Echocardiography of dilated cardiomyopathy in children

Echocardiography is useful and reliable in the diagnosis and management of children with dilated cardiomyopathy. M-mode echocardiography provides quantitative information of left ventricular and left atrial dimensions and left ventricular wall thickness. Left ventricular function including shortening fraction, mean velocity of circumferential fiber shortening (V cf), systolic time intervals, left ventricular wall thickening and thinning rate, isovolumic contraction time, and wall stress can be derived from M-mode study. Left ventricular and left atrial dimensions are usually 1.5 times normal. Left ventricular systolic function is markedly reduced. Shortening fraction can be easily obtained and is the most informative index for assessing the severity of illness. Very low shortening fraction at presentation and follow-up (12-15%) is a poor prognostic sign. Two-dimensional echocardiography is valuable for excluding valvular lesion or coronary artery anomaly and detection of intracardiac thrombus. The left ventricular free wall is usually more severely affected than the ventricular septum as seen by two-dimensional echocardiography.     

Familial inflammatory dilated cardiomyopathy

BACKGROUND: Systematic family screening has recently identified dilated cardiomyopathy as an inherited disorder in up to 30% of cases. Mutations in genes encoding proteins responsible for myocardial architecture have been identified, but additional pathophysiological mechanisms including inflammatory reactions have been proposed. AIMS: Identification and characterization of familial DCM, where at least one affected family member fulfils the criteria for inflammatory DCM may lead to a better understanding of the aetiology and pathogenesis of (inflammatory) DCM. METHODS AND RESULTS: Ten families were examined. In six families, clinical characteristics and mode of inheritance were compatible with pure fDCM, fDCM with conduction defect and autosomal recessive fDCM. In four families, (auto-)immune features were diagnosed in affected and non-affected family members. CONCLUSIONS: Familial DCM with an inflammatory component was identified as a specific subgroup of familial DCM. In most cases, the inflammatory process seems to modify, i.e. aggravate, the "classic, cytoskeletopathic" familial DCM, but in some, especially when taking clinical and genetic aspects into account, inflammatory (auto-)immune features can be addressed as the leading pathogenetic principle. Further elucidation of these families may provide a better insight into pathophysiologic processes and may aid in the development of specific therapeutic strategies.     

Hereditary dilated cardiomyopathy

Dilated cardiomyopathy (DCM) is a common and important cause of morbidity and mortality. Many factors can contribute to the development of this disorder, although most commonly the etiology is unexplained. However, recent studies in individuals with idiopathic DCM now reveal a heritable cause in 20–30% of individuals. Diverse modes of inheritance have been demonstrated, encompassing an autosomal dominant type (by far the most common), together with recessive and X-linked forms, and maternal inheritance through mitochondrial DNA. The hereditary forms of DCM (HDCM) predominantly affect the left ventricle, although inherited abnormalities affecting primarily the right ventricle also are described. HDCM may occur as a primary cardiomyopathy, or secondary to inherited systemic metabolic or neuromuscular disorders. The causative genes for primary HDCM of the autosomal dominant and recessive types have not yet been discovered, but the combination of family pedigree analysis and phenotyping by echocardiography, together with new genetic techniques, should now allow their identification. Knowledge of the gene or genes responsible for HDCM would improve diagnostic accuracy, facilitate genetic counseling, advance understanding of pathogenesis, and provide the starting point for new methods of treatment. Because of the frequently heritable nature of DCM, it is of great importance that a diligent search for all potentially affected family members be undertaken.     

Creatine kinase isoforms as circulating markers of deterioration in idiopathic dilated cardiomyopathy

Background: A proportion of patients with dilated cardiomyopathy (DCM) may have ongoing myocardial damage secondary to viral or immune mediated myocardial inflammation. Hypothesis: The prognostic determinants identify patients with decreased survival but do not provide a measure of myocardial damage. To obtain an objective assessment of myocardial damage in DCM, we measured plasma levels of creatine kinase (CK), its isoenzymes (CK-MM and CK-MB), and separated the isoforms of CK-MM and CK-MB. Methods: The cohort consisted of 77 consecutive patients (61 men, 16 women) with DCM (World Health Organization criteria), aged 49 ± 14 years (range 19–60). Patients had been symptomatic for 29 ± 38 months (range 0.5–200 months) with 48 in New York Heart Association class I/II and 29 in class III/IV at the time of diagnosis. During median follow-up of 27 months from diagnosis (range 0.6–165), 50 patients remained clinically stable and 27 had deteriorated. Results: A significantly higher proportion of patients with DCM had abnormal MB2/MB1 ratio compared with normal volunteers (11,14% vs. 1, 1%, p = 0.003). Patients who deteriorated had higher MB2/MB1 ratio, (1.22 ± 0.62 vs. 0.85 ± 0.56;p = 0.01), and more frequently had abnormal MB2/ MB1 ratio (8,30% vs. 3,6%;p=0.004) and CK and CK-MM activities (5, 19% vs. 2, 4%;p = 0.03) than those who remained stable. Patients with DCM with high CK-MB activity had 3.13-fold increased odds of sudden death or need for cardiac transplantation (95% confidence interval 1.53–6.40, p = 0.008). Thus, CK measurements, in particular CK-MB isoforms, are markers of myocardial damage in a subset of patients with DCM and could be useful in investigating the possibility of persistent myocardial damage in these patients.     

Myocardial energetics in dilated cardiomyopathy

To assess hemodynamic and energetic effects of different drug interventions on idiopathic dilated cardiomyopathy (IDCM), we determined hemodynamic variables of myocardial oxygen consumption (MVO2) in 37 patients with IDCM. Hemodynamics were measured during routine left and right heart catheterization. MVO2 was analyzed from myocardial blood flow (measured by the argon method) and aortocoronary sinus blood oxygen difference. The hemodynamic variable which correlated best with MVO2 was shown to be the systolic stress time integral (STI). Four different representative compounds were tested with respect to their acute effects on myocardial energetics (MVO2/STI) in patients with IDCM who were in compensated heart failure (NYHA class II-III). The drug interventions were performed at rest. Intravenous injection of the vasodilator nitroprusside yielded a 35% reduction in STI and a 30% reduction in MVO2; in other words, the ratio MVO2/STI was not altered. Injection of the calcium sensitizer and phosphodiesterase inhibitor pimobendan also did not alter this ratio, as both STI (36%) and MVO2 (33%) were lowered. The profound reduction in STI (60%) seen with the phosphodiesterase inhibitor enoximone was accompanied by a much smaller decrease in MVO2 (19%); therefore, the ratio of MVO2/STI increased significantly. An increase of this ratio was also seen with the partial beta-1 receptor agonist xamoterol. However, in this case STI did not change, whereas MVO2 increased by 26%. In summary, vasodilation has energy-saving effects, whereas positive inotropism is an energy-consuming process. We conclude that the overall effect on myocardial energetics of a drug which possesses both positive inotropic and vasodilating properties depends on the balance of the two properties.     

Prognostic implication of myocardial texture analysis in idiopathic dilated cardiomyopathy.

BACKGROUND AND AIM: Abnormal myocardial acoustic properties have been reported in patients with idiopathic dilated cardiomyopathy (IDC). The aim of this study was to investigate the relationship between quantitative ultrasonic textural alterations of myocardium and clinical outcome in IDC. METHODS: Baseline clinical and echocardiographic variables were obtained from 28 patients with IDC. By using a videodensitometric approach, quantitative myocardial texture analysis was performed on images obtained from septum and posterior wall (PW). Cyclic variation (CV) index of mean gray level (MGL) was calculated according to the formula: (MGLdiast-MGLsyst)/MGLdiastx100. All patients were followed for an average of 11+/-5 months for the occurrence of cardiac death or repeated hospitalization due to worsening of heart failure. RESULTS: During follow-up, 10 patients experienced cardiac events (6 cardiac deaths and 4 heart failure events). The CV indexes of both septum and PW were significantly lower in patients with cardiac events than those of event free patients (6.8+/-9.6% vs. 13.6+/-8.2%, P<0.05 and 5.3+/-6.4% vs.15.7+/-7.2% P<0.001, respectively). Univariate analysis defined the following variables as predictors of outcome: PW-CV index (chi2=13.0, P=0.0003), transmitral E/A ratio (chi2=12.5, P=0.0004), symptom status (chi2=8.7, P=0.003), and septum-CV index (chi2=4.7, P=0.03). Multivariate stepwise regression analysis showed that the PW-CV index (chi2=7.5, P=0.006) and E/A ratio (chi2=6.5, P=0.01) were the independent predictors of outcome. The event-free survival rate of patients with PW-CV index <11% was significantly lower than those with an index > or = 11 (35.7% vs. 92.8%, P=0,001). CONCLUSION: The assessment of severely depressed CV index provides valuable prognostic information in patients with IDC.     

静息门控心肌灌注显像鉴别诊断缺血性和扩张型心肌病

目的:探讨静息门控心肌灌注显像(GMPS)对扩张型心肌病(DCM)和缺血性心肌病(ICM)的诊断价值.方法:对20例DCM和70例ICM进行~(99m)Tc-MIBI静息GMPS,观察2组心肌血流灌注情况及心功能指标.结果:ICM组心肌灌注显像呈节段性灌注异常;DCM组为非节段性分布的、散在的稀疏区.DCM组的左室射血分数比ICM组明显降低,舒张末心腔容积、收缩末心腔容积及心脏的重量比ICM组明显增大(P<0.01),差异有统计学意义.结论:~(99m)Tc-甲氧基异丁基异腈静息GMPS对DCM和ICM的诊断和鉴别诊断具有较高的应用价值.     

Electrocardiographic features differentiating dilated cardiomyopathy from hypertrophic cardiomyopathy

To determine the usefulness of electrocardiographic (ECG) features in differentiating between hypertrophic cardiomyopathy with features mimicking dilated cardiomyopathy (D-HCM) and true dilated cardiomyopathy (DCM), we compared ECGs of 52 consecutive patients (11 with D-HCM, 41 with DCM). Left atrial dimension, left ventricular internal dimension, and septal and posterior wall thickness were employed as echocardiographic indexes, while QRS duration, amplitude of RV₅ or V₆ + SV₁, number of abnormal Q waves, P-terminal force in V₁, and frontal plane QRS axis were used as ECG parameters. The patients with D-HCM demonstrated a larger number of abnormal Q waves (P < .0001), greater prolongation of QRS duration (P < .0001), and lower amplitude of RV₅ or V₆ + SV₁ (P < .0001). In all cases of D-HCM, atrial overload was observed and abnormal QRS axis in 9 (82%) of the 11 patients. These features were noted in 21 (51%) and 17 (41%), respectively, of the 41 DCM patients (P < .005 and P < .05, respectively). Despite significant differences in the echocardiographic parameters between D-HCM and DCM, excluding left ventricular end-diastolic dimension, ECG abnormalities were more significant between the two groups. The results indicate that ECG features are extremely useful in differentiation between DCM and D-HCM.     

扩张型心肌病心肌病变对心脏再同步化治疗的影响

目的采用前瞻性自身对照研究的方法,应用双核素同时采集法单光子发射计算机断层（SPECT）显像评价扩张型心肌病（DCM）心肌病变的程度与心脏再同步化治疗（CRT）效果之间的关系。方法13例DCM心力衰竭患者成功植入CRT,并于术前及术后半年行SPECT检查,半定量分析方法评价手术前、后左室心肌灌注、代谢变化情况。结果术后左室缺血区、坏死区质量百分比均较术前明显降低[（18．2±12．7）％vs（31．8±15．4）％,P〈0．05;（22．3±14．9）％VS（36．4±14．3）％,P〈0．051。术前坏死区质量百分比、总心肌灌注评分之和（F值）与CRT超声应答呈负相关（r=-0．578,P〈O．05;r=-0．619,P〈0．05）。心肌灌注0分的节段数（N。）与CRT临床症状好转呈正相关（r=0．81,P〈0．05）。术前Nn与术后左室收缩功能改善、左室逆转重塑呈正相关（r=-0．655,P〈0．05;r=0．719,P〈0．05）。结论术前心肌细胞坏死程度越大,CRT疗效越差;CRT治疗可使心肌局部血流灌注改善,部分心肌细胞代谢增强,心肌坏死程度较术前减轻。     

Haemochromatosis gene mutations in idiopathic dilated cardiomyopathy

BACKGROUND—Two common mutations of the haemochromatosis associated gene (HFE) (cys282tyr (C282Y) and his63asp (H63D)) have been implicated in haemochromatosis and as modulators in cardiovascular disease.
OBJECTIVE—To investigate the role of these mutations in the pathogenesis of idiopathic dilated cardiomyopathy.
DESIGN AND SETTING—Case-control and prospective cohort study of patients attending a cardiomyopathy unit in a tertiary referral cardiac centre.
METHODS—207 unrelated white patients with dilated cardiomyopathy, followed up for 259 patient years, and 200 controls were tested for HFE C282Y and H63D mutations by polymerase chain reaction and restriction digestion.
RESULTS—31/207 patients (15%) v 24/200 controls (12%) carried C282Y (adjusted odds ratio (OR) 1.2 (95% confidence interval 0.7 to 2.2)), 74/207 (36%) v 53/200 (27%) carried H63D (OR 1.6 (1.1 to 2.5)), and 10/207 (4.8%) v 4/200 (2%) were compound heterozygotes (OR 2.6 (0.8 to 8.5)). Four patients and six controls were H63D homozygous and one was C282Y homozygous. There was a progressive increase in mean serum iron ([Fe]) and transferrin saturations from patients with no mutation ([Fe] = 16.3 µmol/l, transferrin saturation = 23.7%) through H63D heterozygotes (17.5 µmol/l, 25.8%), C282Y heterozygotes (17.1 µmol/l, 26.6%), H63D homozygotes (20.0 µmol/l, 33.5%), compound heterozygotes (26.8 µmol/l, 41.7%), and C282Y homozygotes (34 µmol/l, 71%). At follow up (median 90 months) the rate of death or cardiac transplantation was 52/207 (25%). C282Y heterozygotes had less ventricular dilatation (mean (SD): 59.9 (1.7) mm v 64.9 (0.9) mm, p < 0.05), better fractional shortening (24 (1.7)% v 18.8 (1.4)%, p < 0.01), and a trend towards improved survival without transplantation. [Fe] and transferrin saturation did not correlate with disease severity and were not associated with reduced survival.
CONCLUSIONS—The frequency of the H63D mutation is significantly increased in patients with idiopathic dilated cardiomyopathy. As H63D has a relatively minor effect on iron status, the mechanism of this association may be unrelated to iron metabolism.


Keywords: dilated cardiomyopathy; genetics; haemochromatosis     

扩张型心肌病280例临床分析

目的:探讨近年来扩张型心肌病(DCM)的临床表现特点及预后。方法:对1998年1月~2004年6月收治的280例DCM患者的临床资料,以及其中187例(66·8%)的随访结果进行分析。结果:男221例(78·9%),女59例(21·1%),起病年龄为(48·0±14·2)岁;确诊时心功能NYHAⅠ~Ⅱ级者60例(21·4%),Ⅲ级者155例(55·4%),Ⅳ级者65例(23·2%);全部患者中8例(2·9%)为家族性DCM,起病年龄(35·4±11·4岁)明显小于散发患者(48·4±14·1)岁,P<0·01。253例(90·4%)患者出现各种心律失常,包括心房颤动71例(25·4%),多源室性期前收缩、短阵室性心动过速分别为90例(32·1%)、130例(46·4%),左束支传导阻滞64例(22·9%)。超声心动图显示,患者以左心房、左心室同时扩大(55·4%)或全心扩大(30·0%)为主;Ⅲ~Ⅳ级患者与Ⅰ~Ⅱ级者相比,左室舒张末期内径[(68·9±8·4)∶(63·8±7·8)mm]明显增大,左室射血分数[(31·8±10·1)%∶(41·8±12·0)%]显著降低,均P<0·01。药物治疗中,血管紧张素转化酶抑制剂和β-受体阻滞剂使用率分别为70·1%(199例)、60·0%(168例)。187例(66·8%)患者获得随访,随访时间(29·8±23·2)个月;随访中死亡50例,其中心力衰竭死亡27例(54·0%),猝死20例(40·0%);另有7例患者接受心脏移植治疗。在未行心脏移植情况下,患者确诊后1、2、5年生存率分别为93·4%、86·0%、65·3%,高于国内以往报道。8例家族性DCM患者5年生存率(40·0%)低于散发患者(68·5%),P<0·05。结论:DCM患者确诊时心功能多已达Ⅲ~Ⅳ级,伴有各种心律失常及心腔扩大;2年和5年生存率较以往已有改善,心力衰竭和猝死仍是主要死亡原因;2·9%的患者为家族性DCM,起病年龄较轻,预后较差。     

扩张型心肌病患者窦性心率震荡的研究

目的 探讨扩张型心肌病患者窦性心律震荡（HRT）的特征及其对预后的影响。方法 选择扩张型心肌病并室早的患者187例，选择同期有室早而无器质性心脏病100例作对照组，分别检测窦性心率震荡的初始（TO）和窦性心率震荡的斜率（TS）：将窦性心率震荡分类为HRT1（TO〈0且TS〉2．5ms／RR间期）、HRT2（TO≥0或TS≤2．5ms／RR间期）、HRT3（TO≥0且TS≤2．5ms／RR间期），分别对不同心功能分级，存活组及死亡组进行分析。结果 扩张型心肌病与对照组比较TO、TS均有显著性差异（P〈0．001和P〈0．005）。在心功能Ⅲ级与Ⅳ级时HRT2,在两组间无显著性差异（p〉0．05），其他不同分级心功能的窦性心率震荡分类均有显著差异（p〈0．05或p〈0．01）。存活组与死亡组比较窦性心率震荡分类亦有显著性差异（HRT1p〈0．01，HRT2p〈0．05，HRT3p〈0．01）。结论 扩张型心肌病患者存在室早后窦性心率震荡减弱或消失现象，窦性心率震荡对其预后有一定的预测价值。     

C-reactive protein and aortic stiffness in patients with idiopathic dilated cardiomyopathy

BACKGROUND: Previous studies have shown an association between C-reactive protein (CRP)and arterial stiffness in most cardiovascular diseases. Increased CRP levels and arterial stiffness have been considered independent predictors of cardiovascular mortality in cardiovascular disease and even in the general population. OBJECTIVE: The aim of this study was to investigate the relationship between CRP, a marker of systemic inflammation and aortic stiffness in patients with idiopathic dilated cardiomyopathy (DCMP). METHODS: Serum CRP levels and aortic stiffness parameters were measured in DCMP patients (n= 37) and age- and gender-matched control subjects (n= 30). High-sensitivity CRP levels were determined by an immunonephelometry assay. Aortic strain (AS) and aortic distensibility (AD) were calculated from the aortic diameters measured using M-mode echocardiography and blood pressure obtained by sphygmomanometry. RESULTS: Serum levels of CRP in DCMP patients were higher than in the control subjects (5.47 +/- 2.06 mg/L and 2.35 +/- 0.47 mg/L, P < 0.001, respectively). AS and AD were significantly decreased in DCMP patients compared to the controls (P < 0.001 and P < 0.001, respectively). There were positive correlations between CRP, and (r = 0.3.64, P = 0.027) smoking (r = 0.3.56, P = 0.024), and increasing age (r = 0.587, P < 0.001), and negative correlations between CRP, and DBP (r =-0.485, P < 0.001), diameter change (DC; r =-0.493, P < 0.001), AS (r =-0.526, P < 0.001), and AD (r =-0.626, P < 0.001). CONCLUSION: We have shown that there is a significant relation between high serum CRP levels and impaired aortic stiffness in patients with idiopathic DCMP. These findings may indicate an important role of CRP in the pathogenesis of impaired aortic stiffness in idiopathic DCMP.     

口服黄芪合剂治疗扩张型心肌病室性心律失常

目的:探讨黄芪合剂对扩张型心肌病(DCM)室性心律失常长期疗效及相关机制.方法:选择65例稳定性DCM室性心律失常患者,根据病毒学检查结果分为阳性组(36例)和阴性组(29例).在常规治疗基础上,阳性组中有25例接受口服黄芪合剂治疗半年(即阳1亚组),11例拒绝而未接受黄芪口服治疗(即阳2亚组);阴性组中有18例接受黄芪治疗(即阴1亚组),11例未接受黄芪治疗(即阴2亚组).治疗前后应用Holter检查观察患者室性心律失常变化、心脏彩色超声多普勒评估心脏左室舒末内径(LVEDd)和左室射血分数(LVEF)、ELISA法检测外周血细胞因子IFN-γ、白细胞介素-4(IL-4)与转化生长因子(TGF)-β1水平.结果:阳1亚组和阴1亚组中分别有3例和2例未坚持服用黄芪或失访而退出.与治疗前相比,阳1亚组、阳2亚组、阴1亚组、阴2亚组室性心律失常改善率分别为:54.5％(12/22)、18.2％(2/11)、43.8％ (7/16)、27.3％(3/11),阳1亚组与阳2亚组比较,差异有统计学意义(P＜0.05);阳1亚组和阴1亚组LVEDd均有缩小(P＜0.05)、LVEF均有升高(P＜0.05),血IFN-γ和TGF-β1水平显著下降(P＜0.01),而IL-4变化无统计学意义;阳2亚组和阴2亚组也有相似表现,但是LVEDd、LVEF和IL-4变化均差异无统计学意义.结论:口服黄芪合剂可以有效控制DCM室性心律失常,其机制可能与降低患者IFN-γ和TGF-β水平、抑制心肌纤维化有关.     

Reversal of dilated to hypertrophic cardiomyopathy after alcohol abstinence

Left ventricular dilatation and systolic dysfunction develop in 14-16% of patients with hypertrophic cardiomyopathy. Such findings may easily be misdiagnosed as dilated cardiomyopathy. It is unknown whether left ventricular dilatation and systolic dysfunction in patients with hypertrophic cardiomyopathy are reversible. A 35-year-old man had been a heavy drinker for 13 years and was abstinent for 1 year. Five years previously he suffered cardiac arrest and, based on echocardiographic, radionuclide, and cardiac catheterization findings, the diagnosis of alcohol-induced dilated cardiomyopathy was established. At presentation the heart was of normal size, with concentric left ventricular hypertrophy and only slightly reduced systolic function. Hypertrophic cardiomyopathy was diagnosed since no other cause for left ventricular hypertrophy could be detected. In hypertrophic cardiomyopathy, alcohol may induce reversible systolic dysfunction and left ventricular dilatation.     

ECG characteristics of dilated cardiomyopathy

To elucidate the electrocardiographic (ECG) characteristics of dilated cardiomyopathy (DCM), the authors analyzed the 12-lead ECGs and echocardiograms in 45 patients with DCM, 54 patients with left ventricular (LV) dilatation secondary to valvular heart disease (VHD), 101 hypertensive patients with LV hypertrophy, and 63 normal control subjects. In addition, serial ECG and echocardiographic changes in DCM during a mean follow-up period of 1.6 years were evaluated. Sokolow's criterion (S wave in lead V₁ [SV₁] + R wave in lead V₅ or V₆ [RV₅ or RV₆] > 35 mm) was met comparably in patients with DCM (69%), VHD (61%), and hypertension (74%) (P = NS). Notably, RV₆ in DCM was the highest among the four groups and correlated with the degree of LV dilatation. In contrast, the R waves in leads I, II, and III (RI, RII, RIII) in DCM were the lowest and were not affected by the degree of LV dilatation, although RII and RIII in VHD and RI in hypertension correlated with the degree of LV dilatation and hypertrophy, respectively. As a result, all voltage ratios of RV6/RI, RII, RIII in DCM were not only the highest, but also increased linearly as the LV dilated progressively during the follow-up period. In particular, RV₆ over the maximum R wave in leads I, II, and III (RV₆/Rmax) in DCM correlated with the degree of LV dilatation and inversely correlated with ejection fraction. Subjects with DCM had a significantly higher RV₆/Rmax than did patients with VHD, hypertension, and normal subjects (3.4 vs 1.7, 1.4, 1.2, respectively; P < .001), and this ratio of ≥3 was seen in 67% of the DCM patients versus 4% of the VHD patients, 1% of the hypertensive patients, and 0% of the normal subjects. Thus, DCM commonly shows the ECG signs of LV hypertrophy, but characteristically has the high voltage ratios of RV6/RI, RII, RIII.     

Comparison of noninvasive measures of contractility in dilated cardiomyopathy

Left ventricular performance is usually quantified by ejection phase indices such as ejection fraction, cardiac output, and fractional shortening. The load-dependence of these measures may result in inaccurate estimation of intrinsic myocardial contractility in states of chronic pressure or volume overload. End-systolic and stress-shortening relations have been proposed as measures of contractile state insofar as they are theoretically independent of preload and incorporate afterload. This article examines the behavior of these relations in response to changes in loading conditions and contractile state and reviews their application utilizing noninvasive methodology, particularly in the setting of dilated cardiomyopathy.     

扩张型心肌病抗心肌抗体的临床观察及其针对性治疗

目的:探讨扩张型心肌病(DCM)的发病机制,观察针对抗心肌抗体进行免疫学治疗的临床疗效和预后.方法:对2001年1月-2007年12月入院治疗的DCM患者(747例)的病史、诊治过程、随访情况进行回顾性分析.结果:747例中抗心肌抗体阳性者527例(70.55%),随访时间为0.9-7.2年,抗心肌线粒体ADP/ATP载体抗体阳性患者(A组)治疗后平均左室舒张末期内径(LVEDd)为(62.53±9.17)mm,左室射血分数(LVEF)为(37.65±11.15)%;抗β1-肾上腺素能受体抗体(抗β1-受体抗体)阳性患者(B组)治疗后LVEDd (61.35±5.68)mm, LVEF (40.65±12.78)%;抗ADP/ATP载体抗体和抗β1-受体抗体均阳性患者(C组)治疗后LVEDd(61.28±7.72)mm, LVEF(38.35±7.05)%.3组与治疗前比较LVEDd差异均有统计学意义(P<0.05)、NYHA心功能分级均改善Ⅰ～Ⅱ级.结论:自身免疫是DCM的常见致病因素,针对抗ADP/ATP载体抗体使用地尔硫和针对抗β1-受体抗体使用美托洛尔治疗DCM均可明显改善患者心脏功能及心室重构,改善预后.     

Prognosis of idiopathic dilated cardiomyopathy

Previous reports in referral populations have emphasized the poor prognosis of dilated cardiomyopathy. This study evaluated mortality and morbidity in patients presenting at a referral center between 1989 and 1993. One hundred seventy-two consecutive patients were studied. At presentation, 82 were in New York Heart Association functional class III/IV. Mean (+/- SD) left ventricular end-diastolic dimension was 69 +/- 11 mm, ejection fraction was 25 +/- 10%, VO2 max was 21 +/- 9 mL/min/kg, and sodium was 136 +/- 9 mM. Treatments included vasodilators (n = 157, 92%), anticoagulation (n = 50, 29%), amiodarone (n = 52, 30%), and cardiac defibrillator (n = 5, 3%). During the follow-up period (mean, 26 +/- 29 months), 16 patients died and 60 developed progressive heart failure; 46 (27%) required cardiac transplantation. The majority of the patients (102, 59%) were stable or improved. Established prognostic determinants (left ventricular end-diastolic dimension, ejection fraction, sodium, and arrhythmia) were of low predictive value for the development of progressive heart failure or sudden death. The 1- and 2-year probabilities of death or transplantation was 16 and 21%, respectively (death only 6 and 7%, respectively). These observations are subject to referral bias, but suggest that the majority of patients can remain stable. Any improvement in survival compared to earlier experience can be due to earlier diagnosis, availability of transplantation, and new heart failure management strategies.     

扩张型心肌病患儿心率变异改变及其与心脏大小和功能的关系

目的探讨扩张型心肌病（DCM）患儿心率变异（HRV）改变及其与心脏大小和收缩舒张功能的相关性。方法对30例DCM患儿（DCM组）和30例健康儿童（对照组）行24h动态心电图检查,并记录HRV指标（SDNN、SDANN、SDNN index、RMSSD、PNN50、TP、LF、HF、LF／HF）;同时行超声心动图检查,测量左室舒张末期内径（LVED）、左房舒张末期内径（LAED）、左心室射血分数（LVEF）、短轴缩短率（LVFS）和二尖瓣E／A峰比值,并分析这两类指标之间的相关性。结果DCM组HRV指标除LF／HF较对照组升高外,其余均较对照组明显降低;SDNN与LAED、LVEF、LVFS和E／A呈中度相关,其余指标与心脏大小和收缩舒张功能无明显的相关性。结论DCM患儿存在明显的自主神经平衡失调,主要表现为迷走神经张力减退,交感神经的相对激活;HRV与心脏大小和收缩舒张功能指标相对独立,其作为一无创性检测指标,可从一个侧面反映DCM患儿的预后,将它们结合起来能更好地指导临床。