黏附分子与扩张型心肌病

黏附分子介导的细胞黏附是基本的生命现象 ,是细胞的生长及分化、炎症、血栓形成等一系列重要生理和病理过程的基础 ;扩张型心肌病一直是心血管领域的难点 ,其发病机制包括病毒的持续感染致心肌炎转化和自身免疫等。黏附分子在扩张型心肌病发生及发展中起着一定的重要作用 ,抗黏附治疗也成为扩张型心肌病防治的重要方面。     

123I]-phenylpentadecanoic acid uptake in patients with dilated cardiomyopathy

BACKGROUND: The rate constant for global fatty acid influx (k(1)) was studied in 12 male patients with dilated cardiomyopathy (DCM). METHOD: 10 normal subjects served as controls. 201-Thallium (201TI) and [123I]-phenyl-pentadecanoic acid (IPPA) were administered during bicycle exercise under fasting conditions. RESULTS: All patients showed non-homogeneous tracer uptake defects for 201TI and IPPA. k(1) was significantly higher in DCM patients than controls. k(1) showed significant inverse correlation between cardiac index, left-ventricular ejection fraction, left-ventricular enddiastolic pressure and echocardiographic left-ventricular ejection fraction. CONCLUSION: We presume that an increased regional rate constant of IPPA influx into the myocardial tissue in patients with DCM reflects a compensatory mechanism of altered myocardium.     

Primary carnitine deficiency: novel mutations and insights into the cardiac phenotype

Solute carrier family 22 member 5 (SLC22A5) encodes a sodium‐dependent ion transporter responsible for shuffling carnitine across the plasma membrane. This process provides energy for the heart, among other organs allowing beta‐oxidation of fatty acids. Mutations in SLC22A5 result in primary carnitine deficiency (PCD), a disorder that manifests with cardiac, skeletal, or metabolic symptoms. We hereby describe two novel mutations in SLC22A5 in two Lebanese families associated exclusively with a cardiac phenotype. The frequency of the cardiac, metabolic and skeletal symptoms in PCD patients remains undefined. All the reported eight PCD patients belonging to five different Lebanese families have an exclusive cardiac phenotype. Carnitine levels appear to be directly linked to the type and position of the mutation and the severity of the phenotypic presentation does not seem to be associated with serum carnitine levels. A comprehensive review of 61 literature‐reported PCD cases revealed an exclusive cardiac manifestation frequency at 62.3% with a very low likelihood of simultaneous occurrence of cardiac and metabolic manifestation.     

Prognostic value of pulsed tissue Doppler imaging for the assessment of left ventricular systolic function in patients with nonischemic dilated cardiomyopathy

There is still some debate regarding the prognostic significance of left ventricular longitudinal systolic dysfunction as assessed by tissue Doppler (TD) imaging in patients with chronic heart failure (HF), since previous studies have included patients with postischemic wall motion abnormalities. Thus, this study was designed to ascertain whether TD-derived longitudinal systolic dysfunction may influence the outcome of patients with nonischemic chronic HF. In 200 consecutive patients with chronic HF secondary to dilated cardiomyopathy and no history of ischemic heart disease, peak systolic mitral annular velocity (S(m) ) was measured by pulsed TD at the septal and lateral annular sites. The end points were cardiac death or hospitalization for worsening HF. Mean follow-up duration was 30 months. In a time independent analysis, averaged S(m) calculated as the average of septal and lateral S(m) , resulted to be a significant predictor of outcome in the study population (area under receiver-operator characteristic curve: cardiovascular death, 0.69, P < 0.0001; cardiovascular events, 0.64, P = 0.0005). In a time-dependent analysis, average S(m) was associated with both cardiovascular death (hazard ratio 0.832, P = 0.0019) and cardiovascular events (hazard ratio 0.904, P = 0.039), independently of other clinical risk factors and echocardiographic parameters of systolic function. Septal S(m) but not lateral S(m) was independently associated with the outcome measures. In conclusion, the assessment of systolic mitral annular velocity by pulsed TD is a useful indicator for prognostic stratification of patients with nonischemic dilated cardiomyopathy and chronic HF.     

参麦注射液治疗扩张型心肌病心力衰竭疗效观察

目的观察参麦注射液治疗扩张型心肌病心力衰竭的疗效。方法选择扩张型心肌病心力衰竭患者80例,分为对照组和治疗组。对照组给予常规药物治疗,治疗组在常规药物治疗基础上加用参麦注射液。结果两组治疗后较治疗前临床症状以及心脏彩超各参数(射血分数、左室内径)以及6min步行试验均有改善。治疗后两组相比差别有统计学意义(P<0.05)。结论参麦注射液可有效治疗扩张型心肌病心力衰竭,安全性较高,无明显不良反应。     

Partial left ventriculectomy improves left ventricular end systolic elastance in patients with idiopathic dilated cardiomyopathy

OBJECTIVE: To assess the effect of partial left ventriculectomy (PLV) on estimate of left ventricular end systolic elastance (Ees), arterial elastance, and ventriculoarterial coupling. PATIENTS: 11 patients with idiopathic dilated cardiomyopathy before and two weeks after PLV, and 11 controls. INTERVENTIONS: Single plane left ventricular angiography with simultaneous measurements of femoral artery pressure was performed during right heart pacing before and after load reduction. RESULTS: PLV increased mean (SD) Ees from 0.52 (0.27) to 1.47 (0.62) mm Hg/ml (p = 0.0004). The increase in Ees remained significant after correction for the change in left ventricular mass (p = 0.004) and end diastolic volume (p = 0.048). As PLV had no effect on arterial elastance, ventriculoarterial coupling improved from 3.25 (2.17) to 1.01 (0.93) (p = 0.017), thereby maximising left ventricular stroke work. CONCLUSION: It appears that PLV improves both Ees and ventriculoarterial coupling, thus increasing left ventricular work efficiency.     

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.     

1例疑似心肌炎的儿童扩张型心肌病外科诊治报告

【摘要】目的：报告1例疑似心肌炎的儿童扩张型心肌病（dilated cardiomyopathy，DCM）在体外膜肺氧合（Extracorporeal membrane oxygenation，ECMO）辅助下成功原位心脏移植的案例并分享诊治经验。方法：对2019年武汉协和医院心外科收治的1例诊断为扩张型心肌病在ECMO辅助下行原位心脏移植的患儿的临床资料进行分析，查阅文献分析病因诊断及诊治经过。结果：7岁儿童因“咳嗽21天，加重伴心慌、呼吸困难10天”，由外院带ECMO和气管插管转入我院。患儿内科药物治疗及ECMO辅助14天后心功能仍差，结合病史、诊治经过及检查资料考虑扩张型心肌病，行原位心脏移植后顺利撤除ECMO，术后心肌病检结果支持诊断。结论：病因诊断对治疗策略的选择至关重要。目前国内儿童DCM以心脏移植为主要治疗手段。