扩张型心肌病患者血清可溶性细胞间黏附分子与血管功能和心功能的关系

目的:观察扩张型心肌病(DCM)患者外周血中可溶性细胞间黏附分子-1(sICAM-1)的水平及与血管功能和心功能的关系.方法:DCM患者65例,正常对照组49例.用酶联免疫吸附法测定2组血清中sICAM-1的水平;用无创动脉功能测定仪测定大动脉弹性指数(C1)和小动脉弹性指数(C2).用NYHA分级评价心功能.结果:①DCM组外周血sICAM-1的水平显著高于正常对照组,而DCM组C2显著低于正常对照组,2组间C1差异无统计学意义;②血清sICAM-1与C2呈负相关.sICAM-1是影响C2的重要因素;③随着心功能下降,血清中sICAM-1的水平逐渐升高,两者呈等级相关.结论:DCM患者心功能和血管内皮功能下降与sICAM-1升高有关.     

Preliminary Survey on the Prevalence Rate of Hypertension in Patients with Dilated Cardiomyopathy

Objectives To investigate the prevalence of hypertension and its primary risk factors in patients with dilated cardiomyopathy (DCM). Methods Three hundred and sixty-two patients with DCM(DCM group)and 401 age-matched residents (control group) were enrolled randomly in the study, the hypertensive prevalence rate were calculated respectively in the two groups and were compared with each other; the patients in the DCM group were divided into two subgroups (hypertension subgroup and non-hypertension subgroup) according to whether the patients have hypertension; the clinical data related to blood pressure was compared between the two subgroups. Results The prevalence of hypertension in DCM group was significantly higher than that in the control group (32.8% vs. 20.1%, P<0.01); There were no significant differences on the age, gender, occupation and left ventricular ejection fraction (LVEF) between the two subgroups, but the mean heart rate and the percentage of patients who had family history of hypertension were significantly higher in the hypertension subgroup than that in the non-hypertension subgroup (P<0.05 and P<0.01). Conclusions The prevalence of hypertension in patients with DCM was high; The increased activity of sympathetic nervous system and the hypertensive genetic factor may be the main risk factors of hypertension in patients with DCM.     

Plasma hemoglobin concentration was related to estimated glomerular filtration rate in elderly patients with ischemic Cardiomyopatny

Objectlves To stuaythe relationship between plasma hemoglobin concentration and estimated glomerular filtration rate (eGFR)in elderly patients with ischemic cardiomyopathy (ICM).Methods Clinical data of patients with coronary heart disease who weredischarged from The First Affiliated Hospital,Chongqing Medicai University between 2005 and 2007 were analyzed retrospectively.Echocardiography resuIts.plasma hemoglobin and creatinine concentration were abstracted from the medical records.The study included235 Chinese Han patients with age 60 years and older with angiography confwmed coronary heart disease.silent myocardial ischemia orangina pectoris,of whom 154 had ICM defined as left ventricular end-diastolic diameter(LVDd),male≥56mm,female≥51 mm(63.51±7.70 mm)measured by M-mode echocardiography.The differences in plasma hemoglobin concentration were analyzed retrospec-were no significant changes in plasma hemoglobin concentration and eGFR;however,plasma hernoglobin concentration was related toeGFR significantly positively in elderly patients with ICM due to coronary heart disease.     

Human homozygous R403W mutant cardiac myosin presents disproportionate enhancement of mechanical and enzymatic properties

Familial hypertrophic cardiomyopathy (FHC) is associated with mutations in 11 genes encoding sarcomeric proteins. Most families present mutations in MYBPC3 and MYH7 encoding cardiac myosin-binding protein C and beta-myosin heavy chain. The consequences of MYH7 mutations have been extensively studied at the molecular level, but controversial results have been obtained with either reduced or augmented myosin motor function depending on the type or homogeneity of myosin studied. In the present study, we took advantage of the accessibility to an explanted heart to analyze for the first time the properties of human homozygous mutant myosin. The patient exhibited eccentric hypertrophy with severely impaired ejection fraction leading to heart transplantation, and carries a homozygous mutation in MYH7 (R403W) and a heterozygous variant in MYBPC3 (V896M). In situ analysis of the left ventricular tissue showed myocyte disarray and hypertrophy plus interstitial fibrosis. In vitro motility assays showed a small, but significant increase in sliding velocity of fluorescent-labeled actin filaments over human mutant cardiac myosin-coated surface compared to control (+18%; P<0.001). Mutant myosin exhibited a large increase in maximal actin-activated ATPase activity (+114%; P<0.05) and Km for actin (+87%; P<0.05) when compared to control. These data show disproportionate enhancement of mechanical and enzymatic properties of human mutant myosin. This suggests inefficient ATP utilization and reduced mechanical efficiency in the myocardial tissue of the patient, which could play an important role in the development of FHC phenotype.     

扩张型心肌病大鼠心肌胶原改建及perindopril的干预研究

目的:观察不同病程扩张型心肌病(DCM)大鼠心肌胶原改建与心功能的关系及perindopril的干预作用。 方法:不同累积剂量ADR心肌病大鼠,分为perindopril干预和非干预组。用胶原Ⅰ、Ⅲ型单克隆抗体免疫组化染色心肌结合图像分析、电镜、电生理技术,观察胶原改建及perindopril的作用。 结果:心肌间质胶原随着ADR累积量的增加而增多。胶原纤维早期较为均匀,晚期粗细不等并有断裂,Ⅰ／Ⅲ型比值升高且与心功损害平行。perin-dopril组动物胶原增生受到明显抑制,Ⅰ／Ⅲ型比值接近正常,心功能得到改善。 结论:心肌胶原病理改建是DCM心功能恶化的原因之一,perindopril可起到一定程度的保护作用。     

Prognostic Impact of Ischemic Mitral Regurgitation Severity and Myocardial Infarct Quantification by Cardiovascular Magnetic Resonance

ObjectivesThis study sought to evaluate the role of cardiac magnetic resonance (CMR) for the quantification of ischemic mitral regurgitation (IMR) and myocardial infarct size (MIS) in patients with ischemic cardiomyopathy (ICM). This study also sought to explore the interaction between IMR severity and MIS and its association with outcomes in patients with ICM.BackgroundIMR occurs secondary to a disease of the left ventricle and is associated with poor outcomes. The role of CMR for the evaluation and risk stratification of patients with ICM and IMR remains uncertain.MethodsConsecutive patients with ICM who underwent baseline CMR were included. MIS was quantified on late gadolinium enhancement imaging as the proportion of left ventricular mass. IMR was quantified with CMR by calculating the mitral regurgitant fraction (MRFraction). Cox proportional hazards models were built to assess the association of IMR and MIS quantification with the combined endpoint of all-cause death or heart transplant.ResultsWe evaluated 578 patients (mean age: 62 ± 11 years, 76% males). The mean left ventricular ejection fraction was 25 ± 11%, with an MIS of 24 ± 16% and MRFraction of 18 ± 17%. Over a median follow-up time of 4.9 years, 198 (34%) patients experienced death or cardiac transplant. On multivariable analysis, after comprehensive medical risk score, subsequent revascularization, implantable cardioverter-defibrillator insertion, and surgical mitral valve intervention were controlled for, the interaction of IMR severity and MIS emerged as a powerful predictor of adverse outcomes (p = 0.008). For patients with significant IMR (MRFraction: ≥35%), the hazard ratio comparing moderate MIS (15% to 29%) versus small MIS (<15%) was 1.51 (0.57 to 3.98), and the hazard ratio comparing large MIS (≥30%) versus small MIS was 5.41 (2.34 to 12.7).ConclusionsRisk associated with IMR is more comprehensively described as an interaction between IMR severity and MIS. Further studies in patients IMR using comprehensive CMR evaluation are needed to verify whether this approach can improve patient selection and procedural outcomes to address IMR.     

Outcomes in Patients With Hypertrophic Cardiomyopathy and Left Ventricular Systolic Dysfunction

BackgroundEnd-stage (ES) hypertrophic cardiomyopathy (HCM) has been considered a particularly grim and unfavorable disease complication, associated with substantial morbidity and mortality, frequently requiring heart transplant. Previous reports have included small numbers of patients with relatively short follow-up, predominantly in prior treatment eras.ObjectivesThe purpose of this study was to re-evaluate clinical profile and prognosis for end-stage heart failure in a large HCM cohort with contemporary management strategies.MethodsPatients at Tufts HCM Institute, from 2004 to 2017, were identified with ES and systolic dysfunction (ejection fraction [EF] <50%), followed for 5.8 ± 4.7 years (up to 18 years).ResultsOf the 2,447 patients, 118 (4.8%) had ES-HCM (EF 39 ± 9%; range 12% to 49%) at age 48 ± 15 years. Notably, over follow-up, 57 patients (48%) achieved clinical stability in New York Heart Association functional classes I/II with medical treatment (or cardiac resynchronization therapy), including 6 patients ≥10 years from ES diagnosis (up to 14 years). In total, 61 other patients (52%) developed refractory heart failure to disabling New York Heart Association functional classes III/IV (5.2%/year); 67% have survived, including 31 with heart transplant. Of the 118 ES patients, 21 had appropriate implantable cardioverter-defibrillator (ICD) therapy terminating potentially lethal tachyarrhythmias, with no difference in frequency of events in patients with EF 35% to 49% versus EF <35% (17% vs. 19%; p = 0.80). With all available treatment modalities, ES-related mortality was 1.9%/year, with 10-year survival of 85% (95% confidence interval: 77% to 94%). Mortality was 4-fold lower than previously reported for ES (8.0%/year), but exceeded 10-fold HCM with preserved EF (0.2%/year; p < 0.001).ConclusionsAlthough ES remains an important complication of HCM, contemporary treatment strategies, including ICDs and heart transplant, are associated with significantly lower mortality than previously considered. Primary prevention ICDs should be considered when EF is <50% in HCM. Rapid heart failure progression is not an inevitable consequence of ES, and some patients experience extended periods of clinical stability.     

四维超声心动图评价扩张型心肌病右心室整体收缩功能及其临床意义

目的探讨四维超声心动图评价扩张型心肌病(DCM)右心室整体收缩功能的意义。方法将本院2017年1月—2018年12月收治的68例DCM患者纳为研究对象,根据普通二维超声测量结果,将右心室内径扩大者纳为A组(n=33),右心室内径正常者纳为B组(n=35),并将同期行健康体检者纳为对照组(n=40),所有研究对象均行常规超声心动图检查并存储留取四维超声图像备分析,统计三组左心室内径、射血分数及右心室容积、射血分数,以及右心室总体应变参数[总体长轴峰值应变(GPSL)、总体圆周峰值应变(GPSC)、总体面积峰值应变(GPSA)和总体径向峰值应变(GPSR)],常规药物治疗4周后,比较DCM患者治疗前后右心室相关参数变化,评价四维超声心动图在评价DCM患者右心室收缩功能中的作用。结果三组左心室舒张期内径、左心室射血分数、右心室容积以及右心室射血分数均存在显著差异(P<0.05);三组GPSL、GPSC、GPSA以及GPSR水平差异显著(P<0.05); DCM患者治疗后,其三尖瓣峰值反流速度、右心室射血分数、GPSL、GPSC、GPSA和GPSR水平均较治疗前显著上升(P <...     

Comparison of time domain and spectral turbulence analysis of the signal-averaged electrocardiogram for the prediction of prognosis in idiopathic dilated cardiomyopathy

Background: Despite significant advances in the treatment of heart failure, the prognosis of patients with idiopathic dilated cardiomyopathy remains poor. Although several of prognostic variables have been shown to be useful in risk stratification of patients with idiopathic dilated cardiomyopathy, their predictive accuracy is low and clinical usefulness uncertain. Hypothesis: This study was undertaken to assess the signal-averaged electrocardiogram (SAECG) in patients with idiopathic dilated cardiomyopathy and to compare the ability of time domain and spectral turbulence analytic techniques to predict clinical outcome. Methods: SAECG analysis was performed in 80 patients with idiopathic dilated cardiomyopathy. Nineteen patients had left bundle-branch block and eight were taking low-dose ami-odarone for life-threatening arrhythmias. Conventional time domain and spectral turbulence analyses of the SAECG were performed using Del Mar 183 software. Results: During a follow-up of 27 ± 19 months, 24 patients developed progressive heart failure, while the others remained clinically stable. Late potentials were detected in 28% of patients and were equally frequent in patients with and without progressive heart failure (38 vs. 23%, p = 0.20). Spectral turbulence analysis was abnormal in 34% of patients, and patients with abnormal results developed progressive heart failure more frequently than those with normal results (50 vs. 17%, p = 0.01). All spectral turbulence analysis parameters were significantly different in patients with progressive heart failure compared with those who remained clinically stable (p≤0.01). Furthermore, progressive heart failure-free survival at 2 years was significantly lower in patients with abnormal compared with normal results (63 vs. 87%;p<0.05), but was similar in patients with and without late potentials (72 vs. 83%;p = 0.30). The relative risk for developing progressive heart failure using spectral turbulence analysis was 3.4 (95% confidence interval 1.2–9.7) and 2.8 (95% confidence interval 1.1–8.7) using time domain analysis. The sensitivity, specificity, and the positive and negative predictive accuracy for identifying patients who developed progressive heart failure were 50,83,50, and 83%, respectively, (p = 0.01) for spectral turbulence analysis, and 36,85,45 and 80%, respectively, (p = 0.09) for time domain analysis. Conclusion: Abnormalities in the SAECG of patients with idiopathic dilated cardiomyopathy are common and appear to provide a noninvasive marker for development of progressive heart failure.