肥厚型心肌病患者左室局部与整体舒张功能的应变率评价

目的:评价应变率成像定量检测肥厚型心肌病(HCM)患者左室局部与整体舒张功能的价值。方法:HCM患者33例,根据室间隔及室壁厚度分为非肥厚组(NH)、轻度肥厚组(MH)、重度肥厚组(SH);对照组31例。测量二尖瓣口血流播散速度(FPV);再获取其心尖四腔、两腔组织速度成像(TVI)图像脱机分析,测量参数:①舒张早期峰值应变(SRe);②舒张晚期峰值应变(SRa);③计算SRe/SRa。结果:与对照组比较,HCM组NH、MH、SH亚组SRe及SRs/SRe均显著降低,且NH>MH>SH;MH、SH组SRa显著降低,而NH组较对照组无显著差异,且NH>MH>SH;NH与MH组间SRe/SRa差异无统计学意义,与NH、MH组比较,SH组SRe/SRa显著减低;与对照组比较,HCM组所有室壁节段平均SRe、SRa及SRe/SRa均显著降低;HCM组所有室壁节段平均SRe及SRe/SRa与FPV呈显著正相关(分别为r=0.75和r=0.70,均为P<0.05),而SRa与FPV并无显著相关性(P>0.05)。结论:HCM患者左室局部与整体舒张功能均较正常人减低,且无论肥厚或非肥厚区域舒张功能均降低,而以肥厚节段舒张功能受损更重。     

尿毒症维持血透伴左室肥厚患者的左室舒张功能研究

采用超声心动图检测20例尿毒症维持血透伴左室肥厚（HD）患者的左室舒张功能，并与11例高血压性心脏病（HHD）、11例肥厚性心肌病（HCM）和11例正常人比较。结果发现，HD、HHD和HCM组的E／A分别为0.91±0.35，1.01±0.52和0.62±0.15（P＜0.05）；Ei/Ai分别为1．10±0.54，1．60±1．09和0.60±0.35（P＜0．05）IRT分别为114±13，101±27和132±23（P＜0．05）。左室心肌重量指数（LVMI）、E／A分别与患者血压呈正、负相关，而与尿毒症毒素不相关。提示：HD患者心脏损害与HHD相似，控制高血压对防治左室肥厚（LVH）、改善心功能有一定意义。     

放射性核素显像评价肥厚型心肌病

对３０例肥厚型心肌病（ＨＣＭ）病人及２９例正常对照者做了心肌灌注显像及心血池显像，结果表明，ＨＣＭ病人均有不同程度的节段性左心室心肌肥厚，以室间隔部位不对称性肥厚最为典型；部份病人尚见有局部心肌灌注缺损，提示有心肌供血不足。心血池显像可见ＨＣＭ病人左心室收缩功能轻微增高，但舒张功能明显减低，以１／３充盈分数（１／３ＦＦ）及峰充盈时（ＴＰＦＲ）最为显著（Ｐ＜０．０１）。     

肥厚性心肌病心肌肥厚部位意义的初步探讨

本文总结了24例肥厚性心肌病,对其肥厚的部位以及临床意义进行了初步的探讨。指出:①肥厚性心肌病根据心肌肥厚部位的不同,可以分成室间隔肥厚、心室中部肥厚和心尖肥厚三种不同亚型;②由于心肌肥厚部位的不同,三种亚型之间存在显著不同的临床表现、心电图和超声心动图特征;③梗阻型室间隔厚度与非梗阻型相比,无显著差异,但梗阻型心肌肥厚的范围可能较广;④二维超声心动图是确定心肌肥厚部位的重要手段和方法。     

应变率成像技术评价肥厚型心肌病左房功能的临床研究

目的探讨应变率成像(SRI)技术评价肥厚型心肌病患者左房功能的临床应用价值。方法选取21例肥厚型心肌病患者和26例除外心血管系统疾病的健康对照者,使用SRI技术检测并比较分析各组左房各壁收缩期、舒张早期、舒张晚期应变率峰值(SSR、ESR、ASR)。结果与健康对照组比较,HCM组左房壁舒张早期应变率峰值(ESR)、收缩期应变率峰值(SSR)均减低,舒张晚期应变率峰值(ASR)增大,差异均有统计学意义(P<0.05)。结论 SRI可定量评估左房功能改变,间接定量反映左室舒缩功能改变。     

肥厚型心肌病发病年龄与心肌肥厚的探讨

目的 探讨肥厚型心肌病（HCM）的发病年龄及其与心肌肥厚程度之间的关系。方法 收集24年来收治的42例HCM病例的临床资料进行回顾性分析。结果 HCM发病的平均年龄为62．1岁，50岁以上病例超过83％，且舒张期末室间隔厚度（IVST）、IVST／左室后壁厚度均明显小于50岁以下者。结论 近年来HCM发病年龄呈现老龄化趋势，随年龄增长心肌肥厚程度反而减轻，两者呈负相关。     

The impact of echocardiography in the diagnosis of hypertrophic cardiomyopathy

The results of 1240 consecutive abnormal echocardiography (ECHO) studies performed between January 1984 and June 1988 were reviewed in order to determine the impact made by the availability of ECHO in the diagnosis and possibly understanding of hypertrophic cardiomyopathy (HCM) in Ethiopia where the condition has not yet been reported. Cardiomyopathies in general were seen in 154 patients; of whom 53 (34.4%) were found to have HCM. The male to female ratio was 3.1:1 (40 males and 13 females) and the mean age was 27.9 yrs, (range 6-80). Asymmetric septal hypertrophy involved the basal one-third of the ventricular septum in 11 (20.8%) patients, basal two-third in 15 (28.3%) and in the majority, 27 (50.9%), it involved the whole septum. Substantial degree of septal hypertrophy (greater than 30 mm), anterolateral wall involvement and severe systolic anterior motion of the mitral valve were seen most commonly among patients with whole septum hypertrophy. These were observed less frequently among patients with basal two-third and basal one-third involvement. These associations, furthermore, appear to indicate presence of obstructive HCM. It is concluded that HCM is not uncommon in Ethiopia. In a country where there are limited diagnostic facilities, ECHO could be used for the classification of HCM and for monitoring patients' response to medical treatment. A prospective study which correlates ECHO clinical finding is needed.     

Polymorphisms of angiotensin-converting enzyme 2 gene associated with magnitude of left ventricular hypertrophy in male patients with hypertrophic cardiomyopathy

Background Even carrying an identical gene mutation, inter- and intra-family variations have been noticed worldwide in the presence and the severity of left ventricular hypertrophy and sudden death in patients with hypertrophic cardiomyopathy （HCM）. Modifier genes may contribute to the diversity. Angiotensin-converting enzyme 2 （ACE2） gene has been established to be associated with parameters of left ventricular hypertrophy in community based male subjects. The objective of the present study was to investigate the association of ACE2 gene polymorphisms with the phenotype of HCM. Methods A total of 261 consecutive HCM patients and 609 healthy controls were enrolled into this study. The polymorphism of rs2106809 and rs6632677 were genotyped by polymerase chain reaction-restriction fragment length polymorphism （PCR-RFLP） and confirmed by sequencing. Logistic regression model and multivariate analysis were used to determine the odds ratio （OR） and 95% confidence intervals （CO of variations of ACE2 for HCM. Results The T allele of rs2106809 and C allele of rs6632677 conferred increasing risk for HCM （OR 1.34, 95%C/ 1.01-1.77, P=0.04; OR 1.11, 95%C/ 1.03-1.21, P=0.002, respectively）, and the 2 single nucleotide polymorphisms （SNPs） were in strong linkage disequilibrium （LD）, the TC haplotype was independently associated with a higher OR for HCM （OR=1.59, 95%C/1.21-1.87） after adjusted for conventional risk factors. And the risk alleles were associated with thicker interventricular septal thickness of HCM （（20.0±6.3） mm vs （17.9±5.5） mm, P=0.03 and （21.3±5.9） mm vs （17.9±5.8） mm, P=0.04, respectively）. No association was found between the two polymorphisms with female patients with HCM. Conclusion Minor alleles of ACE2 gene might be the genetic modifier for the magnitude of left ventricular hypertrophy in male patients with HCM.     

肥厚型心肌病患者晕厥的临床分析(附92例报告)

目的 探讨肥厚型心肌病患者出现晕厥的临床特点.方法 将92例经超声心动图诊断为肥厚型心肌病的住院患者分为晕厥发作组(18例)和无晕厥发作组(74例),就两组的I临床特征、心电变化和超声心动图特点进行分析.结果 晕厥组发生流出道梗阻者[8例(44.4%)]明显超过非晕厥组[11例(14.9%)](P=0.005).晕厥组心肌肥厚部位以室间隔肥厚为主者多见[15例(83.3%)];晕厥组室间隔的厚度[(2.18±0.24)cm]明显高于无晕厥组[(1.91±0.20)cm](P=0.000).晕厥组快速性心律失常的发生例数[11例(61%)]明显多于非晕厥组[18例(24%)](P=0.003).晕厥组QRS波的宽度[(0.107±0.020)s]明显大于无晕厥组[(0.094±0.018)s](P=0.009).晕厥组的ST段下移超过0.1mV的例数[10例(55.6%)]明显多于无晕厥组[22例(29.7%)](P=0.039).结论 肥厚型心肌病患者中,晕厥者主要与流出道梗阻有关.晕厥以室间隔肥厚为主者多见.与室间隔的厚度相关.易发生快速性心律失常.晕厥者的心肌结构排列紊乱-和心电紊乱可能更严重.     

应变和应变率成像评价肥厚型心肌病左室局部心肌收缩和舒张功能的研究

目的：应用应变成像（SI）和应变率成像（SRI）评价肥厚型心肌病（HCM）患者左心室局部心肌收缩和舒张功能。方法：应用SI和SRI技术对34例HCM患者和24例正常人进行检测，获取左室各壁长轴方向基底段、中间段心肌应变和应变率曲线，测量各节段收缩期峰值应变（S）、收缩期峰值应变率（SRs）、舒张早期应变率（SRe）、舒张晚期应变率（SRa）。结果：与正常对照组比较，HCM组左室各节段收缩期峰值应变（S）、收缩期峰值应变率（SRs）、舒张早期应变率（SRe）均明显降低（P〈0．05），左室大部分节段舒张晚期应变率SRa差异无显著性（P〉0.05）。正常对照组约20％节段出现收缩期后缩短（PSS），HCM组约43％节段出现PSS，差异有显著性（P〈0．05）。结论：SI和SRI可敏感地检出HCM患者左室局部心肌收缩和舒张功能的异常，是准确定量评价局部心肌收缩和舒张功能的一种新方法。     

Left Ventricular Regional Systolic Function in Patient with Hypertrophic Cardiomyopathy by Quantitative Tissue Velocity Imaging

The left ventricular regional systolic functions in patients with hypertrophic cardiomyopathy （HCM） were assessed by using quantitative tissue velocity imaging （QTVI）. Left ventricular （LV） regional myocardial velocity along long- and short-axis in 31 HCM patients and 20 healthy subjects were analyzed by QTVI, and the regional myocardial systolic peak velocities （MVS） were measured. Mean MVS at each level including mitral annular, basal, middle and apical segments were calculated. The ratio of MVS along long-axis to that along short-axis （Ri） at basal and middle segments of the LV posterior wall and ventricular septum were calculated. The results showed that mean MVS was slower at each level including mitral annular, basal, middle and apical segments in the HCM patients than that in the healthy subjects （P〈0.01）. There were no significant differences in mean MVS between obstructive and non-obstructive groups in HCM patients. MVS of all regional myocardial segments along long-axis in the HCM patients were significantly slower than that in the healthy subjects （P〈0.05）, but there was no significant difference in MVS of all regional myocardial segments along long-axis between hypertrophied and non-hypertrophied group in the HCM patients. Ri was significantly lower in the HCM patients than that in the healthy subjects. The LV regional myocardial contractility along long-axis was impaired not only in the hypertrophied wall but also in the non-hypertrophied one in patients with HCM, suggesting that QTVI can assess accurately LV regional systolic function in patient with HCM and provides a novel means for an early diagnosis before and independent of hypertrophy.     

血清胰岛素样生长因子-1与肥厚型心肌病左室肥厚的相关性研究

目的 :探讨肥厚型心肌病 (HCM)患者胰岛素样生长因子 - 1(IGF - 1)与HCM心室肌肥厚程度的关系。方法 :分 3组 :正常对照组 (n =2 9)、高血压伴左室肥厚组 (n =2 6 )和HCM组 (n =18)。以超声心动图测算左室重量指数 ,以放射免疫法测定血清IGF - 1水平 ,比较 3组血清IGF - 1水平 ,分析IGF - 1水平和心室肌肥厚程度的相关性。结果 :HCM组和高血压伴左室肥厚组血清IGF - 1水平无统计学差异 ,但两组均显著高于正常对照组 (p <0 .0 1)。血清IGF - 1与左室重量指数高度相关 (r =0 .91,p <0 .0 1)。结论 :IGF - 1可能参与肥厚型心肌病心肌肥厚发生发展的过程。     

Atrial natriuretic peptide gene polymorphism is not associated with hypertrophic cardiomyopathy

Background Hypertrophic cardiomyopathy （HCM） is a primary autosomal dominant inheritant myocardial disease with heterogeneity in clinical manifestations, natural history and prognosis. Even carrying an identical gene mutation among family members, a va[iety of clinical phenotypes have been found in patients with HCM. Modifier genes may contribute to the diversity. The plasma levels of atrial natriuretic peptides （ANP） were found previously to be elevated in HCM. Our studies suggested that ANP gene promoter polymorphism is associated with left ventricular hypertrophy in hypertension. The present study aimed to determine whether the two SNPs in the ANP gene are associated with HCM Methods We determined the relationships between the ANP gene polymorphism and HCM in 262 HCM patients and 614 age- and sex-matched healthy individuals. All of the subjects were genotyped for -A2843G and A188G polymorphisms. Results The genotype frequency in the -A2843G and A188G polymorphisms of the ANP gene was not significantly different between the HCM patients and controls. The -A2843G and A188G polymorphisms were also not associated with clinical phenotype in cardiomyopathy patients. Conclusions The polymorphisms of the ANP gene are not associated with increasing risk of HCM or clinical phenotypes. The variations of the ANP gene may not serve as a genetic modifier for the development of HCM.     

不同病因致左室肥厚的QT离散度

目的 对比观察各种病因引起的左室肥厚的ＱＴ离散度。方法 二维超声心动图同高血压左室最组（ＥＨ）３０例,肥厚型心肌病组（ＨＣＭ）１５例,主动脉瓣狭窄组（ＡＳ）１５例,正常组３０例。比较４组体表心电图ＱＴ离散度（ＱＴｃ）和校正的ＱＴ度苴度（ＱＴｃｄ）。结果 病理性左室肥厚三组的ＱＴｄ和ＱＴｃｄ均明显大于对照组（Ｐ〈０．０１）;ＨＣＭ组和ＡＳ组且明显增大（Ｐ〈０．９５,Ｐ〈０．０１和Ｐ〈０．０５,Ｐ〈０     

Improvement in left ventricular function assessed by tissue Doppler imaging after aortic valve replacement for severe aortic stenosis

INTRODUCTION

The effects of reduction of left ventricular (LV) systemic afterload following aortic valve replacement (AVR) for severe aortic valve stenosis (AS) were investigated, using echocardiography and tissue Doppler imaging (TDI).

METHODS

We compared the preoperative and postoperative echocardiographic assessments of 23 patients with severe AS who had undergone isolated AVR (n = 13) or concomitant AVR with coronary artery bypass grafting (CABG) (n = 10). Conventional echocardiographic evaluations and TDI at the lateral mitral annulus were performed.

RESULTS

Echocardiography was performed at a median of 120 (interquartile range: 66–141) days after AVR. There was significant reduction in aortic transvalvular mean pressure gradient after AVR. Although LV dimensions, mass and ejection fraction remained unchanged, LV diastolic and systolic functions improved (as observed on TDI). Early diastolic (E’), late diastolic (A’) and systolic (S’) mitral annular velocities increased significantly (p < 0.05). There was significant improvement in TDI-derived parameters among the patients who had isolated AVR, while among the patients who had concomitant AVR with CABG, only S’ had significant improvement (p = 0.028).

CONCLUSION

TDI was able to detect improvements in LV systolic and diastolic function after AVR for severe AS. There was less improvement in the TDI-derived diastolic parameters among patients who underwent concomitant AVR with CABG than among patients who underwent isolated AVR.     

超声二维斑点追踪显像技术评价肥厚型心肌病左心室旋转和扭转运动

目的采用超声二维斑点追踪显像技术(STI)探讨肥厚型心肌病(HCM)患者左心室扭转运动(LVtw)的特征。方法随机选取心功能正常的HCM患者及正常对照者各20例,取胸骨旁左心室心尖和心底短轴观二维图像对左心室旋转进行测量分析,计算心底部旋转角度峰值(MV-Prot)及其达峰时间、心尖部旋转角度峰值(AP-Prot)及其达峰时间、扭转角度峰值(Ptw)及达峰时间、收缩期末扭转角度(AVCtw)、等容舒张期末扭转角度(MVOtw)、解旋率(UntwR)、解旋减半时间(HTU),并比较两组间上述各参数的差别。结果(1)与对照组相比,HCM组MV-Prot、AP-Prot、Ptw、AVCtw、MVOtw和HTU测值增加,UntwR测值减低(P均<0.05)。(2)HCM组左心室扭转角度达峰时间测值较对照组明显增加(P<0.05)。(3)HCM组内左心室旋转心尖部达峰时间较心底部测值明显增加(P<0.05)。结论应用STI可无创性评价HCM患者左心室旋转及扭转运动特征,HCM患者左心室旋转运动力学参数较正常人有明显异常改变。     

肥厚型心肌病患者血管紧张素转换酶基因缺失多态性研究

目的 探讨血管紧张素转换酶（ＡＣＥ）基因插入／缺失（Ｉ／Ｄ＿多态必瑟肥厚型心肌病（ＨＣＭ）发病的关系。方法 应用ＰＣＲ技术扩增ＡＣＥ基因目的片段检测基因多态性。结果 ＨＣＭ患者与正常对照组的Ｄ等位基因频率及ＤＤ基因型分布有显著性差异（Ｐ〈０．０１,Ｐ〈０．０５）,ＤＤ基因型ＨＣＭ患者心脏事件发生率及左室壁厚度明显大于２、ＩＤ基因型ＨＣＭ患者（Ｐ〈０．０５,Ｐ〈０．０１）,家族性肥厚型心肌病（ＦＨＣ     

Mutation of Arg723Gly in β-myosin heavy chain gene in five Chinese families with hypertrophic cardiomyopathy

Background Hypertrophic cardiomyopathy （HCM） is a form of cardiomyopathy with an autosomal dominant inherited disease, which is caused by mutations in at least one of the sarcomeric protein genes. Mutations in the beta-myosin heavy chain （β-MHC） are the most common cause of HCM. This study was to reveal the disease-causing gene mutations in Chinese population with HCM, and to analyze the correlation between the genotype and phenotype. Methods The exons 3 to 26 of MYH7 were amplified by PCR, and the PCR products were sequenced in five non-kin HCM patients. A 17-year-old patient was detected to be an Arg723Gly mutation carrier. Then his family was gene-screened, and the correlation between genotype and phenotype was analyzed. Results The mutation of Arg723Gly in a Chinese family with HCM was detected for the first time. With a C-G transversion in nucleotide 13 619 of the MYH7 gene, located at the essential light chain interacting region in S1, the replacement of arginine by glycine took place at amino acid residue 723. A two-dimensional echocardiogram showed moderate asymmetrical septal hypertrophy with left atria enlargement. There was no obstruction in the left ventricular outflow tract. In his family, a total of 13 individuals were diagnosed HCM and 5 of them were dead of congestive heart failure at a mean age of 66-year-old. Eight living members were all detected to carry the mutation, in which 3 developed progressive heart failure. Moreover, the heart function of the people evidently deteriorates when their age are older than 50. The mutation and the disease show co-separated. Conclusion The Arg723Gly mutation is a malignant type. In Chinese the mutation has the similar characters to the former report but has low degree malignant.     

肥厚型心肌病冠状动脉血流动力学相关参数变化

①目的探讨肥厚型心肌病（HCM）病人冠状动脉血流动力学相关参数变化。②方法对9例非梗阻性HCM病人和11例健康志愿者应用冠状动脉内多普勒导丝测定冠状动脉左前降支（LAD）和左回旋支（LCx）血流频谱。③结果HCM组LAD的收缩期时间速度积分（STVI）明显小于对照组（t=5．66，P〈0．01），而舒张期时间速度积分（DTVI）明显大于对照组（t=2．65，P〈0．05）。HCM组中3例出现收缩期逆向血流频谱。HCM组LAD的冠状动脉血流储备（CFR）明显小于对照组（t=4．76，P〈0．01）。LCx的STVI、DTVI和CFR两组间无差异。STVI与室壁厚度呈负相关（r=-0．57，P〈0．01），而DTVI与室壁厚度呈正相关（r=0．43，P〈0．01）。LAD／LCx CFR比值与舒张末期室间隔和左室后壁厚度比值呈负相关（r=-0．67，P〈0．01）。④结论非梗阻性HCM中供应肥厚心肌的冠状动脉存在血流动力学相关参数异常，而这些变化与心肌肥厚程度有关。