。双肺听诊未闻及干湿性啰音慕缦蛄讲嗬┐?心率 90 /min,律齐,心音低钝,肺动脉瓣听诊区第二心音分裂,二尖瓣、三尖瓣及肺动脉听诊区可闻及 4 /6级收缩期杂音,A2 相似文献   

致心律失常性右心室心肌病一例报告

男,21岁。因阵发性心悸、胸闷3年,加重12天入院。病人3年前因剧烈活动出现心悸、胸闷,无心前区疼痛、晕厥、黑蒙,至某医院就诊,诊断为特发性室性心动过速(室速),给予电复律治疗。出院后每于劳累、饮酒后出现心悸、胸闷,每次持续40分钟至3小时不等,有时静脉注射普罗帕酮70mg约30分钟症状可缓解。     

致心律失常性右心室心肌病诊断与治疗

致心律失常性右心室心肌病(ARVC),又称致心律失常性右心室发育不良/心肌病(ARVD/C)为欧洲运动猝死最常见的病因。50%~70%的病例是家族性的[1],主要为常染色体显性遗传,外显率不一。大多数病例死亡时的年龄小于40岁,有些发生于儿童。致心律失常性右心室心肌病的病理特征为右心室内的心肌萎缩和纤维脂肪组织替代。初期的描述是成年人中出现的具有左束支传导阻滞图形的单形性室性心动过速(提示心动过速的右心室起源)以及在肺血管床正常的情况下右心室扩大。认为此病起源于右心室心肌的发育异常,从而称右心室心肌病为致心律失常性右心室发育…     

超声心动图诊断心律失常性右心室发育不良的价值

目的探讨心律失常性右心室发育不良的彩色多普勒超声心动图特征。方法回顾性分析经彩色多普勒超声检查诊断的5例心律失常性右心室发育不良的临床资料,总结心律失常性右心室发育不良超声诊断特点,并对国内外相关文献进行回顾。结果 5例患者彩色多普勒超声检查均有异常改变,4例有不同程度的右心扩大,3例右心室局限性膨出形成室壁瘤,5例均有右心室壁变薄、室壁运动减低等征象,而左心系统及肺动脉正常。结论心律失常性右心室发育不良特异性超声心动图特点为右心扩大合并室壁瘤或室壁变薄;彩色多普勒超声检查对心律失常性右心室发育不良的临床诊断有重要作用。     

致心律失常性右心室心肌病

致心律失常性右心室心肌病(arrhythmogenic fight ventricular cardiomyopathy,ARVC)亦称致心律失常性右心室发育不良,是一种以右心室的结构和功能异常为特点的心肌病变,心肌细胞被脂肪和纤维组织所取代,有一定家族倾向,是年轻人和运动员猝死的主要病因.在30岁以下的猝死患者中,ARVC可占20%.有报道称ARVC发病率约为1/5000,由于存在漏诊、误诊,可能实际发病率要高一些[1].     

致心律失常性右心室心肌病17例临床分析

目的:探讨致心律失常性右心室心肌病(arrhythmogenic right ventricular cardiomyopathy,ARVC)的临床特点,提高对其诊断与治疗的认识.方法:对17例ARVC患者的临床表现及心电图、UCG、电生理检查(心室晚电位和心内电生理检查)、核素显像、心室造影等结果进行回顾性分析,评价其诊断和治疗方法.结果:入院时17例患者均有右心室结构或功能异常;12例心电图表现室性心律失常,其中10例室性期前收缩或室性心动过速呈左束支传导阻滞型,胸导联V1QRS波群时限大于110 ms12例,见Epsilon波2例,胸导联V1～4见T波倒置7例;心室晚电位阳性4例.胺碘酮、利多卡因等治疗有效.行心内电生理检查的3例患者,仅1例射频消融治疗有效.结论:ARVC以室性心律失常为主要表现,心电图有特征性改变,诊断主要依靠UCG.治疗以药物为主,射频消融未显示优越性.     

致心律失常性右心室心肌病心电图和动态心电图临床分析

目的：探讨致心律失常性右心室心肌病（ ARVC）的心电图和动态心电图特点,并对其心电图按病变程度加以分析。方法回顾性分析34例已确诊的ARVC患者的临床资料和辅助检查资料。按心脏磁共振成像（MRI）所示的病变累及部位将34例患者分为3组：A组（右心室局部病变组）11例、B组（右心室弥漫病变组）15例和C组（双心室病变组）8例,比较分析3组患者的临床症状、心电图和24 h动态心电图表现。结果6种临床表现心悸、胸闷、晕厥、室性期前收缩≥1000次、持续室性心动过速（ VT）、非持续VT的发生率在A、B、C 3组间比较差异均无统计学意义（ P均＞0.05）。与A组比较, C组Epsilon 波（4/8 vs．2/11）、V1～V3导联QRS波时限≥110 ms（3/8 vs．1/11）、病理性Q波（3/8 vs．1/11）和V1～V3 S波升支≥55 ms（4/8 vs．2/11）的发生率均明显升高（P＜0.05或P＜0.01）。与A组比较,B组和C组V1导联T波倒置的发生率均明显降低（3/15、1/8 vs．5/11）,超过V3导联的T波倒置发生率均明显增加（6/15、6/8 vs．1/11, P＜0.05和P＜0.01）。24 h动态心电图显示：10例患者为单形性室性期前收缩,发生率29.4％（10/34）,无不良事件出现;24例患者为多形性室性期前收缩,发生率70.6％（24/34）,8例出现不良事件。结论根据心电图中T波倒置的导联位置可以判断ARVC的病变范围及严重程度,而临床症状并无诊断其严重程度的特异性。     

致心律失常性右心室心肌病与心源性猝死

心源性猝死（suddencardiacdeath,SCD）是指因心脏自身疾病而导致的猝死,是所有猝死案例中最常见的一种类型,约占全部猝死案例的80％以上[1]。致心律失常性右心室心肌病（arrhythmogenicrightventrieularcardiomyopathy,AInrC）,     

致心律失常性右心室发育不良的临床研究进展

<正> 1978年Frank和Fontaine首次描述因右心室游离壁脱落、脂肪组织浸润及纤维组织增加引起的以左束支传导阻滞(LBBB),持续性室性心动过速(Ventricular tachycardia, VT)和室性期外收缩(VPC)为主的一组心律失常性临床症候群,并将其命名为致心律失常性右心室发育不良(Arrhythmogenic right ventricular dysplasia,ARVD)。由于本病罕见,病情复杂,诊断困难,通常药疗难以奏效,又易与Uh1病混淆,所     

Evaluation and course of an unusual case of arrhythmogenic right ventricular dysplasia

We report a case of a 42-year-old Caucasian man who presented with isolated right ventricular failure and atrial fibrillation without ventricular arrhythmia. In this report, we describe accurate evaluation by MR imaging confirmed by histopathologic findings as well as imaging progression of this unusual case of arrhythmogenic right ventricular dysplasia.     

Radiofrequency ablation of the ventricular tachycardia with arrhythmogenic right ventricular cardiomyopathy using non-contact mapping

BACKGROUND: Intracardiac non-contact mapping provides a rapid and accurate isopotential mapping that facilitates catheter ablation of the ventricular tachyarrhythmias in arrhythmogenic right ventricular cardiomyopathy (ARVC). METHODS: Thirty-two consecutive patients (26 men and 6 women, mean 37.2 +/- 13.8 years) were treated with ablation. Fourteen patients had a history of syncope/pre-syncope. Two patients had an implantable cardiac defibrillator (ICD) previously implanted. RESULTS: There were 67 ventricular tachycardias (VTs) induced in the 32 patients. The average VT rate was 210 +/- 32.2 (130-310) bpm. There were 42 episodes of VT that had a heart rate > or =200 bpm and 24 of the 32 patients (75%) had > or =2 morphologies of VT. Regional ablation was applied by targeting the earliest VT activation sites under the guidance of non-contact mapping. Acute success was achieved in 84.4% (27/32) patients, and significant improvement was seen in 15.6% (5/32) patients as evidenced by a slower rate of VT. None of the patients experienced syncope/pre-syncope or sudden death during the 28.6 +/- 16 (9-72) month follow-up. There were no complications of the procedure. At the end of follow-up, 81.3% of the patients were free of VT without medication while the rest of the patients achieved a modified success. CONCLUSIONS: The rapid ventricular tachyarrhythmias in ARVC patients can be abolished or improved significantly by regional RF catheter ablation under the guidance of non-contact mapping. There was no sudden cardiac arrest or death in those patients without ICD implantation. Delayed efficacy may occur in some patients after ablation.     

Electroanatomic mapping of endocardial right ventricular activation as a guide for catheter ablation in patients with arrhythmogenic right ventricular dysplasia

Arrhythmogenic right ventricular dysplasia is a structural heart disease characterized by fibrofatty degeneration of right ventricular myocardium and arrhythmias of right ventricular origin. The aim of this study was to characterize endocardial right ventricular activation by electroanatomic mapping as a guide for catheter ablation in patients with arrhythmogenic right ventricular dysplasia. Electroanatomic mapping and entrainment procedures were performed in 5 patients with arrhythmogenic right ventricular dysplasia. Endocardial mapping during ventricular tachycardia demonstrated a focal activation pattern with radial spreading of activation from a site of earliest ventricular activation in all directions. Right ventricular activation time (127 +/- 34 ms) was markedly shorter than tachycardia cycle length (415 +/- 92 ms). The site of earliest ventricular activation was found in an aneurysmal outflow tract (n = 2), at the border of aneurysms near the tricuspid annulus (n = 2), and at the apex of the right ventricle (n = 1). Entrainment mapping criteria of these areas of earliest endocardial activity were consistent with exit sites of a reentrant circuit in an area of abnormal myocardium. Fractionated potentials were found 61 +/- 29 ms before the onset of the QRS complex at these sites. Catheter ablation rendered the "clinical" ventricular tachycardia noninducible in four patients but "nonclinical" faster ventricular tachycardias were inducible in three patients. During the follow-up of 7 +/- 3 months after ablation, the frequency of therapies in 4 patients with an implantable cardioverter defibrillator decreased from 49 +/- 61 episodes per month before ablation, to 0.3 +/- 0.5 episodes per month after ablation (P < 0.05). Electroanatomic mapping during ventricular tachycardia facilitates localization of exit sites in relation to aneurysms in diseased right ventricle and may guide catheter ablation in patients with arrhythmogenic right ventricular dysplasia.     

致心律失常性右室心肌病心脏移植术后的临床病理分析

目的分析致心律失常性右室心肌病的病理学特点及心脏移植术后疗效的病理学观察。方法收集心脏移植术后病理确诊的致心律失常性右室心肌病病例共8例，对受体心脏进行测量、大体观察，行苏木素伊红染色和磷钨酸苏木素染色后光镜观察，1例进行电镜观察，移植术后心内膜心肌活检进行急性细胞性排斥反应分级。结果受体心脏重量均增加，全心增大，心室扩张。左、右心室较广泛的灶性脂肪浸润，右室为重。间质纤维组织增生、灶性瘢痕的形成则多以左室为重。3例室间隔可见灶性脂肪浸润。瓣膜和冠状动脉病理改变轻。1例电镜观察可见病毒颗粒。移植术后急性细胞性排斥反应除1例一次为ⅢA外，其余为0-Ⅱ级。术前临床诊断与病理诊断结果不符合6例。结论致心律失常性右室心肌病以右室心肌组织被脂肪组织和（或）纤维组织所取代为病理特征，可累及左室和室间隔，心脏移植术为治疗晚期患者的可行有效方法。     

Risk stratification for sudden death in arrhythmogenic right ventricular cardiomyopathy

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC) is an uncommon but increasingly recognized inherited cardiomyopathy that is associated with malignant ventricular arrhythmias and sudden cardiac death, particularly in young individuals. The implantable cardioverter-defibrillator (ICD) is widely regarded as the only treatment modality with evidence to support improved survival in patients with ARVC and secondary prevention indications. In contrast, there is no universally accepted risk stratification scheme to guide ICD therapy for primary prevention against sudden cardiac death. Potential benefits must be weighed against the considerable risks of complications and inappropriate shocks in this young patient population. This article tackles the challenges of risk stratification for sudden cardiac death in ARVC and critically appraises available evidence for various proposed risk factors. The authors’ over-arching objective is to provide the clinician with evidence-based guidance to inform decisions regarding the selection of appropriate candidates with ARVC for ICD therapy.     

多模态超声心动图诊断致心律失常性右心室心肌病1例北大核心CSCD

患者男,39岁,阵发性心悸7个月,加重伴全身无力,腹痛、恶心、呕吐1周;5年前偶发心悸,稍作休息可自行消失,未接受干预入院。本次入院后心电图显示室性心动过速、完全性右束支阻滞和T波倒置。超声心动图:标准心尖和胸骨旁四腔心切面见右心室无明显异常,右心室切面及右心室流入道切面分别示右心室侧壁心尖部及右心室下壁的基底段局部室壁膨出和运动幅度明显减低。     

Arrhythmogenic right ventricular cardiomyopathy/dysplasia: a case report of identical twins with heart failure

Arrhythmogenic right ventricular cardiomyopathy/dysplasia is characterized by the progressive replacement of myocardium by fatty or fibrofatty tissue. Presenting symptoms are generally related to ventricular arrhythmias, including sudden cardiac death. Heart failure due to right ventricular and sometimes left ventricular dysfunction is uncommon in the early stages of the disease, but is known to occur in advanced cases. This case report describes identical adolescent twins with presenting symptoms related predominantly to right heart failure.     

Concordance between an electroanatomic mapping system and cardiac MRI in arrhythmogenic right ventricular cardiomyopathy

A 29-year-old man presenting with syncopal ventricular tachycardia was diagnosed with arrhythmogenic right ventricular (RV) cardiomyopathy. Cardiac magnetic resonance imaging (MRI) revealed an unequivocal dyskinetic segment at the basal portion of the RV lateral free wall. Three-dimensional electroanatomic voltage mapping using the EnSite NavX system recorded a low voltage area corresponding to the diseased portion of the right ventricle identified by MRI. This report describes concordance between cardiac MRI and this novel mapping system in arrhythmogenic RV cardiomyopathy.     

Biventricular myocardial strain analysis in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) using cardiovascular magnetic resonance feature tracking

Background

Fibrofatty degeneration of myocardium in ARVC is associated with wall motion abnormalities. The aim of this study was to examine whether Cardiovascular Magnetic Resonance (CMR) based strain analysis using feature tracking (FT) can serve as a quantifiable measure to confirm global and regional ventricular dysfunction in ARVC patients and support the early detection of ARVC.

Methods

We enrolled 20 patients with ARVC, 30 with borderline ARVC and 22 subjects with a positive family history but no clinical signs of a manifest ARVC. 10 healthy volunteers (HV) served as controls. 15 ARVC patients received genotyping for Plakophilin-2 mutation (PKP-2), of which 7 were found to be positive. Cine MR datasets of all subjects were assessed for myocardial strain using FT (TomTec Diogenes Software). Global strain and strain rate in radial, circumferential and longitudinal mode were assessed for the right and left ventricle. In addition strain analysis at a segmental level was performed for the right ventricular free wall.

Results

RV global longitudinal strain rates in ARVC (−0.68 ± 0.36 sec⁻¹) and borderline ARVC (−0.85 ± 0.36 sec⁻¹) were significantly reduced in comparison with HV (−1.38 ± 0.52 sec⁻¹, p ≤ 0.05). Furthermore, in ARVC patients RV global circumferential strain and strain rates at the basal level were significantly reduced compared with HV (strain: −5.1 ± 2.7 vs. -9.2 ± 3.6%; strain rate: −0.31 ± 0.13 sec⁻¹ vs. -0.61 ± 0.21 sec⁻¹). Even for patients with ARVC or borderline ARVC and normal RV ejection fraction (n=30) global longitudinal strain rate proved to be significantly reduced compared with HV (−0.9 ± 0.3 vs. -1.4 ± 0.5 sec⁻¹; p < 0.005). In ARVC patients with PKP-2 mutation there was a clear trend towards a more pronounced impairment in RV global longitudinal strain rate. On ROC analysis RV global longitudinal strain rate and circumferential strain rate at the basal level proved to be the best discriminators between ARVC patients and HV (AUC: 0.9 and 0.92, respectively).

Conclusion

CMR based strain analysis using FT is an objective and useful measure for quantification of wall motion abnormalities in ARVC. It allows differentiation between manifest or borderline ARVC and HV, even if ejection fraction is still normal.     

Arrhythmogenic right ventricular dysplasia in the elderly

Arrhythmogenic right ventricular dysplasia (ARVD) is a syndrome characterized by recurrent ventricular tachycardia. However, cases of ARVD have also presented as dilated cardiomyopathy later associated with ventricular arrhythmias. Histologically, this syndrome manifests as a fibrofatty replacement of the RV myocardium, predisposing the heart to ventricular arrhythmias. ARVD was once considered a disease of the young, but may be underrecognized in the older population. This report presents two cases of elderly men with newly symptomatic ARVD, believed to represent the oldest patients with ARVD described in the literature to date. The diagnosis of ARVD should be considered in individuals of all ages who present with a clinical syndrome consistent with ARVD and supportive evidence on ECG.