Sudden death due to arrhythmogenic right ventricular cardiomyopathy: Two case reports

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a kind of primary myocardial disease characterized by the regional or global replacement of right ventricular myocardium by fatty and fibrolipomatous tissues. The ARVC, usually presenting with different clinical manifestations and pathological changes, were mainly seen in young men and is one of the main causes of sudden death in the young. Here two autopsied cases of Chinese men aged 30 and 23 years old who appeared healthy but died suddenly while at work are reported respectively. One of the victims had extensive and severe pathological changes in his heart involving the left ventricular wall as well as the ventricular septum and the right atrium. Not only was there a global fatty and fibrolipomatous tissue replacement of the right ventricular myocardia, but also mild sarcoplasmic coagulation in the myocardium and focal lymphocytic infiltration in the myocardial interstitium of the right ventricular wall. In addition, slight atherosclerosis of the coronary artery and intimal thickening of the sino-atrial node were observed. It is believed that there are no marked differences in the pathological changes of ARVC between Chinese patients and patients from western countries. The etiology and pathogenesis of ARVC could not be explained by a single cause or factor and they are probably related to various congenital and acquired causes or factors.     

Maternal mortality in the United States

Despite a significant improvement in the US maternal mortality ratio since the early 1900s, it still represents a substantial and frustrating burden, particularly given the fact that - essentially - no progress has been made in most US States since 1982. Additionally, the US Centers for Disease Control and Prevention has stated that most cases are probably preventable. Two disheartening issues within this topic include a gross underestimation of the magnitude of maternal mortality - particularly before 1987, but which likely persists to a lesser degree today - and the continued significant racial disparity in maternal mortality. Explanations for the plateau in maternal mortality include the recent trend of delayed childbearing, with the potential accompanying complications associated with older reproductive age (particularly over 35 years) and multiparity. The impressive increase in multifetal pregnancies related to delayed childbearing and assisted reproductive technology also plays a role. Finally, peripartum cardiomyopathy has become an increasingly recognized source of maternal mortality. Pregnancy-related mortality is largely accounted for by thromboembolic disease, hemorrhage, hypertension and its associated complications, and infection. However, since the inclusion of maternal deaths occurring after 42 days post-delivery as pregnancy related, traumatic injuries - including homicides and suicides - are an alarming source of maternal mortality. An especially important contemporary issue to consider within this topic is cesarean delivery "on maternal request", opponents of which cite concerns not only for immediate morbidity and mortality increased over that associated with a vaginal birth, but also for potential morbidity and mortality associated with future pregnancies. One particularly appealing opportunity to reduce maternal mortality is to recognize, examine, and learn from so-called "near-miss" cases.     

心肌致密化不全患者的常规心电图及心律失常分析

目的回顾性分析心肌致密化不全患者的体表心电图异常改变和心律失常发生情况。方法选择心肌致密化不全患者103例,对体表心电图、动态心电图及心律失常进行分析。结果 101例(98.1%)患者存在不同程度的心电图异常。窦性心律91例(88.3%),持续心房颤动12例(11.7%)。患者多表现P波异常、各种传导阻滞、QRS波起始切迹(类△波)、终末切迹、R波递增不良、异常Q波、ST段下移、T波双向、倒置。患者以室性心律失常多见,室性期前收缩占58.3%,室性心动过速占50.5%,4例发生心室颤动。结论体表心电图是心肌致密化不全的重要异常表现之一,主要特征表现为P波异常、各种传导阻滞、QRS波起始切迹(类△波)、终末切迹、异常Q波及ST-T改变。心律失常类型多样,以室性心律失常多见。     

The role of sarcomere gene mutations in patients with idiopathic dilated cardiomyopathy

We investigated a Danish cohort of 31 unrelated patients with idiopathic dilated cardiomyopathy (IDC), to assess the role that mutations in sarcomere protein genes play in IDC. Patients were genetically screened by capillary electrophoresis single strand conformation polymorphism and subsequently by bidirectional DNA sequencing of conformers in the coding regions of MYH7, MYBPC3, TPM1, ACTC, MYL2, MYL3, TNNT2, CSRP3 and TNNI3. Eight probands carried disease-associated genetic variants (26%). In MYH7, three novel mutations were found; in MYBPC3, one novel variant and two known mutations were found; and in TNNT2, a known mutation was found. One proband was double heterozygous. We find evidence of phenotypic plasticity: three mutations described earlier as HCM causing were found in four cases of IDC, with no history of a hypertrophic phase. Furthermore, one pedigree presented with several cases of classic DCM as well as one case with left ventricular non-compaction. Disease-causing sarcomere gene mutations were found in about one-quarter of IDC patients, and seem to play an important role in the causation of the disease. The genetics is as complex as seen in HCM. Thus, our data suggest that a genetic work-up should include screening of the most prominent sarcomere genes even in the absence of a family history of the disease.     

肝性心肌病的研究进展

肝性心肌病是指肝硬化患者的心脏结构和功能出现异常。其发病机制涉及心肌细胞的凋亡,β受体功能受损,一氧化氮、一氧化碳、内源性大麻素、细菌内毒素、炎症介质等心脏抑制因子的增多等。目前尚无统一的诊断标准,可以结合临床表现、B型利钠肽、N端利钠肽前体、心电图、超声、心脏磁共振等检查做出临床诊断。治疗上主要是在控制肝硬化的基础上给予保护心肌的治疗,适时肝移植可能是最佳治疗方法。     

红景天对肝硬化性心肌病治疗作用的临床研究

目的:观察红景天对肝硬化性心肌病(CCM)的治疗作用。方法:将40例CCM患者随机分为A组(20例)和B组(20例),同时以健康志愿者20例作为正常对照组(C组)。A组采用常规治疗,B组采用常规治疗+红景天治疗,以硝酸还原酶法测定血清NO浓度,以电化学发光法测定血浆N-末端脑钠肽前体(NT-proBNP)浓度,以彩色多普勒超声测定左室射血分数(LVEF)及舒张早期/晚期最大血流速度(E峰/A峰,E/A)比值。结果:A组及B组入院时LVEF、E/A比值显著低于C组,而血浆NT-proBNP及血清NO浓度则显著高于C组(P<0.001);A、B2组入院时、治疗10d、30d、90d比较,血浆NT-proBNP及血清NO浓度逐步下降(P<0.01)。B组治疗10d、30d及90d比较,LVEF及E/A比值于治疗10、30、90d逐步上升(P<0.01)。B组治疗30d与90dLVEF、E/A比值均高于A组,而血浆NT-proBNP浓度和NO均低于A组,B组治疗10d血清NO浓度低于A组(P<0.05或P<0.01)。结论:红景天对CCM有较好的治疗作用。     

钠-葡萄糖共转运蛋白2抑制剂对心室重构的影响

心室重构是多种心血管疾病进展过程中伴随的病理生理反应,心室重构越严重则心血管疾病患者预后越差,并最终导致心力衰竭及死亡,但目前临床可用于延缓或逆转心室重构的药物有限.钠-葡萄糖共转运蛋白2(SGLT-2)抑制剂是一种新型口服降糖药,已有多项研究表明其具有心血管保护作用.本文重点分析了SGLT-2抑制剂对急性心肌梗死、糖...