二维斑点成像技术评价扩张型心肌病患者心脏的纵向扭转

目的 运用二维斑点成像技术评价扩张型心肌病患者(dilated cardiomyopathy,DCM)与健康人间各参数的差异,证实DCM患者心脏纵向扭转的存在.方法 搜集温州医学院附属第一医院2008年6月至2009年9月期间就诊的42例DCM患者作为DCM组和35例健康人作为对照组.两组均行常规超声心动图,检测左心房内径、左心室射血分数、过二尖瓣口舒张早期血流速度及舒张晚期血流速度.应用GE Echopac软件测量得到DCM组和对照组患者心尖四腔心的左心室壁的径向应变、应变率,左心室侧壁、室间隔、心尖及左心室整体纵向峰值扭转角度,对比两组间各参数的差异.结果 (1) DCM组患者左心房内径、左心室收缩末期容积及左心室舒张末期容积均明显大于对照组(P均＜0.01),左心室射血分数明显低于对照组(P＜0.0l),过二尖瓣口舒张早期血流峰速度/舒张晚期血流峰速度两组比较差异无统计学意义(P＞0.05).(2) DCM组患者径向收缩期峰值应变以及收缩期、舒张早期、舒张晚期径向峰值应变率均明显低于对照组(P均＜0.01).(3)对照组左心室侧壁的中间段、基底段以及心尖段呈逆时针扭转,而室间隔的基底段、中间段呈顺时针扭转,DCM组患者左心室侧壁的中间段呈逆时针扭转,而左心室侧壁基底段、心尖段、室间隔的基底段及中间段均呈顺时针扭转.DCM组患者左心室侧壁中间段和基底段、心尖段以及室间隔基底段的扭转角度均明显低于对照组(P均＜0.01).对照组左心室呈较小角度的纵向逆时针扭转(0.76°±2.63°),而DCM组患者左心室呈纵向顺时针扭转(- 1.58°±3.42°),两组扭转角度的差异有统计学意义(P＜0.01).(4)DCM组患者左心室侧壁基底段、中间段与室间隔基底段扭转达峰时间差均与左心室的纵向峰值扭转角度具有相关性(r=0.409,P=0.007; r=0.396,P=0.009).结论 应用二维斑点成像技术,通过分析各节段应变、应变率及纵向扭转角度等参数,证实DCM患者心脏存在着一定角度的纵向顺时针扭转,DCM患者左心室侧壁基底段、中间段与室间隔基底段扭转达峰时间差可能是形成DCM患者心脏纵向扭转的一个原因.     

扩张型心肌病162例心电图的分析

扩张型心肌病１６２例心电图的分析孙群（浙江省诸暨市人民医院内科３１１８００）关键词扩张型心肌病超声心动描记术心电描记术扩张型心肌病系原发性、广泛性的心肌细胞变性和心肌间质纤维化，因心肌各部位病变严重程度不同，心电图表现亦各不相同，作者收集１９８０－０...     

家族性扩张型心肌病2例

家族性扩张型心肌病是指同一家系的血亲中有两名或两名以上的扩张型心肌患者,在临床上扩张型心肌病很常见,但有典型家族病史且有极其相似的症状和体征者很少见.现报道两例如下:     

扩张型心肌病合并肺动脉高压的预测因素研究

目的 对扩张型心肌病(dilated cardiomyopathy,DCM)合并肺动脉高压(pulmonary arterial hypertension,PAH)患者与未合并PAH的患者进行临床对比,探讨DCM患者发生PAH的预测因素.方法 入选2014年12月—2017年12月在山东大学齐鲁医院就诊的DCM患者16...     

老年扩张型心肌病合并高血压患者的临床特征分析

目的分析老年扩张型心肌病患者合并高血压的临床特征。方法选取老年扩张型心肌病患者188例,根据是否合并高血压分为合并组85例和非合并组103例,比较分析2组基本临床资料差异。结果合并组年龄[(70.95±7.86)岁vs (67.97±7.55)岁]和心率[(79.17±20.17)次/min vs (73.78±16.37)次/min]明显高于非合并组(P0.05);合并组糖化血红蛋白水平明显高于非合并组[(6.65±1.53)%vs (6.07±0.40)%,P0.05]。合并组左心室内径和左心室舒张早期快速充盈峰/左心室舒张晚期快速充盈峰比值均小于非合并组(P0.05)。结论老年扩张型心肌病患者是否合并高血压在临床指标上体现出一定差异性,为今后进一步开展精确的个性化治疗提供了研究思路。     

扩张型心肌病心衰患者治疗过程中左心房内径与ptfv1的关系

扩张型心肌病心衰患者治疗过程中左心房内径与ｐｔｆｖ１的关系张缤郭雪娅余静辛楠（兰州医学院附属二院心内科兰州７３００３０）关键词扩张型心肌病超声心动描记术心电描记术本院１９８６年至１９９３年间住院确诊为原发性扩张型心肌病（ＤＣＭ）患者５６例，经治疗２周...     

肥厚性心肌病患者右心室结构与功能变化的分析

目的:应用彩色多普勒组织成像(CDTI)技术探讨肥厚性心肌病(HCM)患者右心室结构与功能的变化。方法:HCM患者35例(HCM组),正常对照组30例,应用CDTI测量左、右心室腔大小,室壁厚度,心室流出道等指标,于心尖四腔切面记录舒张期血流频谱,测量右室等容舒张时间(IRT)、等容收缩时间(ICT)、右室射血时间(RVET)及Tei指数。结果:与正常对照组比较,HCM患者右室前壁厚度[(4.56±0.70)mm比(5.43±0.87)mm]、室间隔厚度[(8.66±2.51)mm比(20.53±3.37)mm]、左室后壁厚度[(9.10±2.24)mm比(13.06±1.68)mm]均明显增加(P<0.05或<0.01),右室Tei指数[(0.39±0.61)比(0.80±0.38)]显著升高(P<0.01),右室流出道、右室舒张末期内径两组间无显著差异(P>0.05)。结论:肥厚性心肌病患者中右心室解剖结构与功能均受影响;右室Tei指数可以反映右心收缩及舒张功能变化。     

多普勒超声检测心肌运动指数评价扩张型心肌病左心功能

目的 :探讨超声检测心肌运动指数对评价扩张型心肌病 (DCM )左心功能的临床价值。方法 :DCM患者 31例 ,正常对照者 6 2例 ,应用多普勒超声心动图记录二尖瓣舒张期和左心室流出道收缩期脉冲多普勒血流频谱 ,测量心肌运动指数。结果 :①与正常对照组相比 ,DCM组等容舒张期时间 [(98.39± 2 0 .83)ms∶(6 6 .4 5±11.32 )ms ,P <0 .0 1]及等容收缩期时间 [(39.5 2± 13.31)ms∶(2 1.4 5± 7.6 5 )ms,P <0 .0 1]明显延长、射血时间[(2 2 3.39± 4 0 .93)ms∶(2 73.0 6± 2 1.0 1)ms ,P <0 .0 1]明显缩短 ,导致心肌运动指数 (0 .6 3± 0 .14∶0 .32± 0 .0 4 ,P <0 .0 1)明显升高 ,相关分析表明 ,心肌运动指数与左心功能障碍程度呈正相关 ;②相关分析显示 ,心肌运动指数与年龄、心率、血压无相关性。结论 :①心肌运动指数是评价DCM患者左心功能简便而准确的多普勒超声新指标 ,且与左心功能障碍程度呈正相关 ;②心肌运动指数不受年龄、心率、血压的影响     

扩张型心肌病心电图的改变及预后估价

扩张型心肌病心电图的改变及预后估价刘静茹１刘今是１郭玉春１刘忠铭２（１吉林省医药骨干研修班２白求恩医科大学第三临床学院长春１３００２１）关键词心肌病，扩张性肥大；心电描记术；超声心动描记术扩张型心肌病（ＤＣＭ）的病因尚不清楚，病毒性心肌炎被认为是主...     

扩张型心肌病蛋白质组学研究

目的:通过蛋白质组学方法比较扩张型心肌病(DCM)患者和正常人心肌组织差异蛋白质谱,找出与DCM相关的差异蛋白,进一步探讨DCM发生的分子生物学机制。方法:选择9例原发病为DCM、左心室射血分数<35%且接受心脏移植手术的患者作为DCM组:心肌组织取自患者自有心脏的左心室游离壁。对照组:心肌组织取自6例不能用作移植的供体心脏。采用双向凝胶电泳(2-DE)分析蛋白质谱差异,热考马斯亮蓝染色,串联质谱(MS-MS)鉴定差异蛋白。采用生物医学研发软件及资料库Ingenuity PathwaysAnalysis分析差异蛋白的定位、功能和相互作用。结果:DCM组和对照组心肌组织2-DE图像中蛋白点的整体分布比较相似,可以检测到超过1 000个蛋白点。通过比较分析,共鉴定出25种差异蛋白,其中在DCM组15种上调,10种下调。使用LOCATE数据库分析差异蛋白的亚细胞定位,提示其中大分子蛋白占40%,细胞骨架蛋白为28%,线粒体蛋白为28%。蛋白的PANTHER分类显示大多数差异蛋白参与了细胞的结构、肌肉收缩、钙离子转运和水解酶的活性等功能。生物学途径分类揭示差异蛋白参与了组织的形成过程、生物调节、发育过程、代谢过程和对刺激的应答过程等。结论:对DCM患者心肌组织蛋白质组学研究发现25种差异蛋白,其中上调表达的蛋白有15种,下调表达的有10种;这些蛋白参与了线粒体能量代谢、心肌收缩、凋亡等过程;提示这些过程可能在DCM发生发展中发挥重要作用。     

阿罗洛尔对扩张型心肌病患者左心室功能的影响

目的:观察阿罗洛尔治疗前后对扩张型心肌病(DCM)患者左心室功能的影响,并对其长期应用的安全性作出初步评价.方法:选择63例DCM伴中或重度心力衰竭(心衰)患者给予常规标准心衰治疗,在使用阿罗洛尔前及12个月后记录观测指标,以纽约心功能分级(NYHA)及二维超声心动图评价心功能.结果:阿罗洛尔治疗12个月后可使部分患者左心室功能得以改善,DCM患者的心率及收缩压双乘积较治疗前显著下降,差异有统计学意义(P<0.001).左心室收缩末期内径较治疗前显著缩小,差异有统计学意义(P<0.001).左心室射血分数(LVEF)由治疗前的0.27±0.08增加至治疗后的0.41±0.09,差异有统计学意义(P<0.001).试验中无因不良反应而终止治疗者.结论:初步的临床试验结果表明,阿罗洛尔治疗12个月后对DCM患者的左心室功能具有一定的改善作用,并具有良好的耐受性.     

Genetics of Idiopathic Dilated Cardiomyopathy

Familial dilated cardiomyopathy (DCM) should be an "evidence-based" diagnosis derived from clinical and echocardiographic screening of informed and consenting relatives of index patients, and on the examination of clinical reports for deceased relatives. Most familial dilated cardiomyopathy pedigrees show an autosomal pattern of inheritance. Very few of them are X-linked and matrilinear. Autosomal recessive inheritance is difficult to be assessed in an evidence-based setting. By linkage analysis, several loci, but no disease gene, have been identified. At present, few cases of familial dilated cardiomyopathy can benefit of a molecular diagnosis. The diagnosis of dystrophin defect-related dilated cardiomyopathy is important for patients and families, especially for carrier detection. These patients present X-linked inheritance, dominant cardiac involvement and raised levels of serum creatine phosphokinase. Defects of the glycoprotein complex associated to dystrophin (DAG) are rare skeletal muscle diseases with possible cardiac involvement. Mitochondrial diseases, both pure cardiomyopathies and multiorgan syndromes involving the heart, are associated to defects of mitochondrial DNA genes or of nuclear genes coding for mitochondrial proteins. Barth's syndrome develops in male children with granulocytopenia, dilated cardiomyopathy, and methylglutaconic aciduria. Cardiomyopathies with atrioventricular block are observed in hemochromatosis, Emery-Dreifuss syndrome, desmin storage disease, and in isolated familial dilated cardiomyopathy. Actin defects were recently identified in 2 unrelated patients with familial dilated cardiomyopathy. Desmin defects were also recently identified in 1 familial dilated cardiomyopathy. The overall knowledge, although in progression, is still limited. Clinical family screening identifies familial forms, preclinical cases, and inheritance pattern. By candidate gene screening, the molecular diagnosis can be provided for dystrophin, DAG, mitochondrial DNA, actin and desmin gene defects.     

Effects of Allopurinol on Coronary Microvascular and Left Ventricular Function in Patients With Idiopathic Dilated Cardiomyopathy

Background

Uric acid (UA) is an independent marker of mortality and associated with increased oxidative stress in patients with congestive heart failure (CHF). The present study aimed to investigate the effect of allopurinol on left ventricular (LV) function and coronary microvascular integrity in patients with idiopathic dilated cardiomyopathy (IDC).

Methods

Thirty-nine consecutive IDC patients were divided into 2 groups: elevated (> 7 mg/dL for men and >6.5 mg/dL for women; n = 24) and normal (n = 15) UA. Allopurinol 300 mg per day was given to the elevated UA group. Patients with elevated UA were assessed after a 3-month treatment period. Echocardiography assessing coronary flow reserve (CFR) and systolic and diastolic LV functions were studied.

Results

LV ejection fraction was significantly lower in elevated UA group: mean (interquartile range), 32.3% (26.0-36.5%) vs 37.3% (35.5-39.1%) (P < 0.01). Also, CFR and LV diastolic and combined function parameters were more prominently impaired in the elevated UA group. After allopurinol treatment, UA was significantly decreased (7.2 mg/dL [6.8-7.8] to 4.4 mg/dL [3.9-5.8]; P < 0.001) and CFR was markedly improved (1.87 [1.63-2.00] to 2.20 [1.87-2.49]; P < 0.001). The therapeutic effect of allopurinol on the reduction of UA from baseline was directly related to the improvement of CFR (r = 0.49; P = 0.01). Mitral A and E/E' were reduced, while S', E', E/A, and E'/A' were increased significantly.

Conclusions

The present study showed that 3-month treatment with allopurinol was significantly associated with reduced UA levels, and improvement of CFR and LV functions in patients with IDC and hyperuricemia.     

Comparison of Mortality Rates and Progression of Left Ventricular Dysfunction in Patients With Idiopathic Dilated Cardiomyopathy and Dilated Versus Nondilated Right Ventricular Cavities

This study assesses the influence of right ventricular (RV) dilation on the progression of left ventricular (LV) dysfunction and survival in patients with idiopathic dilated cardiomyopathy (IDC). Using transthoracic echocardiography, we studied 100 patients with IDC aged 20 to 80 years (mean 55 ± 14); 67% were men. In the apical 4-chamber view, diastolic LV and RV chamber area measurements classified patients into 2 groups: group RV enlargement+ (RV area/LV area>0.5) included 54 patients; group RV enlargement− (no RV enlargement) had RV area/LV area ≤0.5. Echocardiographic studies were repeated in all patients after a mean of 33 ± 16 months. At the time of the initial study, the 2 groups did not differ in age, gender, incidence of atrial fibrillation and diabetes, left ventricular mass, and LV ejection fraction, but the RV enlargement+ group had more severe tricuspid regurgitation and less LV enlargement. After 47 ± 22 months (range 12 to 96), patients in group RV enlargement+ had lower LV ejection fraction (29% vs 34%, p = 0.006) than patients with initial RV enlargement−. At clinical follow-up, mortality was higher (43%) in patients with initial RV enlargement+ than the RV enlargement− patients (15%), p = 0.002. For survivors, the mitral deceleration time averaged 157 ± 36 ms; for nonsurvivors or patients who required transplant, the mitral deceleration time averaged 97 ± 12 ms (p <0.0001). With use of a multivariate Cox model adjusting for LV ejection fraction, LV size, and age, the relative risk ratio of mortality from initial RV enlargement+ was 4.4 (95% confidence limits 1.7 to 11.1) (p = 0.002). Thus, patients with significant RV dilation had nearly triple the mortality over 4 years and more rapidly deteriorating LV function than patients with less initial RV dilation. In IDC, RV enlargement is a strong marker for adverse prognosis that may represent a different morphologic subset.