Apical Left Ventricular Hypertrophy and Mid‐Ventricular Obstruction in Fabry Disease

We report the case of a rare cardiac presentation of Fabry disease. Although concentric left ventricular hypertrophy is a major cardiac finding in Fabry disease, there is no case report of dynamic obstruction at mid‐left ventricular level. We describe a 59‐year‐old‐woman suffering from a severe form of Fabry disease, mimicking an apical hypertrophic cardiomyopathy with mid‐ventricular obstruction. Differentiation of Fabry disease from hypertrophic cardiomyopathy is crucial given the therapeutic and prognostic differences. Fabry disease should always be suspected in an adult, independently of the pattern of left ventricular hypertrophy.     

左心室肥厚心电图诊断标准分析

目的:对比左心室肥厚心电图诊断标准的敏感性和特异性,以评价不同标准的诊断价值.方法:分析364例原发性高血压患者的超声心动图及心电图检查结果.以超声心动图对患者左心室肥厚情况的检查结果为参照,求得不同心电图标准(Sokolow-Lyon标准、Comell标准、Romhilt-Estes评分、Framingham标准及Perugia标准)诊断左心室肥厚的敏感性和特异性.结果:5项心电图诊断标准的敏感性均＜50%;而特异性较高,除Perugia标准外,余4项标准均＞90%.Perugia标准的敏感性最高41%,特异性低89%;Sokolow-Lyon标准的敏感性22%及特异性93%均较低.结论:心电图诊断左心室肥厚的特异性高、敏感性稍差.Perugia标准是Comdl标准、左心室劳损改变、Romhilt-Estes评分等标准有机的结合,因此提高了心电图诊断的敏感性,而特异性未受到明显影响.     

左心室肥厚的影响因素及机制的研究进展

生理和病理条件下,血流动力学超负荷会引起左心室肥厚。目前尚未完全阐明引起心肌肥厚的主要的刺激因素是心脏自身机械拉伸,还是神经体液因子,抑或是两者的共同作用所致。刺激因素进入细胞后,通过引起细胞内生化改变导致第二信使和第三信使的激活,进一步调节转录,激活多种心肌肥厚相关基因的表达。左心室肥厚时会出现一系列的结构改变,包括心肌细胞肥大、间质结缔组织增生以及冠状循环微血管稀少。本文参考近年来相关文献,总结左心室肥厚的影响因素及机制研究方面的发展动态,旨在为读者阐明左心室肥厚的影响因素及机制的总体情况,提供可参考的研究靶点。     

高血压左室肥厚的不同构型

高血压544例（男性336）和无高血压的配对组204例（男性106），应用心脏超声法对左室构型进行研究，依据相对室壁厚度、左室质量指数的分布、前室间隔及其基底部肥厚程度。6种左室构型检出率分别为:正常左室构型，男女分别占52％，42%；离心性肥厚占16％，25％，性别差异显著（P＜0.001）；向心性重构，男女各占10%，9％；向心性肥厚7％，8％；非对称性室间隔肥厚及前室间隔基底部肥厚型，分别占7%，9％。研究表明左室构型与年龄、性别、病程及收缩压有一定关系，但左室肥厚类型的形成可能取决于心脏内在因素，向心性肥厚与离心性肥厚之间不一定有依从关系。     

The Effects of Simvastatin on Left Ventricular Hypertrophy and Left Ventricular Function in Patients with Essential Hypertension

This study is to evaluate the effects of Simvastatin on left ventricular hypertrophy and left ventricular function in patients with essential hypertension. Untreated or noncompliance with drug treatment patients with simple essential hypertension were treated with a therapy on the basis of using Telmisartan to decrease blood pressure (BP). There were 237 patients who had essential hypertension combined with left ventricular hypertrophy as diagnosed by echocardiography, taken after their BPs were decreased to meet the values of the standard normal. Among them, there were only 41 out of the original 237 patients, 17.3%, who had simple essential hypertension combined with left ventricular hypertrophy without any other co-existing disease. They were the patients selected for this study. All patients were randomly, indiscriminately divided into two groups: one was the control group (Group T), treated with the Telmisartan-based monotherapy; the other was the target group (Group TS), treated with the Telmisartan-based plus simvastatin therapy. The changes of left ventricular hypertrophy and left ventricular function were rediagnosed by echocardiography after 1 year. The results we obtained from this study were as follows: (i) The average BPs at the beginning of the study, of simple essential hypertension combined with left ventricular hypertrophy, were high levels (systolic blood pressure (SBP) 189.21 ± 19.91 mm Hg, diastolic blood pressure 101.40 ± 16.92 mm Hg). (ii) The Telmisartan-based plus simvastatin therapy was significantly effective in lowering the SBP (128.26 ± 9.33 mm Hg vs. 139.22 ± 16.34 mm Hg). (iii) After the 1-year treatment, the parameters of left ventricular hypertrophy in both groups were improved. Compared to group T, there were no differences in the characteristics of the subjects, including interventricular septum, left ventricular mass, left ventricular mass index, ejection fraction, left atrium inner diameter at baseline. The patients’ interventricular septum (Group TS 10.30 ± 1.80 mm vs. Group T 10.99 ± 1.68 mm, P < .05), LVM (Group TS 177.43 ± 65.40 g vs. Group T 181.28 ± 65.09 g, P < .05), and LVMI (Group TS 100.97 ± 37.33 g/m² vs. Group T 106.54 ± 27.95 g/m², P < .05), all dropped more prominently (P < .05) in group TS; the ejection fraction rose more remarkably in group TS (Group TS: 57.50 ± 16.41% to 65.43 ± 11.60%, P < .01 while showing no change in Group T); the left ventricular hypertrophy reversed more significantly and the left ventricular systolic function improved more. (iv) The left atrium inner diameter of Group TS decreased (P < .01), the ratio of E/A, which indicates the left ventricular diastolic function, continued to drop further, showing no change to the trend of left ventricular diastolic function declination. Patients who have hypertension with left ventricular hypertrophy usually suffer other accompanying diseases at the same time. Telmisartan-based plus Simvastatin treatment can significantly reduce SBP, reverse left ventricular hypertrophy, improve the left ventricular systolic function, but it has no effect on reversing the left ventricular diastolic function. This experiment indicated that Simvastatin can reverse left ventricular hypertrophy and improve left systolic function.     

Fabry Disease on Peritoneal Dialysis with Cardiac Involvement

Fabry disease (FD) is an X-linked lysosomal storage disorder resulting from a lack of alpha-galactosidase A (AGALA) activity in lysosomes. We herein report a patient with FD revealed by a renal biopsy who survived seven years after the introduction of peritoneal dialysis despite having severe heart failure due to left ventricular hypertrophy (LVH). FD was diagnosed based on a renal biopsy and biochemical analysis showing a low enzymatic activity of AGALA. A microscopic examination at the autopsy revealed marked hypertrophy and vacuolation of cardiac muscle cells. In our case, cardiac involvement determined the prognosis. Peritoneal dialysis is the modality of choice in the long-term management of dialysis patients with FD.     

厄贝沙坦氢氯噻嗪联合瑞舒伐他汀治疗原发性高血压伴左心室肥厚的疗效研究

目的观察厄贝沙坦氢氯噻嗪(安博诺)联合瑞舒伐他汀治疗原发性高血压(EH)伴左室肥厚(LVH)的疗效。方法选取2011年2月—2012年12月我院收治的EH伴LVH患者95例,将其随机分为对照组47例和治疗组48例。对照组常规服用安博诺治疗,治疗组在对照组基础上加用瑞舒伐他汀治疗,两组均治疗6个月。观察治疗前后两组舒张末期室间隔厚度(IVST)、舒张末期左室后壁厚度(PWT)、舒张末期左室内径(LVDd)、左心室质量指数(LVMI)等的变化,并比较不良反应发生情况。结果治疗前两组LVDd、IVST、PWT、LVMI比较,差异均无统计学意义(P0.05);治疗后治疗组LVDd、IVST、PWT、LVMI均低于对照组(P0.05)。对照组不良反应发生率为6.4%(3/47),治疗组为4.2%(2/48),两组不良反应发生率比较,差异无统计学意义(P0.05)。结论安博诺联合瑞舒伐他汀能有效逆转EH患者的LVH,改善左心室功能,较单一用药疗效显著。     

Influence of Left Ventricular Hypertrophy on In-Hospital Outcomes in Acute Exacerbation of Chronic Obstructive Pulmonary Disease

Left ventricular hypertrophy (LVH) is associated with worse outcomes in chronic obstructive pulmonary disease (COPD); however, its role in an acute exacerbation of COPD (AECOPD) has not been reported. This was a retrospective cohort study during 2008–2012 at an academic medical center. AECOPD patients >18 years with available echocardiographic data were included. LVH was defined as LV mass index (LVMI) >95 g/m² (women) and >115g/m² (men). Relative wall thickness was used to classify LVH as concentric (>0.42) or eccentric (<0.42). Outcomes included need for and duration of non-invasive ventilation (NIV) and mechanical ventilation (MV), NIV failure, intensive care unit (ICU) and total length of stay (LOS), and in-hospital mortality. Two-tailed p < 0.05 was considered statistically significant. Of 802 patients with AECOPD, 615 patients with 264 (42.9%) having LVH were included. The LVH cohort had higher LVMI (141.1 ± 39.4 g/m² vs. 79.7 ± 19.1 g/m²; p < 0.001) and lower LV ejection fraction (44.5±21.9% vs. 50.0±21.6%; p ≤ 0.001). The LVH cohort had statistically non-significant longer ICU LOS, and higher NIV and MV use and duration. Of the 264 LVH patients, concentric LVH (198; 75.0%) was predictive of greater NIV use [82 (41.4%) vs. 16 (24.2%), p = 0.01] and duration (1.0 ± 1.9 vs. 0.6 ± 1.4 days, p = 0.01) compared to eccentric LVH. Concentric LVH remained independently associated with NIV use and duration. In-hospital outcomes in patients with AECOPD were comparable in patients with and without LVH. Patients with concentric LVH had higher NIV need and duration in comparison to eccentric LVH.     

Hypertensive Left Ventricular Hypertrophy: Pathophysiology, Assessment and Treatment

Otterstad JE, Smiseth O, Kjeldsen SE. Hypertensive Left Ventricular Hypertrophy: Pathophysiology, Assesment and Treatment.

Left ventricular hypertrophy (LVH) is a strong predictor of cardiovascular morbidity and mortality. LVH is associated with coronary events, and there is an association between cerebrovascular disease and increased left ventricular mass (LVM). Experimental studies have elucidated the importance of non-myocytic cells inducing increased perivascular and interstitial fibrosis along with thickening of the media of intramyocardial coronary arteries in hypertensive LVH. M-mode echocardiography is the most accepted standard for the diagnosis and quantification of LVH, but some controversies exist regarding the ideal methodology for serial assessment of LVM. It is still a matter of debate whether 2-dimensional echo measurements represent a more accurate method. Hopefully, both the introduction of 3-dimensional echo and new Doppler techniques can provide more accurate measurements of LVM and additional information on changes in myocardial fibrosis and stiffness. Experimental studies have shown that normalization of hypertensive myocardial and coronary artery remodelling take place with drugs like angiotensin converting enzyme (ACE)-inhibitors and calcium antagonists. Two meta-analyses suggest that ACE-inhibitors may be the most efficient drugs in reducing LVM, but a clinical correlate to this assumption is at present not available. There are some indications that regression or progression of LVH assessed by ECG and echocardiography may in fact be related to the incidence of cardiovascular events. But large-scale controlled studies of various treatment regimens are still needed to establish whether drug induced regression can improve the prognosis of hypertensive LVH independent of the antihypertensive effect.     

Left Ventricular Noncompaction in a Family with Lamin A/C Gene Mutation

Left ventricular noncompaction is a rare type of cardiomyopathy, the genetics of which are poorly understood to date. Lamin A/C gene mutations have been associated with dilated cardiomyopathy and diseases of the conduction system, but rarely in left ventricular noncompaction cardiomyopathy. This report describes the cases of 4 family members with a lamin A/C gene mutation, 3 of whom had phenotypic expression of left ventricular noncompaction.     

高盐饮食对大鼠左室肥厚的影响

目的　观察高盐饮食对原发性高血压大鼠 (SHR)及正常血压大鼠 (WKY)左室肥厚的影响 ,并探讨其可能机制。方法　SHR和WKY各 2 0只分别分为两组 :( 1)高盐饮食组SHRSL(n =10 )和WKYSL(n =10 )饮用含 2 %NaCl盐水 ;( 2 )正常盐饮食组SHRNS(n =10 )和WKYNS(n =10 )饮用不含NaCl清水 ,共饲养 6周。测量左室重量指数 (LVI) ,心肌细胞直径(CMD) ,放免测定循环ANP以及心肌局部ET 1含量。结果　 ( 1)左室重量指数及心肌细胞直径SHRSL较SHRNS显著增加(P <0 0 1) ,WKYSL较WKYNS有所增加 ,但差异不显著 (P >0 0 5 ) ;( 2循环中ANP浓度WKYSL较WKYNS明显升高 (P <0 0 1) ,SHRSL较SHRNS无明显差别 (P >0 0 5 ) ;( 3 )左室局部ET 1:SHRSL较SHRNS显著增加 (P <0 0 1) ,WKYSL较WKYNS无明显差别 (P >0 0 5 )。结论　高盐饮食可引起SHR左室肥厚 ,盐负荷后SHR心肌局部ET 1含量的增加可能是SHR左室肥厚的原因之一     

单纯收缩期高血压患者左心室肥厚与室性心律失常的关系

目的探讨单纯收缩期高血压的病人左室肥厚与心律失常的关系。方法采用２４ｈ动态心电图和彩色多普勒超声观察患者左室肥厚及心律失常的发生情况，并观察单纯收缩期高血压与舒张期高血压左室结构改变。结果左室肥厚组室性心律失常发生率明显高于非左室肥厚组（Ｐ＞０．０１），单纯收缩期高血压组左室肥厚高于舒张期高血压组。结论左室肥厚可使室性心律失常发生率增加，收缩压增高较舒张压增高更易导致左室肥厚。     

间硝苯地平对左室肥厚大鼠左室功能的保护作用

观察间硝苯地平（m-Nif，ig20mg／kg·d~（-1），持续9w)长期治疗对老龄肾性高血压左室肥厚（LVH）大鼠左室功能的影响，与假手术大鼠相比，LVH大鼠左室顺应性和左室发展压（LVDP）显著下降，左室僵硬度常数显著增高（P＜0.01），m-Nif可显著改善老龄LVH大鼠左室顺应性，使左室僵硬度常数降低，LVDP值增加。表明m-Nif对老龄LVH大鼠左室舒缩功能具有改善作用。     

高血压病程对左室肥厚和功能的影响

采用超声心动图对高血压病患者进行检测，以观察不同病程对左室肥厚（LVH）及功能参数的影响。高血压甲组（病程≤10年）及乙组（病程＞10年）的室间隔厚度（IVSTd），左室后壁厚度（PWPd）、左室舒张期内径（LVEDD）和左室重量指数（LVWI）均明显高于血压正常组。高血压乙组的IVSTd，LVEDD和LVWI亦明显高于甲组。高血压组IVH总的检出率为40．8％。甲、乙两组分别为33．3％和48．9％，LVH类型在两组间无明显差别。高血压甲、乙两组的左室舒张功能受损，主要表现为二尖瓣舒张晚期血流速度峰值（A）增加,E／A比值减少（E为早期峰值）。高血压乙组的左室射血分数（LVEF）较正常血压组低，甲组与正常血压组无差别。结果揭示，高血压病程是影响LVH和左室功能的一个重要因素。     

高血压左室肥厚诊断的探讨

随机选择104例高血压患者（血压≥160／95mmHg）进行心电图（ECG）和超声心动图（UCG）检查。结果UCG左心室肥厚（LVH）检出率为76％，ECGLVH检出率为24％，ECGLVH患者的左心室重量指数（LVMI）显著增大，伴左心宝功能不全者的LVMI也明显增大，多元相关分析示LVMI与舒张末期左心室内径、室间隔厚度、左室后壁厚度呈显著正相关（P＜0．001）。