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.     

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.     

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.     

Changes of Left Ventricular Geometry Shape and Left Ventricular Regional Function in Patients With Dilated Cardiomyopathy

To assess the left ventricle regional systolic and diastolic function, left ventricle geometry and left venticle sphericity indexes in patients with dilated cardiomyopathy （DCM） by quantitative tissue velocity imaging （QTVI）. Methods Thirty normal subjects and 52 DCM patients underwent QTVI and colour Doppler flow imaging study in order to measure the left ventricular regional function along left ventricle apical long-axis view and the left ventricle geometry. Peak tissue velocities of left venticle regional muscular tissue during systole （ Vs）, systolic acceleration （ a）, early diastole（Ve） and left atrium contraction（Va） along left venticle apical long axis view were measured. The indexes of left ventricular regional systolic and diastolic function were mearsured at the same time. The left ventricle geometry shape was reflected from the systolic and diastolic sphericity index （Sis and Sid）, the left ventricular ejection fraction （LVEF） and D wave / A wave （PVd/PVa） of pulmonary veins flowing spectrum reflected the global left ventricular systolic and diastolic function. The Vs, Ve, Va, a, PVd/PVa ratio, LVEF, Sis, Sid and their correlations between normal subjects and patients with DCM were compared and analyzed. Results Vs, Ve, Va, a, PVd/PVa, Sis and Sid in patients with DCM were lower than those in normal persons. There were significant relations between Sis and a （ r = 0. 6142, P 〈 0. 05）, Ve/Va and Sid （ r = 0. 6271, P 〈 0. 05 ）. Conclusions QTVI offer a newer method which has a higher sensitivity and accuracy in evaluating the left venticle regional systolic and diastolic function in DCM patients. There was significant relation between regional cardiac function and left venticle sphericity. （ S Chin J Cardiol 2009; 10（1） ： 9 -14）     

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.     

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。     

左心室收缩不同步对扩张性心肌病患者预后的预测价值

目的:运用组织多普勒超声心动图研究左心室收缩不同步在扩张性心肌病患者中的预后价值.方法:入选62例扩张性心肌病患者,所有患者均行心电图、超声心动图(包括组织多普勒超声心动图)及冠状动脉造影,入选患者根据心室内延迟时间(IVD)分为2组:IVD≤65 ms组(n=10),IVD>65 ms组(n=52),主要终点定义为随访期内的全因死亡.结果:IVD>65 ms组患者有较长的IVD(129±68 ms vs 58±9 ms,P＜0.05),较高的左束支传导阻滞发生率(31% vs 10%,P=0.05).IVD≤65 ms组1例死亡,为心原性猝死;而IVD>65 ms组29例死亡,12例为心原性猝死,17例为进展性心力衰竭.Kaplan-Meier生存分析显示IVD>65 ms组患者有较低的生存率(P＜0.05).在调整其它主要影响因素后,IVD>65ms是死亡的独立预测因子(RR=1.8502,P＜0.01)结论:高IVD是扩张性心肌病患者死亡的独立预测因素.     

Improvement in Left Ventricular Diastolic Stiffness Induced by Physical Training in Patients With Dilated Cardiomyopathy

BackgroundDiastolic dysfunction in long-term heart failure is accompanied by abnormal neurohormonal control and ventricular stiffness. The diastolic phase is determined by a balance between pressure gradients and intrinsic ventricular wall properties: according to a mathematical model, the latter (ie, left ventricular [LV] elastance, K_LV) may be calculated by the formula: K_LV = (70/[DT-20])² mm Hg/mL, where DT is the transmitral Doppler deceleration time.Methods and ResultsIn 54 patients with chronic systolic heart failure (39 men, 15 women; age 65 ± 10 years; New York Heart Association [NYHA], 2.3 ± 0.9; ejection fraction [EF], 32% ± 5%), we analyzed the relationship between K_LV and an index of neurohormonal derangement (levels of brain natriuretic peptide [BNP]), and investigated whether 3 months of physical training could modulate diastolic operating stiffness. Patients were randomized to physical training (n = 27) or to a control group (n = 27). Before and after training, patients underwent Doppler echocardiogram and cardiopulmonary stress test. At baseline, ventricular stiffness was related to BNP levels (P < .01). Training improved NYHA class, exercise performance, and estimated pulmonary pressure. BNP was reduced. Ventricular volumes, mean blood pressure, and EF remained unchanged. A 27% reduction of elastance was observed (K_LV, 0.111 ± 0.044 from 0.195 ± 0.089 mm Hg/mL; P < .01), whose magnitude was related to changes in BNP (P < .05) and to K_LV at baseline (P < .01). No changes in K_LV were observed in controls after 3 months (0.192 ± 0.115 from 0.195 ± 0.121 mm Hg/mL).ConclusionsIn heart failure, left ventricular diastolic stiffness is related to neurohormonal derangement and is modified by physical training. This improvement in LV compliance could result from a combination of hemodynamic improvement and regression of the fibrotic process.     

Ventricular Arrhythmias and Left Ventricular Dysfunction in Familial Cardiomyopathy

ABSTRACT. In familial cardiomyopathy (CM), different forms of myocardial abnormalities including asymmetric and symmetric hypertrophy and dilated left ventricles are presented, mostly showing varying hereditary penetrance. This study presents a family with CM including three major clinical manifestations: severe ventricular arrhythmias, repolarization abnormalitites and left ventricular hypertrophy. This triad was strikingly consistent in the two generations examined. The familial pattern with an autosomal dominant inheritance did not show any linkage to the HLA region.     

非缺血性扩张性心肌病左心室收缩不同步的预测价值研究

目的运用组织多普勒超声心动图研究左心室收缩不同步在非缺血性扩张型心肌病患者中的预后价值。方法入选62例非缺血性扩张型心肌病患者,所有患者均行心电图、超声心动图(包括组织多普勒超声心动图)及冠状动脉造影,入选患者根据心室内延迟时间(IVD)分为两组:≤65ms(组1,n=10)65ms(组2,n=52),主要终点定义为随访期内的全因死亡。结果组2患者有较长的心室内延迟时间〔(129±68)ms vs(57.5±8.7)ms;P=0.013〕,较高的左束支传导阻滞发生率(30.8%vs 10%;P=0.05),较高的病死率(55.8%vs 10%;P0.05)。组1中1例患者发生心源性猝死;而组2中有12例发生,且其他原因引起的死亡也发生在组2。Kaplan-Meier生存分析显示组2患者有较低的生存率(P=0.045),多因素分析显示IVD是惟一具有显著差异的死亡预测因子(P0.05)。结论高IVD是非缺血性扩张型心肌病患者死亡的独立预测因素,这些患者应较早考虑接受心脏再同步化治疗。     

Regional Heterogeneity of Systolic Dysfunction Is Associated with Ventricular Dyssynchrony in Patients with Idiopathic Dilated Cardiomyopathy and Narrow QRS Complex

Background: Regional heterogeneity of left ventricular (LV) contraction, known as dyssynergy, in idiopathic dilated cardiomyopathy (IDC) patients has been previously reported, but no comprehensive analysis of this abnormality has been made. The purpose of this study was to test the hypothesis that regional heterogeneity of systolic dysfunction is associated with LV dyssynchrony in IDC patients with a narrow QRS complex using novel three‐dimensional (3D) speckle‐tracking strain. Methods: We studied 54 consecutive IDC patients with ejection fraction (EF) of 34 ± 12% and QRS duration of 102 ± 13 msec (all <120 msec), and 30 age‐matched normal controls. The 3D speckle‐tracking LV dyssynchrony (LV dyssynchrony index) was quantified from all 16 LV sites to determine the standard deviation (SD) of time‐to‐peak strain. Similarly, regional heterogeneity of LV systolic function (LV dyssynergy index) was quantified from all 16 LV sites to establish the SD of peak 3D speckle‐tracking strain. Results: The LV dyssynergy and dyssynchrony indices of IDC patients were significantly larger than those of normal controls. Furthermore, IDC patients showed significantly higher Z‐scores for septum and inferior regions than for the free wall (3.34 ± 1.21 vs. 1.69 ± 1.06 and 2.79 ± 1.30 vs. 1.69 ± 1.06, respectively, P < 0.001). An important findings of multivariable analysis was that the LV dyssynergy index (β = 0.69, P < 0.001) and LVEF (β = ?0.34, P = 0.001) were independent determinants of the LV dyssynchrony index. Conclusion: 3D speckle‐tracking strain revealed that the myocardial systolic dysfunction of IDC patients with a narrow QRS complex has a marked heterogeneous regional distribution. This regional heterogeneity as well as systolic dysfunction is thought to lead to LV dyssynchrony.     

Dilated Cardiomyopathy With Left Ventricular Thrombi as a Presenting Feature of Cushing Disease

We report on a 20-year-old man with dilated cardiomyopathy and intracardiac thrombi associated with Cushing disease (CD). The patient presented with symptomatic heart failure. Follow-up echocardiography showed 2 thrombi in the apex of the left ventricle, which resolved after intravenous heparin therapy. The patient was first treated symptomatically, and then trans-sphenoidal adenomectomy was performed. Although cortisol excess alone may not be sufficient to produce severe cardiomyopathy, progressive improvement of cardiac function was observed within 3 years after surgery.     

Use of Color Kinesis for Evaluation of Left Ventricular Filling in Patients With Dilated Cardiomyopathy and Mitral Regurgitation

Objectives. We tested the feasibility of using analysis of color kinesis images to objectively assess global and regional left ventricular (LV) diastolic function in patients with dilated cardiomyopathy (DCM). In addition, the ability of this technique to track drug-induced changes on LV diastolic properties was studied.

Background. Diastolic dysfunction contributes to symptomatology in patients with DCM. The assessment of LV diastolic function using conventional Doppler echocardiography is indirect and is confounded by multiple variables. Moreover, the noninvasive evaluation of regional diastolic properties is difficult. In contrast, color kinesis directly tracks and color-encodes regional diastolic endocardial motion.

Methods. We studied 24 patients with DCM and mitral regurgitation (MR) and 24 age-matched normal subjects. Transmitral and pulmonary vein flow velocities were measured using pulsed Doppler echocardiography. Diastolic color kinesis images were used to calculate indexes of magnitude and timing of global and regional diastolic function. Diastolic asynchrony was evaluated in different subgroups of patients with DCM. The effects of drug infusions (nitroprusside and dobutamine) were also studied.

Results. Color kinesis indexes of global diastolic function showed significant differences between patients with DCM and normal subjects. Compared with Doppler indexes, color kinesis was less confounded by MR and was capable of differentiating between drug-induced lusitropic and vasodilator effects. Diastolic asynchrony was increased in patients with DCM and severe MR.

Conclusions. Quantitative analysis of global and regional LV diastolic function in patients with DCM using color kinesis is feasible.     

左心室重构对扩张型心肌病心力衰竭近期死亡的意义

目的:研究左心室重构指标对扩张型心肌病心力衰竭近期死亡的估测价值.方法:随机选取扩张型心肌病心力衰竭患者103例,于入选当时行常规经胸心脏彩色多普勒超声心动图检查,测量左心房内径(LADs),左心室内径,左心室射血分数,球形指数等指标.所有患者随访1～12个月,平均(6.57±3.53)个月,记录死亡事件(死亡者为死亡组)及存活时间(存活者为存活组).对比死亡与存活者上述各指标的差异,并采取相关分析和多因素分析法筛选与近期死亡密切相关的指标.结果:在随访过程中,死亡21例,其中死于心力衰竭者17例.死亡组的患者其左心房收缩末期内径及左心室舒张末期内径明显扩大,球形指数-1显著大于存活组,有显著性差异(P＜0.05),同时收缩功能障碍和心功能分级也明显重于存活组.经Cox多因素回归分析,与生存时间相关的指标只有心功能分级(B＝0.833,P=0.024),LADs(B＝0.073,p=0.028)和球形指数-1 (B＝1.401,p=0.028),其中球形指数-1的相关性最大.结论:心功能分级、左心房收缩末期内径及左心室球形指数-1对扩张型心肌病心力衰竭的近期死亡有重要预测价值,其中评价左心室形态的球形指数-1价值最大,而衡量左心室大小及收缩功能的指标对近期死亡并无影响.     

组织多普勒技术评价扩张型心肌病左室心功能的临床价值

目的探讨组织多普勒技术(TDI)检测扩张型心肌病(DCM)左室心功能的临床价值。方法选择DCM病人和正常健康者各40例,用TDI于胸骨旁长轴检测室间隔及左室后壁中间段短轴方向心肌运动速度(MV),并计算心肌运动速度阶差(MVG);经心尖窗检测左室前壁、后壁、下壁、侧壁和前壁、后壁室间隔中间段长轴方向MV。结果DCM组病人出现4种MV频谱形态异常,长轴、短轴方向MV均明显低于正常对照组(P<0.05或P<0.01);DCM组MVG也明显低于正常对照组(P<0.05或P<0.01)。结论TDI可定量评价DCM病人左室心肌功能,丰富了DCM的超声诊断手段。     

主动脉瓣置换术后显著肥大左心室的逆转

目的 :探讨主动脉瓣置换术后显著肥大左心室的逆转和收缩功能的恢复趋势及其影响因素。方法 :分析 1991年 1月至 2 0 0 0年 12月间 3 2例左心室显著肥大患者施行主动脉瓣置换术后超声心动图随访资料。结果 :主动脉瓣置换术后左心室形态呈进行性缩小、心肌肥厚显著减轻。影响显著肥大左心室术后完全逆转的主要术前因素是左心室的收缩末内径≥ 6 0cm、舒张末容积指数≥ 2 10ml/m2 和重量指数≥ 15 0g/m2 (P <0 0 1)。术前射血分数≥ 0 40者术后射血分数和短轴内径缩短率的恢复显著优于术前射血分数 <0 40者 (P <0 0 5～ 0 0 1)。术后射血分数≥ 0 5 0或短轴内径缩短率≥ 2 5 %与术后恢复时间呈正相关 ,与术前左心室收缩末内径≥ 6 0cm呈负相关 (R2 =0 5 5 ,P <0 0 1)。　　结论 :在左心室的收缩末内径≥ 6 0cm或舒张末容积指数≥ 2 10ml/m2 、重量指数≥ 15 0 g/m2 和射血分数 <0 40前手术 ,有利于术后左心室逆转和左心室收缩功能恢复 ,术后 6个月～ 1年是恢复的关键期。