病毒性心肌炎及扩张型心肌病患者心内膜心肌活检标本中免疫球蛋白的检测

对病理学确诊的25例病毒性心肌炎（VMC）和10例扩张型心肌病（DCM）患者心内膜心肌活检标本，运用ABC技术，进行了免疫球蛋白IgG、IgM、IgA和补体C3的检测。结果：20例VMC和9例DCM的标本中，发现了IgG和IgM的沉积，主要分布于心肌肌膜和毛细血管内皮，IgG的肌膜沉积与同步做的病理切片中观察到的心肌细胞坏死和炎性细胞浸润有病理形态学联系。两组患者中均未发现IgA和C3沉积。结果显示，IgG是参与VMC和DCM心肌损伤的主要免疫球蛋白，心肌病变与抗体诱导的免疫反应有关。     

同种异体原位心脏移植成功3例报告

目的:总结分析3例同种异体原位心脏移植术的临床经验。方法:采用Shumway&Stanford方法对3例终末期扩张型心肌病病人行同种异体原位心脏移植术。其中1例伴有严重糖尿病和中度肺动脉高压,术前心跳骤停心肺复苏成功;1例极度肥胖,体重108kg(身高172cm);1例62岁高龄伴肾功能不全和中度肺动脉高压。供体均为脑死亡者,供心保护采用4℃改良StThomas液;术后免疫抑制治疗采用环孢素A、骁悉、强的松三联治疗,根据血环孢素浓度及心内膜活检调整环孢素A用量。结果:3例均康复,未发生术后超急性或急性排斥反应。例2术后出现一过性右心功能不全,经大量利尿和无创通气治疗,48h后完全恢复;例3术后并发急性肾功能衰竭和肺部感染,经连续肾脏替代(CRRT)和调整抗生素治疗痊愈。病人心功能均恢复正常。例1需长期胰岛素治疗糖尿病。3例均完全过上了正常人的生活,2例已恢复手术前工作。结论:同种异体原位心脏移植是治疗终末期心脏病的有效方法。     

小剂量倍他乐克治疗晚期扩张性心肌病疗效及安全性观察

目的 :评价小剂量倍他乐克治疗晚期扩张性心肌病 (DCM )的疗效及安全性。方法 :2 0例晚期扩张性心肌病患者 ,经充分抗心衰治疗后 ,按入院顺序 ,随机分为A组 (倍他乐克治疗组 )和B组 (常规治疗组 ) ,比较各组治疗前后及两组间临床指标及生活质量改善情况。结果 :两组临床症状、生活质量均较入院时明显改善。A组左室射血分数(LVEF)、6分钟步行距离 (D)较B组提高 ;心率 (HR) ,心率、平均动脉压 (MBp)乘积较B组下降 ,差异有显著性 (P <0 .0 1) ;A组再住院率、住院日数较B组低 ,所有患者均能良好耐受倍他乐克 ,无心功能恶化。结论 :在充分使用血管紧张素转换酶抑制剂 (ACEI)、利尿剂和洋地黄的基础上 ,采用小剂量倍他乐克治疗晚期扩张性心肌病 ,疗效及安全性均肯定     

Severe constrictive pericarditis as an unsuspected cause of death in a patient with idiopathic hypereosinophilic syndrome and restrictive cardiomyopathy

A 43-year-old man with idiopathic hypereosinophilic syndrome survived a relatively long term (6 1/2 years) before he succumbed to intractable heart failure. Six months before death, his chronic heart failure from restrictive cardiomyopathy was well compensated. Autopsy demonstrated severe constrictive pericarditis which was not suspected antemortem. Constrictive pericarditis as a late complication of idiopathic hypereosinophilic syndrome is discussed.     

~(123)I-MIBG心肌显像预测依那普利对扩张型心肌病患者治疗效果的临床价值

目的探讨123I-MIBG心肌显像在治疗前预测依那普利对扩张型心肌病(DCM)患者治疗效果的临床价值。方法对24例DCM患者于依那普利治疗前行早期(20min)及延迟(3h)123I-MIBG心肌显像,采用心/上纵隔(H/M)比和心脏放射性洗脱率(WR)作为相对半定量计数分析,与超声心功能参数进行对比。根据123I-MI-BG心肌显像延迟相的H/M分为3组延迟H/M≥1.7为组Ⅰ,1.5<延迟H/M<1.7为组Ⅱ,延迟H/M≤1.5为组Ⅲ。组Ⅰ和组Ⅱ在平均治疗4.5个月后重复以上检查。结果治疗前3组间超声心功能参数比较均无统计学差异。治疗后组Ⅰ的左室射血分数(LVEF)和左室收缩末径(LVDs)明显改善,早期H/M和延迟H/M均明显改善(P<0.05),而WR无明显变化。治疗后组Ⅱ的延迟H/M明显改善(P<0.05),而早期H/M和WR均无明显变化,心功能参数也无改善。组Ⅰ和组Ⅱ患者均能耐受依那普利治疗,而组Ⅲ患者均不能耐受依那普利治疗。结论123I-MIBG心肌显像在治疗前预测依那普利对DCM患者的治疗效果方面有一定价值。     

肥厚型心肌病预后评估的研究进展

肥厚型心肌病是最常见的遗传性心脏病,其临床表现及预后极富多样性。该病患者的不良转归主要有:猝死、心力衰竭以及心房颤动所致的栓塞事件等。现就影响肥厚型心肌病预后的因素作一综述。     

Anti-mitochondrial antibodies in patients with dilated cardiomyopathy (anti-M7) are directed against flavoenzymes with covalently bound FAD

Anti-mitochondrial antibodies (anti-M7) in sera from patients with dilated cardiomyopathy and myocarditis recognize, besides mitochondrial antigens, bacterial sarcosine dehydrogenase. The common target antigen was identified as the covalently bound FAD of mitochondrial and bacterial flavoenzymes. Thus, anti-M7-positive serum reacted on Western blots exclusively with covalently flavinylated enzymes. The antigenic specificity of anti-M7 sera was reproduced by an antiserum raised in rabbits with 6-hydroxy- D -nicotine oxidase. The heart mitochondrial membrane antigen recognized by anti-M7 serum was identified as the flavoprotein subunit of succinate dehydrogenase, the antigens in rat liver mitochondrial matrix as the flavoenzymes dimethylglycine dehydrogenase and sarcosine dehydrogenase. Anti-M7 serum contained a specific anti-flavoenzyme antibody fraction. Nanomolar concentrations of FAD and riboflavin inhibited the immune reaction on Western blots and in ELISA, and incubation with FAD-agarose depleted the anti-M7 activity of the serum. N-terminally deleted dimethylglycine dehydrogenase proteins were only immunoprecipitated by anti-M7 sera when the FAD was covalently incorporated. An affinity constant (K_D) of 10^?8 M was established for the anti-flavoenzyme antibodies by competitive ELISA. Of patients with cardiomyopathy and myocarditis, 36% and 25%, respectively, were anti-flavoenzyme-positive by Western blot and ELISA, but only two of 15 patients with other heart diseases and none of 50 healthy controls.     

Incidence and Clinical Significance of Ventricular Late Potentials in Idiopathic Dilated Cardiomyopathy Compared to Coronary Artery Disease

Objective: This prospective study was designed to compare incidence and clinical significance of ventricular late potentials between patients with idiopathic dilated cardiomyopathy (IDC) and postinfarct patients (CAD) using exactly the same method of signal-averaged electrocardiography (SAECG) in both patient groups. Methods: Time-domain analysis of SAECG was performed in 120 consecutive patients with IDC, 120 patients with CAD, and 60 healthy controls. Ventricular late potentials were detected in 27 of 120 patients with IDC (23%) compared to 41 of 120 patients with CAD (34%; P < 0.05). Results: Ventricular late potentials were found in 2 of 60 controls (3%). During 15 ± 7 months follow-up, serious arrhythmic events occurred in 17 of 120 patients with IDC (14%) and in 13 of 120 patients with CAD (11%). The sensitivity of ventricular late potentials for future arrhythmic events was 35% for IDC compared to 77% for CAD (P < 0.05). The positive predictive value of late potentials detected by time-domain analysis was 22% for IDC versus 24% for CAD (P = ns). Conclusion: In this selected patient population with IDC and CAD, time-domain analysis of SAECG revealed a lower incidence of ventricular ate potentials in patients with IDC as compared to postinfarct patients. Whereas ventricular late potentials had a high sensitivity but a low positive predictive value for identification of postinfarct patients with serious arrhythmic events during follow-up, both sensitivity and positive predictive value of ventricular late potentials for future serious arrhythmic events were low in the setting of IDC.     

Isolated noncompaction of the ventricular myocardium

Isolated noncompaction of the ventricular myocardium is a recently described anomaly. We report the first case of this anomaly presenting as a restrictive cardiomyopathy, and the first association of this entity with endocardial fibrosis.