Secondary Cardiac Lymphoma Presenting as Sick Sinus Syndrome and Atrial Fibrillation Which Required Leadless Pacemaker Implantation

Cardiac involvement of malignant lymphoma is relatively common, although such a phenomenon has subclinical manifestations that are difficult to detect. We herein describe a patient with atrial fibrillation and sick sinus syndrome as the main symptoms. Computed tomography showed a mass in the right atrium extending into the superior vena cava (SVC). We implanted the patient with a leadless pacemaker. Transvenous biopsy revealed a diffuse large B-cell lymphoma. The patient was treated successfully with chemotherapy including rituximab. This case suggested that cardiac lymphoma may cause sick sinus syndrome, and leadless pacemaker implantation is a safe treatment option in patients with partial SVC obstruction.     

抗胆碱药联合益血生治疗病窦综合征疗效观察

目的评估抗胆碱药并益血生对病窦综合征病人的用药及疗效.方法全部病例经EKG、DCE、阿托品试验,部分经食道心房调搏等多项检查确诊.观察组34例口服阿托品及益血生胶囊,依用药方式不同又分为A、B、C组.结果观察组显效率为35.3%,对照组为29.0%;观察组总有效率为79.5%,对照组为64.5%(P＜0.05),其中观察组中B组、C组的总有效率较高,与对照组比较有统计学意义(P＜0.01).结论抗胆碱药山莨菪碱(654-2)与益血生联合治疗病窦综合征疗效确切、安全,不良反应少.     

病态窦房结综合征的临床研究概况

探讨导致病态窦房结综合征的作用机制,并且就中西医对病态窦房结综合征的病因、发病机制、治疗方法、用药、药理等临床研究进展进行综述.     

Mutation‐Specific Effects of Polymorphism H558R in SCN5A‐Related Sick Sinus Syndrome

Effects of H558R on SSS‐Related Mutant Channels. Introduction: Mutations in SCN5A, the gene encoding α subunit of cardiac type sodium channel, Na_v1.5, lead to familial sick sinus syndrome (SSS). Although several molecular mechanisms for this genetic condition have been explored, the underlying mechanisms for the variable genotype–phenotype relationships have not been well addressed. One of the important contributors to such relationships is the genetic background such as single‐nucleotide polymorphisms. Methods and Results: To clarify the effects of a common polymorphism in SCN5A gene, H558R, on SCN5A‐related SSS phenotype, we investigated the electrophysiological properties of all of the 13 known SSS‐related hNa_v1.5 mutant channels on both H558 and R558 background. Electrophysiological properties of hNa_v1.5 mutant channels were investigated by the whole‐cell patch clamp technique in HEK293 cells. When peak currents were affected by the mutation, cell surface biotinylation was performed to quantify the fraction of correctly cell membrane‐targeted mutant channels. Loss‐of‐function defect of D1275N in SCN5A was rescued by R558 through enhancing cell surface targeting and improving steady‐state activation of the mutant channels. In contrast, the defects of mutants E161K, P1298L, and R1632H were aggravated in the R558 background, mainly due to the reduced steady‐state availability of mutant channels. The electrophysiological properties of the remaining SSS‐related hNa_v1.5 mutants including the missense mutants (L212P, T220I, DelF1617, T187I, R878C, G1408R), and the truncated mutants (W1421X, K1578fs/52, R1623X) were not significantly affected by H558R. Conclusion: We conclude that polymorphism H558R has mutation‐specific effects on SCN5A‐related SSS. Our data highlight the importance of common genetic variants in modulating phenotypes of genetic diseases. (J Cardiovasc Electrophysiol, Vol. 21, pp. 564‐573, May 2010)     

干细胞移植治疗病态窦房结综合征

病态窦房结综合征是指窦房结及其周围组织病变和功能减退而引起一系列心律失常的综合征。传统的治疗方法是植入电子起搏器,随着分子生物学技术的发展,生物起搏为病态窦房结综合征的治疗开辟了一个全新的领域。生物起搏是指利用细胞分子生物学及其相关技术对受损的自律性节律点或特殊传导系统的细胞进行修复或替代,使心脏的起搏和传导功能得以恢复。生物起搏包括基因生物起搏,细胞生物起搏和基因工程干细胞生物起搏。现就干细胞移植用于治疗病态窦房结综合征的研究取得的进展与存在问题做一综述。     

频率适应性起搏器对老年病态窦房结综合征患者生活质量影响的评价

病态窦房结综合征患者往往伴有窦房结变时功能不全。频率适应性起搏器的主要适应证是心脏变时功能不全的病态窦房结综合征患者,现探讨频率适应性起搏器对老年病态窦房结综合征患者生活质量影响的评价。     

Selecting the Appropriate Pacing Mode for Patients with Sick Sinus Syndrome: Evidence from Randomized Clinical Trials

Several observational studies have indicated that selection of pacing mode may be important for the clinical outcome in patients with symptomatic bradycardia, affecting the development of atrial fibrillation (AF), thromboembolism, congestive heart failure, mortality and quality of life. In this paper we present and discuss the most recent data from six randomized trials on mode selection in patients with sick sinus syndrome (SSS). In pacing mode selection, VVI(R) pacing is the least attractive solution, increasing the incidence of AF and-as compared with AAI(R) pacing, also the incidence of heart failure, thromboembolism and death. VVI(R) pacing should not be used as the primary pacing mode in patients with SSS, who haven't chronic AF. AAIR pacing is superior to DDDR pacing, reducing AF and preserving left ventricular function. Single site right ventricular pacing-VVI(R) or DDD(R) mode-causes an abnormal ventricular activation and contraction (called ventricular desynchronization), which results in a reduced left ventricular function. Despite the risk of AV block, we consider AAIR pacing to be the optimal pacing mode for isolated SSS today and an algorithm to select patients for AAIR pacing is suggested. Trials on new pacemaker algorithms minimizing right ventricular pacing as well as trials testing alternative pacing sites and multisite pacing to reduce ventricular desynchronization can be expected within the next years.     

特发性病窦综合征的临床和电生理特点

对６７例特发性病窦和７３例伴有器质性心脏病的病窦病人的临床和电生理进行了对比分析，结果表明特发性病窦多数表现为心动过缓型，有症状者少，快速心律失常发生率低，结外病窦的比例明显高于对照组，ＳＮＲＴ和Ｃ－ＳＮＲＴ明显短于对照组．     

Evaluation of pacemaker telemetry as a diagnostic feature for detecting atrial tachyarrhythmias in patients with sick sinus syndrome.

AIM: The aim of the present study was to validate pacemaker telemetry as a diagnostic feature for detecting atrial tachyarrhythmias (AT) during pacemaker treatment in patients with sick sinus syndrome (SSS). METHODS AND RESULTS: Patients with SSS and bradytachy syndrome (n = 28, 20 women), mean age 71 +/- 10.3 years, were included. The patients were treated with AAIR (n = 14) or DDDR pacing. At a routine follow-up visit pacemaker telemetry was reset and the patients underwent Holter recording for at least 24 h. Episodes of atrial fibrillation (AF) during Holter recording were compared with episodes of AT detected by the pacemaker. Only episodes of AF lasting for at least 1 min during Holter recording were registered. AT detected by the pacemaker telemetry was defined as: an atrial high rate episode with a rate of > or = 220 bpm for > or = 5 min, atrial sensing with a rate of > or = 170 bpm in > or = 5% of total counted beats, mode-switching in > or = 5% of total time recorded or a mode-switching episode of > or = 5 min. Twenty-eight Holter recordings (mean duration 31.5 h, range 20-72 h) were used for evaluation. Ten patients had one or more episodes of AF lasting at least 1 min on their Holter recordings. Nine of these patients had AT detected by their pacemaker telemetry according to our criteria. None of the patients had AT detected by the pacemaker telemetry and not by the Holter recording. The specificity and sensitivity for detection of AT recorded by the pacemaker telemetry in this study was 100% and 90%, respectively. The false-positive rate was 0%. CONCLUSION: Pacemaker telemetry was found to be a reliable tool for detecting AT in patients with SSS.     

病态窦房结综合征患者心脏变时功能不全的临床研究

目的探讨病态窦房结综合征患者心脏的变时功能。方法对60例病态窦房结综合征患者（观察组）和40例正常窦性心律者（对照组）作运动平板试验，测定静息心率、运动时的最大心率、运动时间、代谢当量；计算2级运动时的心率变时性指数（CRI）、运动后1min心率恢复值。结果观察组静息心率、最大心率、CRI明显低于对照组，差异有显著统计学意义（P〈0．01），观察组心率上升幅度、运动时间、代谢当量、运动后1min心率恢复值均低于对照组，差异有统计学意义（P〈O．05）；观察组心脏变时功能不全31例（517％），明显多于对照组4例（10％），差异有显著统计学意义（P〈0．01）。结论病态窦房结综合征患者常发生心脏变时功能不全。     

病窦综合征伴非典型性胸痛患者冠状动脉造影结果分析

探讨病窦综合征患者非典型性胸痛的病因及临床意义。对 9例临床诊断为病窦综合征且需安装心脏永久起搏器、同时伴有非典型胸痛的患者 ,在行心脏永久起搏器置入术的同时行冠状动脉造影术 ,分析冠状动脉病变及左室功能。结果 :9例患者冠状动脉均正常 ,但在心动过缓患者中可见冠状动脉血流速度明显缓慢。部分患者左室明显扩大、左室射血分数及室壁运动降低。术后诊断 :单纯病窦综合征 4例 ,病窦综合征合并扩张型心肌病 4例 ,1例病窦综合征合并退行性瓣膜病。 6例患者在起搏器术后胸痛基本消失 ,另 3例术后胸痛发作次数和程度明显减轻 ,一般不需要特殊治疗。结论 :病窦综合征患者的非典型性胸痛不具有冠状动脉病变基础 ,多与心动过缓所导致的冠状动脉血流缓慢有关 ,在安装起搏器后胸痛多消失。病因诊断应注意在退行性变与扩张型心肌病之间鉴别。     

起搏器置入后新出现起搏器依赖及右室起搏相关心室不同步研究

目的:研究置入起搏器5年以上患者新出现起搏器依赖及右室起搏相关心室不同步的情况。方法:选取置入时为非起搏器依赖且置入起搏器已超过5年患者272例进行随访,测量自身心率,记录心室起搏比例;将起搏器心室频率逐渐降至30次/min,观察30s,无患者自身心律出现,判定为起搏器依赖;将自身心率<50次/min且心室起搏比例>90%者判定为右室起搏相关心室不同步患者,对起搏器依赖及右室起搏相关心室不同步患者提出个别随访方案和更换建议。结果:136例病窦综合征患者中有4例(2.9%)进展为起搏器依赖;116例房室传导阻滞患者中有10例(8.6%)进展为起搏器依赖;全部病例有15例(5.5%)进展为起搏器依赖;有43例自身心率<50次/min且心室起搏比例>90%,占全部病例的15.8%。结论:房室传导阻滞患者较之病窦综合征患者,更容易进展为起搏器依赖患者;置入起搏器5年以上患者中约有5.5%进展为起搏器依赖;置入起搏器5年以上患者中右室起搏相关心室不同步人群比例约15.8%。     

心室起搏管理与精确心室起搏功能减少心室起搏的对照研究

目的比较心室起搏管理（management ventricular pacing,MVP）功能与精确心室起搏（refined ventricular pacing,RVP）功能减少右心室起搏的百分比的差异。方法 50例患者按照随机表1：1分成两组,每组25例,分别为MVP组和RVP组。MVP组为植入美敦力Adapt ADDR01起搏器,术后关闭MVP功能1个月;RVP功能组为植入Vitatron双腔起搏器TA1系列或CA3系列,术后关闭RVP功能1个月。1个月后开启MVP功能或RVP功能,术后1、3、6个月采用起搏器程控仪测试各项起搏参数,比较不同起搏功能下的心室起搏百分比。结果两组3个月后、6个月后心室起搏百分比中位数显著低于同组1个月后,差异均有统计学意义（MVP组：0.20vs.75.30,P〈0.01;0.10vs.75.30,P〈0.01。RVP组：6.00vs.88.40,P〈0.01;26.00vs.88.40,P〈0.01）。术后3个月、6个月MVP组的心室起搏百分比中位数低于RVP组,差异有统计学意义[0.20vs.6.00,P=0.02;0.10vs.26.00,P〈0.01]。结论 MVP功能在减少心室起搏百分比方面优于RVP功能,能够更有效的减少右心室起搏累计百分比。     

Electrophysiological studies in thyrotoxicosis with and without associated sick sinus syndrome

Electrophysiological studies in 13 patients with thyrotoxicosis (5 men and 8 women, aged 17 to 76 years) are reported. Five patients presented with features of sick sinus syndrome (SSS) (Group A) while the remaining 8 patients (Group B) had no detectable cardiovascular abnormality. Sinus node function (corrected sinus node recovery and sinoatrial conduction time) was abnormal in all Group A but normal in Group B patients. Intra-atrial, artioventricular (AV) nodal, and infranodal conduction time and effective refractory period of atrium were normal in all patients in both groups. Effective refractory period of AV node was decreased in 6 patients (3 in each group). All Group A patients received radioiodine with complete clinical remission of sick sinus state in 4 subjects. Repeat electrophysiological studies in two of these patients, 6 and 12 months after treatment, showed complete normalization of sinus node function. This is the first reported electrophysiological study documenting the occurrence of SSS in thyrotoxicosis reversed by effective antithyroid treatment. We suggest that attempts should be made to identify underlying thyrotoxicosis in all patients with SSS, especially in the older age group. Appropriate medical treatment may prevent unnecessary implantation of permanent pacemakers in such patients.     

病态窦房结综合征的功能检查价值评估及预后

目的：探讨病态窦房结综合征（ＳＳＳ）功能检查的价值及治疗方法对预后的影响。方法：９２例ＳＳＳ患者（其中随访６８例）按动态心电图表现分４型，分析功能检查（动态心电图、经食管心房调搏、阿托品试验、固有心率）对各型的临床意义。结果：动态心电图对发作期ＩＩ、ＩＩ、ＩＶ型具有诊断价值。黑或晕厥患者的长间歇时间（３．７７±１．４４秒）显著长于无症状或症状轻者（２．５８±０．３７秒）（Ｐ＜０．００１）；ＩＶ型出现黑或晕厥的发生率较其他各型高（Ｐ＜０．０１）；Ｉ型患者的窦房结恢复时间稍低于其他型（Ｐ＜０．０５）。固有心率测定较阿托品试验更可靠。结论：应根据ＳＳＳ各型特点有目的的选择功能检查。ＩＩ、ＩＶ型患者起搏治疗可减少猝死率。     

Atrial pacing in the management of sick sinus syndrome: Long-term observation for conduction disturbances and supraventricular tachyarrhythmias

Atrial pacing (AAI) in sick sinus syndrome (SSS) has been questionedbecause of the risk of distal conduction disturbances (DCD)and atrial tachyarrhythmias. The authors studied the incidenceof clinically relevant DCD and arrhythmias in 52 SSS patientswith AAI. The observation time was 25–67 (mean 48) months. Invasive electrophysiologic investigation was performed preoperativelyin 29 cases and preoperative atrial pacing to Wenckebach blockor to 150 ppm in 23. The preoperative investigation showed prolongedHV-time in three cases and Wenckebach block at 110ppm in onecase, while six patients had LAH-block, five RBB-block and twofirst degree A V-block. No further DCD developed in any of thesepatients. Second-degree type IA V-block developed in two patientsafter 2 and 19 months, respectively. One had been treated withdigitalis and verapamil and was asymptomatic. The other patient,who had a low ventricular rate, experienced near-syncope. Thesetwo blocks could not be predicted from the preoperative andperoperative investigations. In five patients (10%) permanent atrial fibrillation developedduring the follow-up period. The incidence of concomitant supraventriculartachyarrhythmias in the patients with the bradycardia-tachycardiasyndrome appeared to be unaffected by the pacemaker treatment.