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     

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.     

特发性室速的临床特点分析

目的探讨特发性室速的临床特点.方法收集23例特发性室速和21例病因明确的继发性室速病人的资料,分别从临床表现、心电图特点和治疗进行对比分析.结果两组比较,特发性室速病人年龄较轻,病程较长,出现晕厥及血压下降者较少;室速发作时,左室特发性室速心电图主要表现为右束支传导阻滞(RBBB) 电轴左偏,右室特发性室速主要表现为左束支传导阻滞(LBBB) 电轴右偏(或下偏):射频消融对特发性室速治愈率高,并发症少;特发性室速预后好.结论掌握特发性室速的临床特点,有利于此类病人的诊断、治疗和预后判断.     

致心律失常型右心室心肌病伴发心律失常性心肌病1例

致心律失常型右心室心肌病(arrhythmogenic right ventricular cardiomyopathy,ARVC)是一种遗传性心肌病,以右心室心肌被纤维和脂肪组织替代为病理特征,约50％的患者双心室受累[1].西方人群该病患病率约为1/5000 ～1/1000,临床比较少见.在该病发展的不同阶段,先后出现心律失常、右心室结构及功能异常以及全心衰竭等临床表现,是35岁以下人群心脏性猝死(sudden cardiac death,SCD)的重要原因之一[2].该病的心律失常以室性心律失常为特征,但房性心律失常同样很多见,长时间的快速性房性心律失常可以导致心功能进一步恶化.本文报告1例北京大学第三医院收治的ARVC伴发房性心动过速,并导致心律失常性心肌病的病例.     

Screening for arrhythmogenic right ventricular cardiomyopathy: a value of genetic testing

To the editor:We read with great interest the article by Yan and o-author 1 entitled A case of sudden death due to arrhythmogenic right ventricular cardiomyopathy,which was published in the previous issue of Chinese Medical Journal. They presented a case of sudden death due to arrhythmogenic right ventricular cardiomyopathy (ARVC) based on autopsy findings.In lethal cases due to ARVC,they also suggested taking genetic testing as a regular examination to identify their family members who are at risk of ARVC,and seek treatment early.This case gives detailed information and highlights the structural abnormalities and management of the ARVC.     

右室不同起搏部位对老年病窦综合征患者左心室收缩功能的影响

目的 比较右室不同起搏部位对老年病窦综合征(SSS)患者左心室收缩功能的影响.方法 选取该院2014-2016年收治的78例SSS患者,按照右室不同起搏部位分为右室心尖部(RVA)起搏组(40例)和右室流出道(RVOT)起搏组(38例),比较术前、术后3个月和术后9个月两组起搏器QRS波时限、总计右心室起搏百分比和左心室收缩功能指标.结果 两组患者术前QRS波时限和各项左心室收缩功能指标比较,差异均无统计学意义(P＞0.05).术后3个月和术后9个月,RVA起搏组患者QRS波时限较RVOT起搏组长,差异有统计学意义(P＜0.05).术后9个月两组总计起搏百分比比较,差异无统计学意义(P＞0.05);而RVOT起搏组左心室射血分数高于RVA起搏组,左心室舒张末内径小于RVA起搏组,差异均有统计学意义(P＜0.05).结论 RVOT起搏对SSS患者左心室收缩功能的作用优于RVA起搏.     

单导管射频消融治疗主肺动脉干室性早搏／室性心动过速临床观察

目的探讨起源于主肺动脉干（MSPA）室性早搏（PVCs）/室性心动过速（VT）的心电图特征、标测方法及单导管射频消融治疗的疗效与安全性。方法选取经肺动脉造影证实起源于MSPA的PVCs／VT患者12例（MSPA组）以及右心室流出道（RVOT）前间隔起源的PVCs／VT患者59例（RVOT组）,均采用单导管常规标测技术进行标测、消融。比较分析两组患者心电图特征,随访MSPA组患者的疗效。结果MSPA组患者体表心电图具有下壁导联R波振幅高以及胸前导联移形较早（多位于V2～V3）的特点,其有效靶点高电压（8V）可起搏心室,并与自发PVCs/VT的QRS波形12导联完全相同,有效靶点心室电位较PVCs／VT体表心电图QRS波起始点提早（33.91±3.69）ms,10例可记录到远场A、V波,6例可记录到融合或分离的尖峰或碎裂电位。所有消融治疗均成功,无并发症发生,随访期间无复发病例。结论起源于MSPA的PVCs／VT并非罕见．单导管标测及消融安全、有效．     

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.     

右室间隔部起搏对慢快综合征患者血流动力学及抗心律失常的研究

目的:探讨对慢快综合征患者行间隔部起搏后远期的血流动力学影响及间隔部起搏对阵发性房颤的控制效果。方法:需要植入DDD型起搏器的78例慢快综合征患者,按照心室电极的位置,随机分为右心室间隔部(RVS)起搏和右室心尖(RVA)起搏两组,对起搏器植入前、植入后第6个月和第12个月的QRS波宽度、左室射血分数、左室舒张末期内径、P波离散度、房颤负荷以及术中阈值、电极阻抗、感知差异进行分析。结果:术后6个月、12个月随访两组患者,RVS组患者QRS时限较术前有延长趋势但未达显著水平(P>0.05),RVA组患者QRS时限较术前显著延长(P<0.05);RVS组患者心功能和左室舒张末径较手术前无明显变化,RVA组患者心功能较手术前明显降低,左室舒张末径较手术前明显增加,两组相比,RVA组患者左室射血分数较RVS组患者低,两组左室舒张末期内径有明显差异(P<0.05);术后RVS组患者较术前P波离散度和房颤负荷无明显变化,而RVA组患者较术前P波离散度和房颤负荷增加。结论:右心室间隔部起搏是安全、有效的,右心室间隔部起搏将稳定或改善慢快综合征患者的远期心功能,最大限度减少阵发性房颤的发作。     

射频消融治疗特发性室性心动过速9例临床报告

目的观察射频消融技术治疗特发性室性心动过速 (IVT)的临床效果。方法根据发作IVT时的体表 12导联心电图定位起源部位 ,行心内电生理检查 ,寻找稳定诱发IVT的条件 ,采用激动标测或 (和 )起搏标测相结合的方法标测靶点 ,对起源左心室间隔部的IVT寻找Purkinie电位 (p电位 ) ,标测靶点后放电消融。结果 11例呈RBBB图形伴电轴左偏或重度右偏 ,靶点在左心室间隔面中、下部 ,10例找到p电位。 7例为LBBB图形伴电轴不偏或右偏 ,靶点在右心室流出道 ;1例为LBBB图形伴电轴左偏 ,靶点在右心室流人道。l例IVT同时起源右室流出道的间隔部和游离壁 ,l例左心室特发性室性心动过速 (ILVT)合并房室结折返性心动过速的患者先消融房室结双径路 ,再行ILVT的消融。 1例右心室特发性室性心动过速 (IRVT)消融虽未立即成功 ,因延迟作用 3周后心动过速消失。消融成功患者的临床症状消失 ,未出现手术相关的并发症 ,随访期内所有病例存活。结论射频消融治疗IVT临床疗效肯定、成功率高、安全 ,应作为首选的根治方法。     

Left posterior fascicular block: a new endpoint of ablation for verapamil-sensitive idiopathic ventricular tachycardia

Verapamil-sensitive idiopathic left ventricular tachycardia with a right bundle-branch block and left-axis deviation morphology, which occurs in young adults without structural heart disease is an uncommon but well described clinical arrhythmia. This ventricular tachycardia (VT) is known to be due to re-entry mechanism and the re-entrant circuit is localized in the left ventricular septum.1-10 However, the relationship between Purkinje potential and the reentrant circuit is still controversia…     

应用多普勒超声心动图和心导管同步描记术对室速血流动力学的研究

用超速抑制刺激方式致健康犬不同心室起源部位的室性心动过速，应用彩色多普勒超声和心导管同步描记术测得血流动力学的一系列指标对室速发作时的左室收缩及舒张功能进行研究。结果表明：不同心室起源部位的室性心动过速对左室收缩及舒张功能影响的严重程度不同，以左右室心尖诱发的室速影响程度最小，左右室侧壁处次之，以左右室流出道部位诱发的室速对左室收缩及舒张功能影响的严重程度最大。     

右心室流出道起搏和右心室心尖部起搏的临床观察

目的比较右心室流出道起搏和右心室心尖部起搏对心脏功能的影响。方法将安装埋藏式DDD心脏起搏器的患者随机分成右心室流出道起搏组和右心室心尖部起搏组,比较两组患者术后的QRS波宽度、术前和术后的左心室舒张末内径(LVEDD)、左心室射血分数(LVEF)、左房内径(LA)。结果两组患者QRS波宽度间差异有统计学意义(P<0.05);术后3个月及6个月时,LVEDD、LVEF、LA间比较差异无统计学意义(P>0.05)。结论右心室流出道起搏和右心室心尖部起搏对患者LVEDD、LVEF和LA的影响无差别,但右心室流出道起搏者的QRS波宽度明显较窄。     

射频消融治疗心室流出道特发性室性心律失常的临床评价

目的探讨射频消融治疗心室流出道特发性室性心动过速（VT）和室性早搏（PVC）的临床效果、心电图及电生理特征。方法采用起搏标测、激动标测和非接触标测的方法对105例心室流出道室性心律失常的患者进行射频消融治疗,其中男42例,女63例,年龄47±16（12—73）岁。VT27例,PVC78例。结果（1）105例患者中97例成功,8例失败,成功率93．3％。15例复发,复发者再次消融后成功。（2）起源于右室流出道84例,左室流出道21例。其中起源于主动脉瓣上Valsalva左冠窦16例,主动脉瓣下左室流出道心内膜2例,主动脉瓣与二尖瓣环纤维连接处3例。（3）术中出现1例急性心包压塞,2例少量心包积液。结论射频导管消融治疗心室流出道特发性室性心律失常是一种安全、有效的方法。     

致心律失常性右室心肌病DSP基因突变与表现型的关系

【目的】 在ARVC患者中筛选DSP基因突变,并与患者的表现型进行对比分析。【方法】 收集ARVC患者40例,评估患者的临床指标,并随访心脏事件(室性心律失常、心衰和猝死)的发生。对其中32例ARVC患者的DSP基因突变进行筛选。 【结果】 患者出现症状的时间为32.2 ± 12.7岁,男性患者的比例较高(85.0%),最常见的症状是心悸(82.5%),其次是胸痛(25.0%)和晕厥(22.5%)。T波倒置(75.0%)是最常见的心电图表现,随后QRS波时限延长(45.0%)和Epsilon波(35.0%)。伴左束支传导阻滞的室性心动过速有28例(70.0%)。32例ARVC患者中共7例(21.9%)患者发现了DSP突变,共检测出6个突变位点,对照组染色体(n = 200)上并未发现这几个位点有改变。比较DSP和非DSP突变患者的临床特征,结果发现右室与左室容积之比两组之间的比较有意义(1.33 ± 0.24 vs. 1.28 ± 0.14,P < 0.05)。研究期间,共有3例患者死亡,平均死亡率是7.5%,DSP突变组有1例死亡,而非DSP突变组有2例死亡患者死亡。比较两组的生存时间无差别(19.43 ± 2.38 vs. 28.65 ± 0.90, P > 0.05)。【结论】 DSP基因突变的检出率为21.9%,与国外的研究结果基本一致,但中国DSP突变患者的右室扩大较为明显,左室累及较少。     

Clinical study of 39 Chinese patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy

Background There are few studies on the clinical profile of Chinese patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy （ARVD/C）. The purpose of this study was to describe the clinical characteristics of ARVD/C patients from China, particularly to define the features of electrocardiograph and treatment outcomes.
Methods Thirty-nine patients hospitalized in Fu Wai Cardiovascular Hospital from 1998 to 2006 were included. The data were obtained from the medical archive and the follow-up records.
Results Of these patients 33 were male and 6 female （age at the first presentation was （34.9 ± 9.8） years）. The most common symptoms were palpitation （62%） and syncope （44%）. Right precordial QRSd 〉 110 ms was detected in 69% of the patients, epsilon wave in 59%, and a ratio of QRSd in V1＋V2＋V3/V4＋V5＋V6 ≥ 1.2 in 82%. The most frequent features of electrocardiogram in patients without right bundle-branch block were T-wave inversions and S-wave upstroke in V1-V3 〉55 ms （96% and 90% of 28 patients, respectively）. Radiofrequency catheter ablation （RFCA） for ventricular tachycardia （VT） was successful in 15 （68%） of 22 patients. The recurrence rate of VT was 46% （7/15） during the follow-up of （16.7 ± 11.2） months. Seven patients had cardioverter/defibrillator （ICD） implanted plus drug therapy and 17 patients took antiarrhythmic drugs alone. During the follow-up of （35.6 ± 19.0） months, all patients with ICD implanted received at least one appropriate ICD shock. One patient died of ventricular fibrillation suddenly and one patient underwent heart transplantation for progressive biventricular heart failure during the drug therapy alone.
Conclusions This study demonstrated the clinical and ECG features of the 39 ARVD/C Chinese patients. ICD provided life-saving protection by effectively terminating malignant arrhythmias, and the high recurrence of VT was the major problem of RFCA therapy.     

Ⅲ度房室传导阻滞患者行右室流出道间隔部与右室心尖部起搏的对照研究

目的观察右心室不同部位起搏对Ⅲ度房室传导阻滞行起搏器置入患者心功能的影响。方法 45例行双腔起搏器置入的Ⅲ度房室传导阻滞患者,按心室电极固定部位分右室心尖部（RVA）起搏组和右室流出道间隔部（RVOTS）起搏组,于术后3、12以及24个月末对两组患者行超声心动图检查、采血测血浆B型钠尿肽（BNP）以及行6分钟步行距离（6MHW）测试。结果术后随访3个月时,两组超声心动图检查结果无统计学差异,两组患者BNP水平均无〉400 pg/ml者,6MHW亦无明显差异。术后12个月时,RVA组左室舒张末期内径（LVEDD）较术后3个月时增大（P〉0.05）,LVEF较术后3个月时下降（P〉0.05）,术后24个月,这种差异更明显（P〈0.05）。但RVOTS组的LVEF以及LVEDD在术后12个月以及24个月时与术后3个月时无明显差异（P〉0.05）。术后24个月时,RVA组BNP〉100 pg/ml者9例,而RVOTS组中只有1例,而术后24个月时RVA有2例患者BNP〉400 pg/ml,但RVOTS组无患者BNP〉400 pg/ml。在术后24个月时,RVA组患者6MHW较术后3个月时明显减少,而RVOTS组患者却明显增加（P〈0.05）。结论右室流出道间隔部起搏安全、有效,比右心室心尖部起搏更有利于双心室电激动的同步性,避免心功能的恶化。     

电解剖标测系统引导经导管消融治疗ARVC

目的探讨电解剖标测（CARTO）系统引导经盐水灌注导管消融治疗致心律失常右室心肌病（ARVC）的室性心动过速方法，并观察其疗效。方法3例患者均通过CARTO系统进行电解剖标测，分别进行激动标测和电压标测，结合标测碎裂电位和拖带标测等方法确定并消融室速。结果共诱发出4种形态的室速，其心电图形态不同，但均为左束支阻滞形室速。在CARTO系统标测指导下对室速进行消融成功。随访3～12个月，未发生室速。术中无并发症发生。结论ARVC所致的室速可在CARTO系统标测下行射频消融，且安全有效。     

Implantation of Lumenless Pacing Leads at the Inter-atrial Septum and Right Ventricular Outflow Tract with Deflectable Catheter-sheath

Current permanent right ventricular and right atrial endocardial pacing leads are implanted utilizing a central lumen stylet. Right ventricular apex pacing initiates an abnormal asynchronous electrical activation pattern, which results in asynchronous ventricular contraction and relaxation. When pacing from right atrial appendage, the conduction time between two atria will be prolonged, which results in heterogeneity for both depolarization and repolarization. Six patients with Class Ⅰ indication for permanent pacing were implanted with either single chamber or dual chamber pacemaker. The SelectSecure 3830 4-French （Fr） lumenless lead and the SelectSite C304 8.5-Fr steerable catheter-sheath （Medtronic Inc., USA） were used. Pre-selected pacing sites included inter-atrial septum and right ventricular outflow tract, which were defined by ECG and fluoroscopic criteria. All the implanting procedures were successful without complication. Testing results （mean atrial pacing threshold： 0.87 V; mean P wave amplitude： 2.28 mV; mean ventricular pacing threshold： 0.53V; mean R wave amplitude： 8.75 mV） were satisfactory. It is concluded that implantation of a 4-Fr lumenless pacing lead by using a streerable catheter-sheath to achieve inter-atrial septum or right ventricular outflow tract pacing is safe and feasible.     

起搏QRS波时限与左心结构和功能的关系

目的探讨长期右室心尖起搏患者的起搏QRS时限与左心结构、左心室收缩功能及心室间不同步的关系。方法长期右室心尖部起搏患者共105例,通过常规体表心电图测得起搏QRS(pQRS)时限,运用常规心脏超声心动图检测主动脉根部内径(AO)、左房内径(LAD)、收缩末期左心室内径(LVDs)、舒张末期左心室内径(LVDd)、室间隔厚度(IVST)、左室后壁厚度(LVPWT)及左室射血分数(LVEF),分析pQRS时限与以上各心超指标的关系。结果 pQRS时限与LVDd、LVDs及IVST呈正相关(r分别为0.334、0.385和0.289,前两者P<0.01,后者P<0.05),与LVEF负相关(r=-0.312,P<0.05);pQRS时限与LAD的相关性更显著(r=0.595,P<0.01)。以pQRS时限≥180ms诊断左房扩大时,敏感度和特异度分别为86.49%和67.74%。结论对于长期右室心尖部起搏的患者,pQRS时限与左心大小及左心室收缩功能相关;pQRS时限延长(≥180ms),提示左心房扩大。