心律失常性右心室心肌病一例分析

心律失常性右心室心肌病（ARVC）或称心律失常性右心室发育不良,主要由编码细胞桥粒蛋白的基因突变引起,导致细胞间交通障碍,进行性心肌细胞缺失和纤维脂肪组织取代右心室心肌细胞[1],以频繁发作的室性心动过速、心力衰竭和猝死为特征.美国心脏学会（AHA）和我国中华医学会分别于1996年及1999年正式将致心律失常性右室心肌病列为第四类原发性心肌病,与扩张型心肌病等并列.该类疾患的心电图有其独特表现.我们临床随访1例致心律失常性右室心肌病患者,将其心电图特点及相关基因检测结果结合国内外文献分析报道如下.     

左心室起源致心律失常性心肌病的临床特征与MRI影像特征

目的:分析左心室起源致心律失常性心肌病的临床特征与MRI影像特征.方法:采集我院2015年1月—2020年2月诊治的28例左心室起源致心律失常性心肌病(ALVC)患者作为ALVC组;将同期诊治的30例致心律失常性右心室心肌病(ARVC)患者作为ARVC组,分析患者病史、临床表现等具体临床特征;给予患者MRI检查,统计患...     

致心律失常性右室心肌病的临床诊断特点

致心律失常性右室心肌病（ARVC）是遗传性心肌病的一种,以右心室心肌细胞被纤维、脂肪组织代替为主要病理特征.临床主要表现为反复发生心律失常、心衰和猝死,在年轻人及运动员猝死中具有重要地位.因此,有必要进一步加深对该疾病的病理、生理和临床特征的认识和进一步研究.该文就ARVC的临床特点、心电图表现、超声心动图特点和核磁共振成像特点作一综述.     

致心律失常性右室心肌病的临床诊断探讨

致心律失常性右室心肌病 (ARVC)是一种主要累及右心室心肌组织的疾病[1] 。它在临床表现、心电图、超声心动图等诸方面有特殊临床特点。过去国内报道较少 ,与对该病认识不足有关。现将自 1994年至 2 0 0 1年收治的 16例ARVC患者资料汇总报告如下。1　临床资料1 1　一般资料 :16例ARVC患者中 ,男 13例 ,女 3例 ,年龄 12～ 4 0岁。均进行了心电图、超声心动图、X线胸片检查 ,3例做了活动平板心电图检查 ,6例做了2 4h动态心电图检查 ,12例行心内电生理检测 ,6例进行了射频消融治疗。1 2　临床表现 :ARVC中以男性为多 ,均为中青年人 ,以…     

致心律失常性右室心肌病新进展

<正>致心律失常性右室心肌病/发育不良(arrhythmogenic right ventricular cardiomyopathy/dysplasia,ARVC/ARVD),是一种少见的原发性心肌疾病。病因尚不清楚,无明确的人群发病率资料,发病年龄较广,从婴儿到成年人均有(4个月至59岁)。ARVC在心脏移植者中占1％,在青少年心源性猝死中占11.2％,其中46.7％为运动员,因此引起世界性的观注。现将ARVC的发病机制、临床表现、诊断、治疗等的新进展加以综述。     

评估心电图诊断致心律失常性右心室心肌病病变程度的临床研究

目的探讨心电图表现与致心律失常性右心室心肌病(ARVC)病变程度的关系。方法依照病变侵犯部位将59例ARVC患者分为双心室组、弥漫病变组和局部病变组比较3组病变累及部位、临床症状、心电图和24h动态心电图表现。结果局部病变组以右心室流出道和右心室心尖部多见,弥漫性病变组以右心室流出道和右心室游离壁多见,双心室组患者病变以右心室流出道和右心室游离壁多见。3组临床症状差异均无统计学意义性(均P>0.05)。3组心电图差异有统计学意义(P<0.05)。24 h动态心电图监测:17例为单形性室性期前收缩,无不良事件,均出现心悸表现;42例患者多形性室性期前收缩,其中9例昏厥,3例猝死。超声心动图检查:17例提示累及右心室心肌病。结论 ARVC临床症状与病变程度无明显关系,根据心电图T波倒置导联位置能够判断病变程度和范围。     

7例致心律失常右室心肌病临床分析

致心律失常右室心肌病(ARVC)是一种较少见的由右室发育不良导致的心肌疾病,以心律失常,心力衰竭猝死为主要表现,多见于青少年.患者右室常存在功能及结构异常,以右室心肌逐渐被脂肪及纤维组织代替为特征.     

致心律失常性右室心肌病的诊断与治疗

致心律失常性右室心肌病（Arrhythmogenic right ventricular cardioyopathy ARVC）是一种以频繁发作的室性心动过速（VT）、心力衰竭和猝死为特征的遗传相关性疾病。此病多发于中青年，男性居多。国外有报道在年轻人群中近20％的猝死是ARVC引起的。目前大多数ARVC的病例报告都来源于欧美人群。我国报告病例不多，尚没有关于在我国大规模人群中ARVC发病率的报告，但随着对此病研究的不断深入，研究者对ARVC患者的家族史、临床特点及治疗情况有了更为详细的了解。     

致心律失常性右室心肌病的诊疗进展

1概述 致心律失常性右室心肌病（arrhythmogenic rightventricular cardiomyopathy,ARVC）又称致心律失常性右室发育不良（arrhythmogenic right ventricular dysplasia,ARVD）,是以右室心肌被纤维脂肪取代为特征的心肌病,通常表现为局限性右室病变,亦可弥漫性进展,侵犯左室。     

致心律失常性右室心肌病的临床观察及鉴别诊断

致心律失常性右室心肌病(ARVC)是一种主要累及右室心肌组织的疾病。过去对该病认识不足,随着医疗技术的不断提高,对该病的研究有很大的进展,使之对该病的临床表现以及诊断治疗有新的认识。现将我院近年来收治的7例ARVC患者报告如下：     

致心律失常型右心室心肌病伴发心律失常性心肌病1例

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a kind of inherited cardio-myopathy, which is characterized by fibro-fatty replacement of right ventricular myocardium, leading to ventricular arrhythmia. However, rapid atrial arrhythmias are also common, including atrial fibrillation, atrial flutter and atrial tachycardia. Long term rapid atrial arrhythmia can lead to further deterioration of cardiac function. This case is a 51-year-old male. He was admitted to Department of Cardiology, Peking University Third Hospital with palpitation and fatigue after exercise. Electrocardiogram showed incessant atrial tachycardia. Echocardiography revealed dilation of all his four chambers, especially the right ventricle, with the left ventricular ejection fraction of 40% and the right ventricular hypokinesis. Cardiac magnetic resonance imaging found that the right ventricle was significantly enlarged, and the right ventricular aneurysm had formed; the right ventricular ejection fraction was as low as 8%, and the left ventricular ejection fraction was 35%. The patients met the diagnostic criteria of ARVC, and both left and right ventricles were involved. His physical activities were restricted, and metoprolol, digoxin, spironolactone and ramipril were given. Rivaroxaban was also given because atrial tachycardia could cause left atrial thrombosis and embolism. His atrial tachycardia converted spontaneously to normal sinus rhythm after these treatments. Since the patient had severe right ventricular dysfunction, frequent premature ventricular beats and non-sustained ventricular tachycardia on Holter monitoring, indicating a high risk of sudden death, implantable cardioverter defibrillator (ICD) was implanted. After discharge from hospital, physical activity restriction and the above medicines were continued. As rapid atrial arrhythmia could lead to inappropriate ICD shocks, amiodarone was added to prevent the recurrence of atrial tachycardia, and also control ventricular arrhythmia. After 6 months, echocardiography was repeated and showed that the left ventricle diameter was reduced significantly, and the left ventricular ejection fraction increased to 60%, while the size of right ventricle and right atrium decreased slightly. According to the clinical manifestations and outcomes, he was diagnosed with ARVC associated with arrhythmia induced cardiomyopathy. According to the results of his cardiac magnetic resonance imaging, the patient had left ventricular involvement caused by ARVC, and the persistent atrial tachycardia led to left ventricular systolic dysfunction.     

致心律失常性右心室心肌病临床分析

目的 :分析致心律失常性右心室心肌病的临床特点。方法 :将 2 3例致心律失常性右心室心肌病患者常规行超声心动图、心电图、胸片、动态心电图、心房调搏及心内电生理检查。结果 :本组 2 3例患者多有晕厥发作 ,频发室性早搏 (95 7% ) ,右心室源性短阵室速 (87% ) ,心电图多为右束支传导阻滞 (87% ) ,超声心动图右心室内径为 (5 0 1± 8 83)mm ,右心房内径为 (48± 8 79)mm ,右心室与左心室舒张末期内径之比为 1 0 9,右心功能减退 ,射血分数 (EF)为(0 32 5± 0 0 90 2 )。结论 :致心律失常性右心室心肌病 ,多有发作性晕厥 ,右束支传导阻滞 ,频发室早及左束支传导阻滞型室速 ,右心室、右心房增大 ,右心室功能减退 ,右心室与左心室舒张末期内径之比增大     

Clinical and familial study of arrhythmogenic right ventricular cardiomyopathy

Objective　To explore the characteristics of arrhythmogenic right ventricular cardiomyopathy (ARVC). Methods　Seven patients with arrhythmogenic right ventricular cardiomyopathy and 34 members of three families were studied. All patients and family members underwent history collection, clinical examination, electrocardiogram (ECG), two-dimensional echocardiography (2-DE) and a signal averaging electrocardiogram. Programmed ventricular stimulation was performed in five patients. Results　All patients and family members had normal morphologic characteristics and normal function of the left ventricular by 2-DE. Fourteen persons had abnormal findings indicating ARVC. Five had enlargement of the right ventricular with diffused hypocontractility, eight had thin and systolic bulging in the focal anterior wall with hypokinesia and one had bulging of the inferior wall. Twenty-five persons (seven patients and 18 family members) had abnormal findings in ECG. Positive ventricular late potential was recorded in 13 persons (six patients). Two to three monomorphic ventricular tachycardia (VT) with left bundle branch block (LBBB) configurations were induced in five patients. Ventricular fibrillation was induced in two patients during the electrophysiologic study (EPS). Five patients had very high pacing threshold and/or ineffective pacing in one or many regions of the right ventricle. Two members of one family died suddenly. One member was a dwarf with ARVC. Spontaneous VT with a left bundle branch block (LBBB) configuration was recorded in five patients, polymorphic VT with extremely short coupling interval in one, and premature ventricular complexes with LBBB configuration in 12 (six patients). Conclusion　Our familial study strongly suggests that ARVC may be a hereditary disease and it is helpful in the diagnosis and detection of ARVC. The most common manifestations were abnormal structure and function of the right ventricle and abnormal ECG of repolarization and ventricular arrhythmia which originates from the right ventricle.     

缺血性心肌病与扩张型心肌病的超声心动图特征比较与鉴别诊断

目的分析超声心动图在心肌病患者中的应用效果及临床价值。方法选取2018年7月至2019年5月本院收治的因射血分数低、心功能不全,且超声检查提示心脏扩张的100例患者为研究对象,所有患者实施冠状动脉造影后,结合临床综合诊断,明确缺血性心肌病者35例,扩张型心肌病者40例,酒精性心肌病16例,克山病9例。结果经分析,两组患者右室内径、左房内径、左室舒张末内径、二尖瓣舒张晚期血流速度峰值、主动脉瓣口收缩期峰值速度、左室舒末容积、左室缩末容积、射血分数比较差异具有统计学意义(P<0.05)。结论超声心动图能够准确鉴别缺血性心肌病及扩张型心肌病,可以为临床诊断以及治疗该疾病提供科学依据,值得临床推广。     

致心律失常性右心室心肌病临床分析

OBJECTIVE: To investigate the clinical features of arrthythmogenic right ventricular cardiomyopathy (ARVC), and to evaluate the diagnosis of ARVC. METHODS: Twenty-three cases of ARVC underwent ECG, chest x-ray, Holter, transesophageal atrial pacing (TEAP) and intracardiac electrophysiological examination. RESULTS: A syncope attack occurred; the percentage of frequent ventricular premature beats was 95.7%, salvos of the right ventricular originated ventricular tachycardia (VT) was 87%, and the right bundle branch block (RBBB) demonstrated by ECG was 87%; UCG showed that the enlarged right ventricular diametric was (50.1 +/- 8.83) mm, and the right atrium diametric was (48 +/- 8.79) mm; the ratio of right ventricular diametric to left ventricular diametric in the end-diastolic period was 1.09. The right ventricular function [ejection fraction: (0.325 +/- 0.0902)] decreased, which was confirmed by echocardiogram. CONCLUSION: The diagnosis of ARVE can be established on the basis of the repeated syncope attack; RBBB, frequent ventricular premature beats, VT of LBBB patterms, an enlarged right heart and decreased right ventricular function can be found, the ratio of right ventricular dimaetric to left ventricular diametric increases in the end-diastolic period.     

快速心房起搏制作猪心动过速心肌病动物模型研究

目的探讨通过快速心房起搏构建猪心动过速性心肌病模型的可行性。方法10只健康小猪经穿刺颈静脉途径植人AAI型起搏器,8只给予400次／min的快速右心房起搏2周,2只不起搏作为对照组,起搏前后应用超声心动图观测收缩、舒张末期左心室容积大小及左心室射血分数（LVEF）,并检测血心房钠尿肽（ANP）水平;通过苏木精-伊红染色观察左心室心肌病理改变。结果快速起搏2周后,猪左心室收缩末期容积（LVESV）和舒张末期容积（LVEDV）均显著增加（P〈0．05）;血浆中ANP显著增加,苏木精一伊红染色可见心室组织细胞排列紊乱,出现局灶性坏死、肌溶解、水肿、间质胶原结缔组织增生、炎性细胞浸润。结论短期快速心房起搏是建立猪心动过速心肌病动物模型简便而有效的方法。     

扩张型心肌病并发心律失常相关因素研究

目的探讨扩张型心肌病心律失常的相关因素。方法对符合WHO诊断标准的118例扩张型心肌病(DCM)患者的超声心动图与心电图检查结果进行一系列对比分析。结果118例房性心律失常在心房扩大组较无心房扩大组明显增高(P<0.05~<0.001),其中心房颤动的发生与心房腔扩大的程度密切相关;心室扩大的程度与各种室性心律失常的发生无明显关系;左室扩大程度与传导阻滞的发生有关,左室内径>60mm组发生传导阻滞较<60mm组显著增高(P<0.05~<0.001)。EF值<30mm组复杂心律失常发生率较EF值>30mm组明显增高(P<0.05~<0.001)。结论DCM并发房性心律失常与心房扩大有关,尤其心房颤动与心房扩大程度密切相关;室性心律失常与左室腔扩大程度无关,而可能与心肌组织病变程度有关;传导阻滞与左室腔扩大程度相关;并发复杂心律失常与心功能损害程度密切相关。     

缺血性心肌病死亡病因分析

目的通过对55例缺血性心肌病死亡病例回顾分析,总结其与死亡相关的危险因素。方法对55例缺血性心肌病患者死亡的临床资料及相关检查,进行分析总结。结果死亡患者年龄以80-89岁最多发生。既往有高血压病史者44例。反复心绞痛史者45例,心肌梗死史20例。心衰病程2年者31例。死亡病因：心衰伴呼衰17例;心跳骤停16例。所有病例均有心律失常发生,并发房颤32例;室性心律失常26例。电解质紊乱者38例,低钠血症25例。大多数患者合并低脂血症、低血清总蛋白、低白蛋白血症、肾功能损伤及血清肌钙蛋白增高等。结论缺血性心肌病末期患者,其肺部感染、心律失常、低钠血症、低脂、低白蛋白血症、肌钙蛋白持续增高,左室射血分数降低这些因素均为病情恶化指标,与死亡密切相关。     

高血压并长期嗜酒致心肌疾病20例分析

目的探讨高血压并长期嗜酒致心肌疾病患者的临床特点及发病机制。方法对20例高血压并长期嗜酒患者心肌疾病的临床表现及辅助检查结果进行回顾性分析。结果20例高血压并嗜酒致心肌疾病的平均发病年龄44.7±6.8岁,临床主要表现为心力衰竭和心律失常。超声心动图示左心房内径平均41.2±8.56mm,右心室内径平均17.0±5.1mm,左心室舒张末期内径平均62.7±8.46mm,室间隔厚度平均11.16±3.47mm,左室后壁舒张末期厚度平均11.5±2.75mm,左室射血分数平均35.6±7.2%。结论高血压并长期饮酒使心肌疾病发病提前,心力衰竭严重,心律失常类型复杂。     

P波离散度与扩张型心肌病的关系

目的：研究P波离散度（PWD）在扩张型心肌病中的表现。方法：研究对象分为两组，研究组为53例扩张型心肌病患者，对照组为53例健康查体者。测量心电图P波最大时限（Pmax）及P波最小时限（Pmin），PWD=Pmax—Pmin。用超声心动图测量左心房内径（LAD）、舒张末期左心室内径（LVEDD）、收缩末期左心室内径（LVESD）及左心室射血分数（LVEF）。结果：扩张性型心肌病患者的Pmax及PWD均明显延长，与对照组相比，差异显著（P〈0．001），而Pmin两组间无显著差异（P〉0．05）。Pmax及PWD与LAD均呈正相关（r=0．533，P〈0．001；r=0．636，P〈0．001），而Pmax及PWD与LVEF均呈负相关（r=-0．672，P〈0．001；r=-0．746，P〈0．001）。结论：在扩张型心肌病患者中PWD明显延长，并与其心功能不全程度明显相关。