Hypertrophic cardiomyopathy masquerading as infiltrative restrictive cardiomyopathy and refractory congestive failure-resolution with catheter ablation of atrial flutter – A case report

We report a case of a young male presenting as Restrictive cardiomyopathy, refractory heart failure and syncope due to typical right atrial flutter complicating hypertrophic cardiomyopathy.Successful catheter ablation of the flutter promptly ameliorated the congestive failure with resolution of restrictive physiology.     

石斛合剂对高脂高糖糖尿病大鼠心肌细胞Ca^2+代谢的影响

目的以糖尿病心肌病大鼠为研究对象,探讨石斛合剂对糖尿病心肌病心肌细胞Ca^2+代谢的影响。方法 Wistar大鼠24只,取5只作为正常组,余19只采用高脂高糖饮食联合链脲佐菌素腹腔注射进行糖尿病心肌病造模。成模后,随机分为模型组、二甲双胍组、石斛合剂组。二甲双胍组、石斛合剂组分别予二甲双胍、石斛合剂灌胃,正常组、模型组予生理盐水灌胃。4周后,测空腹血糖,取左心室心肌组织进行组织病理学观察和心肌细胞内游离钙(MyoCa^2+)浓度测定,Western blot法检测肌浆网Ca^2+-ATP酶(SERCA2a)、L型钙通道α1C亚单位蛋白(CACNA1C)蛋白表达,RT-PCR法测定SERCA2a mRNA表达。结果石斛合剂组心肌纤维较模型组、二甲双胍组排列整齐,断裂较少。与正常组比较,模型组血糖、心肌细胞MyoCa^2+含量和CACNA1C蛋白表达明显升高(P<0.01,P<0.05),SERCA2a mRNA和蛋白表达明显降低(P<0.01)。与模型组比较,石斛合剂组血糖、心肌细胞MyoCa^2+含量和CACNA1C蛋白表达明显降低(P<0.01,P<0.05),SERCA2a mRNA和蛋白表达明显升高(P<0.01,P<0.05);二甲双胍组血糖明显降低(P<0.01)。与二甲双胍组比较,石斛合剂组心肌细胞MyoCa^2+含量明显降低(P<0.01,P<0.05),血糖、SERCA2a mRNA和蛋白表达明显升高(P<0.05)。结论石斛合剂通过提高SERCA2a mRNA和蛋白表达,加快摄取Ca^2+的速率,降低细胞质Ca^2+负荷,缓解钙超载,最终达到维持糖尿病心肌病钙稳态的作用,有效维持心肌细胞的正常收缩舒张功能;其降糖作用不是预防糖尿病心肌病的主要机理,而在于抑制CACNA1C蛋白表达,抑制钙诱导钙释放过程。     

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery: Diagnoses and Surgical Results in 12 Pediatric Patients

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital condition. It responds well to early diagnosis and treatment, but otherwise the prognosis is poor. We present our case series of 12 patients (mean age, 2 ± 2.58 yr; age range, 2 mo–8 yr), emphasizing the diagnostic process and discussing our surgical results. The diagnosis of ALCAPA should be suspected in infants who have dilated cardiomyopathy with electrocardiographic changes that suggest ischemia, and in older children who have isolated mitral regurgitation. When clinical suspicion is high, the results of 2-dimensional echocardiography combined with color-flow Doppler studies in expert hands can establish the diagnosis, thus avoiding angiography in critically ill infants. The treatment of choice in our patients was transfer and reimplantation of the left coronary artery onto the ascending aorta. There were 2 deaths: both were infants in extremis who underwent emergency surgery. An older child with severe ventricular dysfunction was given mechanical ventricular assistance and then heart transplantation. As of this report, all 10 survivors remained well and asymptomatic.     

扩张型心肌病家兔左室壁复极异质性的改变及意义

目的：观察离体心脏左心室三层心肌的单相动作电位的改变，以探讨扩张型心肌病易发心室颤动与三层心肌跨室壁复极不均一性的关系。方法：用阿霉素制作扩张型心肌病家兔模型，测定其室颤阈值（VFT）以及心外膜、中层心肌和心内膜心肌细胞的单相动作电位复极90％时程（APD90）、跨室壁复极离散度（TDR）。结果：扩张型心肌病VFT明显降低（P＜0．001），三层心肌细胞APD90均明显延长（P＜0．001），中层心肌细胞较心外膜、心内膜下心肌细胞延长更为显著（P＜0．05）；扩张型心肌病跨室壁复极离散度增加（P＜0．01）。结论：中层心肌细胞APD90明显延长、跨室壁复极离散度增加、三层心肌复极不均一性增加可能是扩张型心肌病容易发生心室颤动的重要原因。     

盐酸法舒地尔治疗缺血性心肌病心力衰竭

目的用6 min步行试验和多普勒超声心动图评价盐酸法舒地尔治疗缺血性心肌病心力衰竭的疗效。方法将60例因缺血性心肌病而导致慢性心力衰竭的患者分为2组各30例,均采用常规治疗,治疗组同时行静脉滴注盐酸法舒地尔60 mg每日1次。评估2组治疗前后6 min步行距离、左心室收缩末期内径(LVESD)、左心室收缩末期容积(LDESV)、左心室射血分数(LVEF)情况。结果用药3个月后治疗组6 min步行距离、LVESD、LVESV、VLEF较对照组均明显改善(P均<0.05)。结论在常规治疗基础上加用盐酸法舒地尔可提高运动耐量,改善患者心脏收缩功能,且耐受性良好。     

Usefulness of PCR Strategies For Early Diagnosis of Chagas'' Disease Reactivation and Treatment Follow-Up in Heart Transplantation

Heart transplantation (HTx) is a useful therapy for end‐stage Chaga? cardiomyopathy; however, Chagas reactivation remains a mayor complication. Parasitological methods offer poor diagnostic sensitivity, and use of more sensitive tools such as the Polymerase chain reaction (PCR) is usually necessary. In the present study, reactivation incidence and PCR usefulness for early reactivation diagnosis, as well as for treatment response evaluation during follow‐up, were analyzed using Strout parasite detection test, in 10 of 222 consecutive HTx patients suffering Chagas cardiomyopathy. PCR strategies targeted to minicircle sequences (kDNA, detection limit 1 parasite/ 10 mL blood) and miniexon genes (SL‐DNA, 200 parasite/10 mL) were performed to compare parasite burdens between samples. No patients received prophylactic antiprotozoal therapy (benznidazole). Five patients (50%) exhibited clinical reactivation within a mean period of 71.6 days; positive Strout results were observed in most cases presenting clinical manifestations. kDNA‐PCR was positive 38–85 days before reactivation, whereas SLDNA‐PCR became positive only 7–21 days later, revealing post‐HTx parasitic load enhancement present prior to clinical reactivation development. Reactivations were successfully treated with benznidazole and generated negative PCR results. Results observed in this study indicate the value of PCR testing for an early diagnosis of Chagas reactivation as well as for monitoring treatment efficacy.