扩张型心肌病的室性心律失常分析

２０例扩张型心肌病有ＶＡ，其中３５％频发室早，５５％有非持续性室速。常规心电图示４０％有ＶＡ，其中２０％有频发室早，未检出ＶＴ。ＶＴ组与非ＶＴ组心功能、血Ｋ＾＋、左室舒张末期内径、成对室早及多源室早发生率无显著差异，但ＶＴ组左室收缩末期内径显著增大，短轴缩短率明显降低，频发室早发生率显著增高。     

充血性心肌病发生心律失常研究

目的研究充血性心肌病(CM)发生心律失常的特点。方法129例CM患者均行常规心电图、24h动态心电图检查,对CM患者进行心律失常研究。结果CM发生心律失常为:室性期前收缩与室性心动过速78例(60.5%),房室传导阻滞21例(16.3%),心房颤动34例(26.4%),束支传导阻滞35例(27.1%),窦性心动过速8例(6.2%),病态窦房结综合征4例(3.1%)。室性期前收缩与室性心动过速患者组左室舒张末内径[(69.6±12.0)mm]同无室性期前收缩和室性心动过速组相比[(64.0±13.2)mm]无显著差异(P>0.05)。房室传导阻滞、束支传导阻滞组左室舒张末内径为[(69.2±15.2)mm],与无房室传导阻滞和束支传导阻滞组[(65.2±10.8)mm]相比有显著差异,P<0.05。结论CM患者发生心律失常以室性期前收缩与室性心动过速最常见,房室传导阻滞、束支传导阻滞组与无此种心律失常的CM组LVED有明显差异。     

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.     

原发性扩张型心肌病心律失常的临床分析

原发性扩张型心肌病系原因不明的心肌病变,无其它原因可解释的充血性心力衰竭,X线摄片和超声心动图证实心肌普遍增大,心腔主要为左和/或右室增大,心室壁变薄,运动功能弥漫性降低。心电图示传导阻滞,心肌损害和异常Q波及异位心律等,无冠心病易患因素,无高血压、心绞痛和心肌梗塞病史,除外其它类型心肌病,原发性扩张型心肌病的诊断即可成立。     

40例肥厚型心肌病与严重室性心律失常临床探讨

分析４０例肥厚型心肌病严重室性心律失常与室间隔厚度、心肌肥厚的广泛程度、心功能不会的严重程度有关。     

扩张型心肌病心律失常86例临床分析

本文对86例扩张型心肌病（DCM）患者的心律失常及有关因素作了分析，发现室性心律失常（VA）最为常见，其次为心房颤动（Af），左束支阻滞及Ⅰ°房室传导阻滞。VA的发生与左心室扩张程度有关，而与心衰程度无明显关系．Af与左心房扩大的程度有关，快速房颤往往加重心衰程度。     

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.     

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

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.     

原因不明晕厥病人电生理检查,心电图及动态心电图的初步分析

将41例原因不明晕厥病人的电生理检查(EPS)、心电图(ECG)及动态心电图(Hotler)结果作对比分析,结果表明:总体上,三者结果之间无一定关系,但对电生理指导治疗有效的20例分析的结果表明,ECG及Holter对房室结功能不良、希蒲系统功能不良及室性心动过速引起晕厥者,可能有提示作用,并可提供EPS的适应症。     

Electrocardiographical case. Young woman with frequent syncope attacks

A 29-year-old woman with frequent syncope attacks was referred for electrophysiological study and consideration for radio-frequency ablation of her ventricular arrhythmias. Her ECG showed features of right ventricular outflow tract premature contraction. Differential diagnoses for the causes of syncope in this patient include: right ventricular outflow tract tachycardia, arrythmogenic right ventricular dysplasia, and neurocardiogenic syncope. She underwent a tilt table test, which showed a malignant cardioinhibitory response. She developed abrupt syncope with 32 seconds of asystole during the test. She was given intravenous atropine and was resuscitated. A dual chamber rate-responsive pacemaker was implanted for her the next day. She was discharged well subsequently. Although the prognosis in patients with prolonged aystole in malignant vasovagal syncope is unknown, most doctors will still choose to implant a permanent pacemaker for patients with malignant neurocardiogenic syncope when the sinus arrest is prolonged.     

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。     

3．0T MRI 评价心脏正常结构及功能的实验研究

目的:探讨心脏磁共振(cardiac magnetic resonance imaging,CMRI)电影成像对于评价中国小型猪心脏结构及功能的价值。方法:应用快速稳态平衡进动(fast imaging employing stead-state acquisition,FIESTA)电影序列对15只健康中国小型猪进行CMRI检查,图像经Segment进行分析,并测量左室室壁厚度,左、右心室收缩及舒张末期容积,肺动脉主干管径,并将CMRI测量的结果与心脏超声(cardiac ultra sound,CUS)所测量的对应的左室室壁厚度,左、右室收缩及舒张末期容积,肺动脉主干管径进行相关性研究。此外,还用MRI测量舒张末期左、右心室肌质量。结果:(1)CMRI测得的舒张末期右心室肌质量,舒张末期左心室肌质量,舒张末期右心室与左心室肌质量比,右室舒张末期长、短径,右室舒张末期容积,右室射血分数,左室射血分数结果分别为(10±1)g,(38±2)g,0.25±0.03,(51.4±3.8)mm,(26.8±2.9)mm,(54±4)m L,0.54±0.04,0.53±0.06。(2)CMRI测得的室间隔厚度,左室后壁厚度,左室舒张末期内径,左室收缩末期内径及主肺动脉干管径分别为(9.8±0.8)mm,(8.1±0.9)mm,(38.7±1.3)mm,(27.6±1.9)mm,(18.9±1.1)mm,CUS测得的相应值分别为(9.3±0.5)mm,(9.1±0.9)mm,(36.7±1.3)mm,(24.3±2.5)mm,(16.3±1.2)mm,CMRI测定的相应值均高于CUS测得的相应值,但两种技术下测得的结果具有良好相关性(r依次为0.976、0.860、0.849、0.699和0.720,均有P<0.05)。结论:CMRI是无创性评价心脏左右心室形态及功能的检查方法。