先天性QT间期延长综合征猝死的临床分析

1988年1月～1995年6月我们对四个家系和7例散发型共43例先天性QT间期延长综合征（CLQTS）进行调查，7例摔死，2例生前已明确诊断，服用且受体阻滞剂有效，因自行减量或温服摔死。另5例在家系调查中发现，首次发病即摔死。临床资料例1，女。35岁，1983～1987年反复曼厥数十次，记录到的心电图为尖端扭转性室速（TPd），电复律和右心室起搏超速抑制能终止急性发作。诱因：1．体育活动；2．情绪激动；3．月经来潮；4．见血；5疼痛。窦律时QT。0．33～0．38秒。超声心动图正常。多次血钾检查正常，听力正常。两次心内电生理检查均未诱发室…     

慢性心力衰竭心脏再同步治疗超级反应患者的临床特征

目的接受心脏再同步治疗（CRT）的慢性心力衰竭患者中,部分患者心脏结构和功能显著改善,甚至可以恢复至接近正常,即所谓的超级反应患者（super－responders,SR）,本文回顾分析这些超级反应患者的临床特征,寻找有价值的CRT超级反应患者的预测指标。方法2005年6月至2010年1月在本中心因慢性心力衰竭植入CRT·P／CRT—D的患者共69例,其中有较完整随访资料的50例纳入本研究。CRT超级反应患者判断标准为左心室射血分数（LVEF）绝对值增加≥0．20。回顾性分析50例患者中超级反应患者和非超级反应患者的临床特征,比较两组患者之间的差异。结果平均随访时间（13．9±11．5）个月,达到超级反应标准患者11例（22％）,与术前相比LVEF显著升高（0．58±0．05对0．33±0．05,P〈0．001）。超级反应组和非超级反应组术前资料除性别（女性比例：8／11对11／39,P=0．013）、QRS时限[（163．2±19．3）ms对（140．2±29．1）ms,P=0．018]、左束支阻滞的比例（10／11对20／39,P＝0．04）外差异无统计学意义。多元logistic回归分析显示女性合并完全性左束支阻滞是CRT超级反应的惟一独立预测因子。结论CRT中超级反应患者以女性、QRS时限增宽以及合并左束支阻滞患者多见。女性合并左束支阻滞是CRT超级反应的独立预测因子。     

阵发性心房颤动环肺静脉隔离术后心率变异性时域指标半年随访观察

目的观察环肺静脉口部消融隔离治疗阵发性心房颤动（AF）术后心率及心率变异性（HRV）时域指标的长期变化。方法共45例行射频消融术的阵发性AF患者,术前、术后第3天、术后1、3、6个月在本院行24h动态心电图检查。分别测定平均心率（meanHR）,时域指标RR间期标准差（SDNN）,RR间期平均值的标准差（SDANN）,相邻RR间期差的均方根（RMMSD）,相邻RR间期差值超过50ms的RR间期所占百分数（PNN50）。结果共33例患者的动态心电图资料符合要求而纳入结果分析。其中男23例,女10例,年龄（59±10）岁,左心房直径（35±5）mm,左心室射血分数0．63±0．06。术后3d,1、3、6个月meanHR较术前增快,差异有统计学意义。而HRV时域指标均出现减弱,部分指标如SDNN、SDANN在6个月时其变化仍然有统计学意义。是否进行右心房峡部消融对心率及HRV指标变化无影响。术中有无明显“去迷走神经”反射者之间差异也无统计学意义。结论环肺静脉口部消融隔离术后平均心率增快,部分HRV时域指标减弱至少维持6个月。这种对自主神经系统的损伤作用可能是射频消融治疗AF的机制之一。     

心脏再同步治疗难治性心力衰竭的临床应用

目的评价心脏再同步治疗（CRT）难治性心力衰竭的临床疗效。方法21例难治性心力衰竭患者接受CRT治疗;左心室导线采用经心脏静脉血管径路植入或开胸直接缝合左心室心外膜导线。植入术后定期随访,分析患者心功能、活动耐量、生活质量评分、QRS时限、左心室射血分数（LVEF）、左心室舒张末内径（LVEDD）等指标的变化,评价CRT的临床疗效,测试起搏参数并在超声心动图指导下优化AV间期和VV间期。结果21例患者成功植入CRT,其中3例患者因心脏静脉血管畸形而采用开胸植入左心室心外膜导线。平均随访（9．8±9．5）个月,死亡3例;2例患者因心力衰竭症状复发再住院;其余患者心功能分级、生活质量评分、活动耐量较植入术前有显著改善;植入术后1个月LVEF明显提高（0．31±0．06对0．37±0．05,P〈0．01）;二尖瓣反流程度[（2．24±0．89）级对（1．43±0．75）级,P〈0．01];LVEDD植入术后6个月明显减小（（71．91±8．38）mm对（68．82±10．80）mm,P〈0．05];QRS时限分析显示CRT治疗有效组术后明显变窄（（162．50±24．08）ms对（142．50±19．15）ms,P〈0．05],无效组变化不明显[（148．00±10．95）ms对（138．00±4．47）ms,P〉0．05]。1例患者左心室导线脱位。右心房和右心室起搏导线的阈值、感知灵敏度及阻抗均在正常范围,左心室导线各参数,除1例患者阈值升高外余均正常。3例开胸植入心外膜导线患者除1例于植入术后第5个月心脏性猝死,余2例分别随访18个月和26个月,左心室导线阈值分别为0．5V／0．4ms和1．0V／0．4ms。CRT治疗的总临床有效率为76．2％。结论CRT治疗难治性心力衰竭有效,能恢复患者心脏电活动和机械活动的同步性,显著改善心功能。QRS时限变窄是CRT疗效的重要预测指标。     

血清D-二聚体在急性主动脉夹层诊断价值的Meta分析

目的 评估血清D-二聚体(DD)诊断急性主动脉夹层(AAD)的灵敏度和特异度.方法 使用aortic、dissection和D-dimer三个关键词全面检索英文数据库.采用MS Excel 2003、Review Manager 5.1、STATA 12和Meta DiSc 1.4进行数据管理、计算和统计分析.结果 最终共纳入9个研究,包括532例AAD患者和780例健康对照者.DD临界值取500 ng/ml时,综合灵敏度、特异度及其95％可信区间分别为0.97[0.94-0.98]、0.61[0.53-0.69].结论 DD可作为排除AAD的血清学指标.若DD水平小于500 ng/ml,对于非高危患者,则无需行进一步检查.     

单纯碎裂电位指导心房颤动消融的初步临床观察

Objective To evaluate the feasibility of mdiofrequency catheter ablation of atrial fibrilla-tion (AF) guided by complex fractionated atrial electrograms (CFAEs). Methods Twenty-two patients with drug refractory and symptomatic AF(16 paroxysmal, 6 persisten) were enrolled. Using Carto, the left atrial or biatrial replica was created during spontaneous or induced AF, and areas associated with CFAEs were identi-fied. Radiofrequency ablation at the site with CFAEs was performed and the end points were to eliminate CFAEs or convert to sinus rhythm. Results Thirteen patients(59%)were converted to sinus rhythm, (7 cases conver-ted directly to sinus rhythm, and 6 via the intermediate atrial tachycardia(AT) or atrial flutter (AFL). The re-maining nine patients required cardioversion with D. C. shock or drug. Repeat ablation was performed in 6 pa-tients (5 AT/AFL, 1 paroxysmal AF). During(10.9 ±4.8) months follow-up, 16 patients (73%) were free of arrhythmia and symptoms. CFAEs were most commonly found along the left interatrial septum, pulmonary veins, left atrial roof. CFAEs ablation prolonged AFCL[(157 ± 18) ms vs (211 ± 32) ms, P < 0.05]. Only one patient had pericardial tamponade that required pericardiocentesis. Conclusion Radiofrequeney catheter abla-tion of atrial fibrillation (AF) guided by CFAEs is safe and effective.     

78例特发性右室流出道室速的射频导管消融和随访

目的 研究特发性右室室速射频导管消融的安全性和有效性。方法 78例发作时呈左束支阻滞图形伴电轴下偏的特发性右室流出道室速进行射频导管消融和随访,采用起搏标侧法,最佳起搏标测点（起搏时12导联心电图与自然发作室速的12导联心电图相同）为消融靶点。结果 78例中73例（93.5%)消融成功,消融靶点位于前,中和后间隔分别为33%、41%和26%。临床和电生理研究发现,特发性右室室速有两种类型,即室早室速型和单纯室速型;室早室速型43例,单纯室速型35例。随访0.5-7年,3例复发,除1例右束支传导阻滞外,无手术相关并发症。结论 射频消融治疗右室特发性室性心律失常安全有效。     

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.     

心脏再同步化治疗后血浆NT-ProBNP水平变化观察

目的:研究心脏再同步化治疗(cardiac resynchronization therapy,CRT)前后血浆前体脑钠肽(NT-ProBNP)水平的变化是否能够反映CRT植入后心衰患者心脏结构和功能改善程度。方法:对本中心33例植入CRT/CRT-D患者的随访资料进行分析,随访时间均>6个月,根据治疗效果将患者分为有反应组和无反应组,有反应者定义为术后左室收缩末容积缩小≥15%,心功能分级NYHA下降≥1级;无反应者为术后左室收缩末容积缩小<15%,NYHA下降<1级、因心衰再入院或心源性死亡。结果:有反应组术后NT-ProBNP水平明显降低[(2.90±0.57)ng/L vs(3.38±0.47)ng/L,P=0.001]。随访时间≥6个月时,NT-ProBNP水平的降低幅度(ΔNT-ProBNP%)与左室舒张末容积缩小幅度(ΔLVEDV%)呈线性回归(b=-0.499,R2=0.489,P=0.001);无反应组术后NT-ProBNP与术前相比无显著性差别[(3.53±0.42)ng/L,P>0.05];NT-ProBNP水平的变化与ΔLVEDV%无线性回归关系(P>0.05)。结论:CRT/CRT-...     

短QT综合征

短QT综合征（short QT syndrome,SQTS）是一种以体表心电图QT间期明显缩短为特点,伴或不伴各种房性、室性心律失常,有高猝死风险的心脏离子通道病,自2000年Gussak等提出短QT综合征的概念以来,对该病的认识不断深入。现就近年短QT综合征的研究进展综述如下。