1例成功筛选右心室致心律失常心肌病患者的护理

正2011年美国心律协会(HRS)和欧洲心律协会(EHRA)正式提出致心律失常心肌病(arrhythmogenic ventricular cardiomyopathy,AVC)的定义[1],将AVC分为三个亚型,右心室型(ARVC),左右心室型和右心室型(LDAC)[2]。近年在一次多中心研究[3]中对50例发生心脏性猝死、心室有纤维脂肪浸润的心肌病患者进行尸检,发现累及不同心室的AVC在不同种族中有显著性差     

致心律失常性右心室心肌病的诊断现状

致心律失常性右心室心肌病（arrhythmogenic right ventricular cardiomyopathy,ARVC）是临床最常见可能导致患者猝死的器质性心脏病。ARVC病变主要累及右心室，并以右心室部分心肌被纤维和（或）脂肪组织替代、右心室扩大、室壁变薄和发生单个或多个室壁瘤为主要表现。     

致心律失常性右心室心肌病1例报告

1引言 致心律失常性右心室心肌病(ARVC)是以病变累及右心室为特征,以右心室性心律失常为突出表现的一种疾病,临床较为少见,现报道1例如下. 2病例报告     

原发性肺动脉高压并致心律失常性右室心肌病漏诊一例

致心律失常性右室心肌病(arrythmogenic right ventricular cardiomyopathy,ARVC)是一种特殊类型的心肌病,又称致心律失常性右心室发育不良、羊皮纸心,1995年WHO命名为致心律失常性右室心肌病,并将其增列为与扩张性、肥厚性、限制性心肌病并列的第四类心肌病。ARVC病因尚不明确,也无根治方法,是导致年轻人猝死的主要原因之一。近年我院收治1例原发性肺动脉高压合并ARVC,临床少见,现报告如下。     

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…     

致心律失常性右室心肌病心电图表现研究进展

致心律失常性右室心肌病(Arrhythmogenic right ventricu-lar cardiomyopathy,ARVC)是一种罕见遗传性心肌病,以右室心肌细胞逐渐被脂肪和纤维组织代替[1,2],造成右心室壁变薄,心室腔扩大,患者以心律失常表现为主,包括室早、持续性和非持续性的室速。病变可累及双侧心室,导致心功能不全[3-5],     

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.     

致心律失常性右室心肌病的磁共振诊断价值及研究进展

致心律失常性右室心肌病(arrhythmogenic right ventricular cardiomyopathy,ARVC)是一种以脂肪或纤维脂肪进行性替代右室心肌为特征的遗传性心肌病,是青年人和运动员心源性猝死的常见原因。该病不单局限于右室,部分患者左室也有不同程度的受累。目前,ARVC的诊断基于标准修订版(revised task force criteria,rTFC)的多学科综合评估。心脏磁共振是一种理想的、非侵入性的成像技术,它提供了心脏组织学、功能及形态学等信息,在ARVC的早期诊断和定性、定量分析中发挥着重要作用。笔者就心脏磁共振在ARVC的诊断价值及研究进展予以综述。     

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].     

致心律失常右室心肌病的心电学诊断

致心律失常性右室心肌病(ARVC),又称致心律失常性右室发育不良/心肌病(ARVD/C),目前已经明确其是一种遗传性疾病,至少50%的病例表现为典型的常染色体显性遗传,也有常染色体隐性遗传的报道。2006年新颁布的心肌病分类将ARVC归属于遗传性原发性心肌病。根据临床研究和参加体育运动前的筛查资料,估计ARVC在一般人群的患病率为1/1 000～1/5 000。发病年龄80%在40岁之前,男性占     

Factors Predisposing to the Development of Atrial Fibrillation

Atrial fibrillation (AF) is in most patients (approximately 70%) associated with organic heart disease including valvular heart disease, coronary artery disease, hypertension, hypertrophic cardiomyopathy, dilated cardiomyopathy, and congenital heart disease, mostly atrial septal defect in adults. In many chronic conditions, determining whether AF is the result or is unrelated to the underlying heart disease, remains unclear. The list of possible etiologies also include cardiac amyloidosis, hemochromatosis and endomyocardial fibrosis. Other heart diseases, such as mitral valve prolapse (without mitral regurgitation), calcifications of the mitral annulus, atrial myxoma, pheochomocytoma, and idiopathic dilated right atrium may present with AF. Atrial fibrillation may occur in the absence of detectable organic heart disease, the so-called “lone AF”, in about 30% of cases. The term “idiopathic AF” implies the absence of any detectable etiology including hyperthyroidism, chronic obstructive lung disease, overt sinus node dysfunction, and overt or concealed preexcitation (Wolff-Parkinson-White syndrome), only to mention a few of other uncommon causes of AF. The autonomous nervous system may contribute to the occurrence of AF in some patients. AF occurs commonly. In patients with valvular heart disease, AF is common, particularly when the mitral valve is involved. The occurrence of AF is unrelated to the severity of mitral stenosis or mitral regurgitation but is more common in patients with enlarged left atrium and congestive heart failure. In patients with coronary artery disease, AF occurs predominantly in older patients, males, and patients with left ventricular dysfunction. Important predictive factors of AF include hypertension, left ventricular hypertrophy and diabetes. The risk of the development of AF, in an individual patient, is often difficult to assess. Increasing age, presence of valvular heart disease, and congestive heart failure increase the risk of atrial fibrillation.     

Performance of temporary epicardial stainless steel wire electrodes used to treat atrial fibrillation: a study in patients following open heart surgery

AF is the most common arrhythmia following open heart surgery. Transthoracic cardioversion is used when pharmacological treatment fails to restore SR, or is ineffective in controlling ventricular response rate. We report on the performance of temporary atrial defibrillation wire electrodes implanted on the epicardium of patients undergoing open heart surgery. Epicardial stainless steel wire electrodes for both pacing/sensing and atrial defibrillation were placed at the left and right atrium during open heart surgery in 100 consecutive patients (age 65 +/- 9 years; male/female 77/23). Electrophysiological studies performed postoperatively revealed a test shock (0.3 J) impedance of 96 +/- 12 omega (monophasic) and 97 +/- 13 omega (biphasic). AF was induced by burst stimulation in 84 patients. All patients were successfully converted to SR. The mean energy of successful shocks was 3.1 +/- 1.9 J. Atrial pacing and sensing were accomplished in all patients. Pacing threshold was 1.9 +/- 1.7 V (0.5 ms) in the left atrium and 2.1 +/- 2 V in the right atrium. P wave sensing was 2.5 +/- 1.6 mV in the left atrium and 2.3 +/- 1.4 mV in the right atrium. No complications were observed with shock application, nor with lead extraction. Atrial defibrillation using temporary epicardial wire electrodes can be performed successfully and safely in patients following cardiac operations. The shock energy required to restore SR is low. Electrical cardioversion in the absence of anesthesia should be feasible.     

Right atrial spontaneous echo contrast and thrombi in atrial fibrillation: a transesophageal echocardiography study.

BACKGROUND: Previous studies have reported the clinical and echocardiographic findings of patients with left atrial spontaneous echo contrast (SEC) and thrombi. We sought to study these characteristics in patients with right atrial SEC and thrombi. METHODS: We reviewed 580 consecutive patients from the ACUTE (Assessment of Cardioversion Using Transesophageal Echocardiography) Registry and found 79 patients (14%, aged 67 +/-13 years, 67 male) with transesophageal echocardiography (TEE) findings of right atrial SEC or thrombi (group 1). This group was compared with a control group of 75 consecutive patients (group 2) (aged 68 +/- 13 years, P = not significant; 49 male, P <.005) from the registry with no TEE findings of SEC or thrombi in the left or right atrium. RESULTS: Atrial fibrillation was present in 60 of 79 group 1 patients (76%). Five right atrial (6%) and 11 left atrial (14%) thrombi were identified. Both left ventricular ejection fraction (39% +/- 16% versus 47% +/- 14%; P =.0005) and presence of right ventricular dysfunction (n = 44 versus 18; P =.0001) differed significantly between groups 1 and 2, respectively. Right atrial area (24 +/- 6 cm(2) versus 22 +/- 6 cm(2); P = .02) was larger in patients in group 1. Left atrial SEC was present in 68 of 79 group 1 patients (86%). Patients with right atrial thrombi and right atrial SEC had a longer duration of arrhythmia (524 +/-812 days versus 147 +/-368 days, P <.05) than patients with right atrial SEC only. CONCLUSIONS: Right atrial SEC has a prevalence of 14% in patients with atrial arrhythmia who undergo TEE-guided cardioversion. Right atrial thrombi are a rare finding and were seen in fewer than 1% (5/580) of patients with atrial arrhythmia. Right atrial thrombi among patients on anticoagulation therapy were not associated with clinically significant pulmonary embolism.     

Arrhythmogenic right ventricular cardiomyopathy/dysplasia: a review and update

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a predominantly genetically determined and heritable form of cardiomyopathy that is characterized pathologically by the replacement of myocytes by adipose and fibrous tissue and leads to right ventricular failure, arrhythmias, and sudden cardiac death. The estimated prevalence of ARVC/D in the general population ranges from 1 in 2,000 to 1 in 5,000, men are more frequently affected than women, with an approximate ratio of 3:1. ARVC/D can be inherited as an autosomal dominant disease with reduced penetrance and variable expression, autosomal recessive inheritance is also described. There have been 12 genes identified which are linked to ARVC/D, encoding several components of the cardiac desmosome. Dysfunctional desmosomes resulting in defective cell adhesion proteins, such as plakoglobin (JUP), desmoplakin (DSP), plakophilin-2 (PKP-2), and desmoglein-2 (DSG-2) consequently cause loss of electrical coupling between cardiac myocytes, leading to myocyte cell death, fibrofatty replacement and arrhythmias. Diagnosis is based on the finding a combination of characteristic abnormalities in family history, electrocardiography, cardiac imaging as well as endomyocardial biopsy (original task force criteria). Therapeutic options remain limited because of the progressive nature of ARVC/D. Competitive athletics should be avoided. Patients with ARVC/D with a history of having been resuscitated from sudden cardiac death, patients with syncope, very young patients, and those who have marked right ventricular involvement are at the highest risk for arrhythmic death and also, the presence of left ventricular involvement is a risk factor. Several authors concluded that patients who meet the Task Force criteria for ARVC/D are at high risk for sudden cardiac death and should undergo ICD placement for primary and secondary prevention, regardless of electrophysiologic testing results. The role of electrophysiologic study and VT catheter ablation in ARVC/D remains poorly defined, and is frequently used as a palliative measure for patients with refractory VT. The progressive nature of ARVC/D suggests that catheter ablation would not be a long-term curative procedure. Sotalol proved to be highly effective in patients with ARVC/D and inducible as well as non-inducible ventricular tachycardia; if it is ineffective in inducible ventricular tachycardia response to other antiarrhythmic drugs is unlikely and therefore non-pharmacological therapy without further drug testing should be considered. Orthotopic heart transplantation is considered in patients with progressive heart failure and intractable recurrent ventricular arrhythmias     

Effects of Systolic Atrial Function on Plasma Renin Activity and Natriuretic Peptide Secretion after High Rate Atrial and Ventricular Pacing in Dogs

Rapid atrial rates cause electrical, structural remodeling, and neuro-humoral changes. This study compares the effects of mechanical remodeling on plasma renin activity (PRA) and atrial natriuretic peptide (ANP) secretion. Eight beagles were subjected to rapid atrial pacing (AP) at 400 beats/min for 16 days. After complete recovery of left ventricular function, they underwent rapid ventricular pacing (VP) at 240 beats/min of equal duration. Left atrial systolic maximal dimension (LAmax) and left atrial appendage (LAA) peak late emptying velocity (LAA-E) were assessed by echocardiography. Blood samples were taken from the right atrium and from the peripheral vein. LAmax after AP and VP enlarged significantly (2.16 ± 0.21 cm vs 2.41 ± 0.23 cm, P = 0.002). Compared with baseline, LAA-E velocities were significantly reduced (0.65 ± 0.12 m/s vs 0.26 ± 0.16 m/s, P = 0.001) after AP only. AP caused a significant elevation of PRA in right atrial (9.28 ± 4.23 nmol/L per hour) and peripheral samples compared with baseline values (4.82 ± 2.53 nmol/L per hour, P = 0.04). ANP levels increased after AP (1117.12 ± 252.21 fmol/L) with respect to baseline values (824.37 ± 159.08 fmol/L, P = 0.001). There was no difference in PRA and ANP levels between atrial and peripheral samples. Atrial size and impaired systolic appendage function play an important role in secretion of PRA and ANP. Both neuro-humoral pathways may be therapeutic targets in the treatment of patients with AF.     

Atrial Epicardial Pacing with Long Stimulus to P Wave Interval in a Patient with Arrhythmogenic Right Ventricular Dysplasia Complicated by Right Atrial Thrombosis

Atrial epicardial pacing with a long stimulus to P wave interval in a patient with arrhythmogenic right ventricular dysplasia complicated by right atrial thrombosis is discussed. Arrhythmogenic right ventricular dysplasia (ARVD) is associated with a high incidence of malignant ventricular arrhythmias. Most patients with ARVD need antiarrhythmic drugs, catheter ablation, or an implantable cardioverter defibrillator. We report a patient with ARVD in whom effective treatment with sotalol caused severe, symptomatic sinus bradycardia requiring permanent pacing. Due to leftward displacement of the right ventricle and the presence of two thrombi in the right atrium, an epicardial atrial lead and AAI pacemaker were implanted. A long stimulus to P wave interval caused by severe dilatation of the right atrium was recorded. During a 6 months of follow-up on sotalol treatment there were neither ventricular tachycardia (VT) attacks nor pacing problems.     

Atrial Pacing Lead Location Alters the Hemodynamic Effects of Atrial-Ventricular Delay in Dogs with Pacing Induced Cardiomyopathy

HETTRICK, D.A., et al .: Atrial Pacing Lead Location Alters the Hemodynamic Effects of Atrial Ventricular Delay in Dogs with Pacing Induced Cardiomyopathy. The role of atrial lead location in cardiovascular function in the presence of impaired ventricular dysfunction is unknown. We tested the hypothesis that left atrial (LA) and left ventricular (LV) hemodynamics are affected by alterations in AV delay and are influenced by atrial pacing site in dogs with dilated cardiomyopathy. Dogs   (n = 7)   were chronically paced at 220 beats/min for 3 weeks to produce cardiomyopathy and then instrumented for measurement of LA, LV end diastolic pressure (LVEDP) and mean arterial pressure (MAP), LA volume, LV short-axis diameter, and aortic and pulmonary venous blood flow. Hemodynamics were measured after instrumentation and during atrial overdrive pacing from the right atrial appendage (RAA), coronary sinus ostium (CSO) and lower LA lateral wall (LAW). The AV node was then ablated, and hemodynamics were compared during dual chamber AV pacing (right ventricular apex) from each atrial lead location at several AV delays between 20 and 350 ms. Atrial overdrive pacing from different sites did not alter hemodynamics. Cardiac output (CO), stroke volume, LVEDP, MAP and +dLVP/dt demonstrated significant (P < 0.05) variation with AV delay during dual chamber pacing. CO was higher during LAW pacing than RAA and CSO pacing (   2.3 ± 0.4   vs   2.1 ± 0.3   vs   2.0 ± 0.3 l/min   , respectively) at an AV delay of 120 ms. Also, MAP was higher in the LAW than RAA and CSO (   65 ± 9   vs   59 ± 9   vs   54 ± 11 mmHg   , respectively) at an AV delay of 350 ms. Atrial lead location affects indices of LV performance independent of AV delay during dual chamber pacing in dogs with cardiomyopathy. (PACE 2003; 26[Pt. I]:853–861)     

Successful Radiofrequency Catheter Ablation of Automatic Atrial Tachycardia with Regression of the Cardiomyopathy Picture

Ectopic atrial tachycardia (EAT) is often refractory to pharmacological suppression, and if uncontrolled, it can lead to cardiomyopathy. Although RF current catheter ablation therapy has been effective in eliminating the arrhythmia, there is limited information. particularly in adult patients with regard to the reversal of the tachycardia induced cardiomyopathy. Four adult patients, 20–56 years of age, and a 6-year-old boy, were referred with refractory EAT. Four patients had heart failure and three had depressed LV function by echocardiographic criteria. AH patients underwent electrophysiological study, and RF ablation was successful in abolishing the arrhythmogenic foci. Of these, four were located in the right atrium and one in the left atrium, and were identified by recording of the earliest atrial activation. No complications occurred. Termination of the EAT resulted in symptomatic improvement. Serial echocardiographic assessment of LV function indicated a significant reversal of the cardiomyopathy picture with reduction in chamber size and recovery in systolic function; indices of diastolic dysfunction persisted in one patient. Chronic, uncontrolled EAT can cause tachycardia induced cardiomyopathy. The picture of the cardiomyopathy resolves after elimination of the focus. RF ablation is both effective and safe, and may be considered as early therapy, particularly in patients with incessant EAT and ventricular dysfunction.     

组织多普勒成像技术评价阵发性房颤心房收缩和电-机械时间的变化

目的：采用组织多普勒成像技术(TDl)，观察阵发性房颤(PAF)患者左、右心房和房室环处心房收缩时间(A)和电—机械时间(P—A)的变化。方法：PAF组61例，正常组32例，取心尖四腔观，将TDI取样容积分别置于室间隔房室环、左室侧壁二尖瓣环和右室侧壁三尖瓣环处，分别记录各点的的运动频谱，测量不同部位P—A和A值。结果：与对照组比较，PAF组左、右心房径明显增大；各部位的P—A、A均显著延长；P—A、A与左、右心房径之间无明显相关，而与房颤持续时间、病史及年龄呈相关性，线性回归分析显示房颤持续时间是P—A的主要影响因素。结论：阵发性房颤患者心房收缩、电—机械时间显著延长，且与左房、右房扩大无关，而可能取决于房颤事件的持续时间。     

Atrial Pressure and Experimental Atrial Fibrillation

SIDERIS, D.A., et al .: Atrial Pressure and Experimental Atrial Fibrillation . A possible profibrillatory effect on the atria of an elevated atrial pressure and the site of atrial stimulation was examined. In 15 anesthetized dogs, right or left atrial or biatrial pacing was applied at a high rate (300–600/min) for 5 seconds at double threshold intensity under a wide range of atrial pressures achieved by venous or arterial transfusion or bleeding. Induction of atrial fibrillation in 236 of 1,971 pacing runs was associated with a significantly higher (P < 0.001) atrial pressure (21.6 ± 12.2 mmHg, mean ± SD) than maintenance of sinus rhythm (16.8 ± 11.1 mmHg in 1,735 of 1,971 pacing runs). Stimulation of the right atrium resulted in atrial fibrillation more frequently than left atrial or biatrial stimulation, with biatrial stimulation less frequent than right or left atrial stimulation. The induction of atrial fibrillation was related to the atrial pressure and to the site of stimulation but not to the pacing rate or the prepacing heart rate. The prepacing heart rate, associated with failure to induce sustained atrial fibrillation, was higher than that associated with atrial fibrillation in 12 of 15 experiments (significantly in 6) and not significantly lower in 3 of 15. Atrial fibrillation lasting 1 minute or more was more frequently associated with simultaneous stimulation of both atria than of either atrium alone. Thus, an elevated atrial pressure may facilitate the induction of atrial fibrillation. The site of stimulation also plays an important role for both the induction and maintenance of atrial fibrillation in this model.