心脏磁共振成像识别肥厚型心肌病患者不良预后危险因素的研究进展

肥厚型心肌病（HCM）是最常见的遗传性心血管疾病之一,以不能由全身系统性疾病或其他心脏疾病解释的左心室肥厚为主要临床特征。HCM患者的不良事件主要包括猝死、心力衰竭、心房颤动等,而心脏猝死（SCD）是年轻HCM患者的主要死亡原因。心脏磁共振成像以多序列多参数成像为HCM高危患者的识别和植入式心律转复除颤器的植入提供了参...     

肥厚型心肌病的磁共振电影成像分析

目的 :探讨肥厚型心肌病 (HCM)的左室形态及功能的变化 ,评价 HCM的磁共振影像诊断价值。方法 :2 4例 HCM患者 ,行磁共振快速自旋回波 (TSE)多层面平扫和磁共振电影检查 ,用 ARGU S专用心功能分析软件分析左室心肌质量、各节段心壁厚度、心肌增厚率、射血分数、心腔 (左室 )容积及时间 -容积变化曲线等参数。结果 :1HCM心肌质量显著增加 (t=2 4.6 ,P <0 .0 1) ,病变主要累及左室前、侧壁及室间隔。 2肥厚心肌的心肌收缩能力低于正常心肌 (t=15 .1,P <0 .0 1)。 3左室心腔容积较正常降低 ,且舒张能力下降 (t=2 5 .4,P <0 .0 1)。 4梗阻性 H CM的左室流出道狭窄明显 ,左心射血时限延长。结论 :磁共振成像能全面了解 HCM心脏形态及左心功能异常 ,具有重要的临床应用价值 ,尤其是对心尖肥厚型的诊断 ,明显优于心脏 B超     

肥厚型梗阻性心肌病的心律失常分析

目的 探讨肥厚性心肌病(HCM)者左室流出道梗阻对心律失常的影响.方法 回顾分析84例HCM者,根据超声心动图(UCG)检查分成梗阻组(A组)38例和非梗阻组(B组)46例,以动态心电图(DCG)结果,进行心律失常比较.结果 ①房性早搏(PAS)、室性早搏(PVS)、窦房结功能障碍、房室阻滞、室内阻滞的发生率,两组比较无统计学意义;②频发PVS发生率两组比较有统计学意义(P＜0.05);③频发PAS、短阵室上性心动过速、心房扑动、心房颤动、成对PVS、多源PVS、短阵室性心动过速发生率,两组比较有显著性差异(P＜0.01).结论 HCM易发生心律失常,A组快速心律失常发生率明显高于B组,左室流出道梗阻能增加HDCM者快速心律失常的发生,但不增加缓慢心律失常的发生.     

肥厚型心肌病患者临床特征及肥厚节段分析

目的 对肥厚型心肌病患者临床特征及其受累肥厚节段的分布和程度进行分析.方法 连续收集2004年3月至2007年3月225例临床诊断或可疑的肥厚型心脏病患者的临床资料,包括症状、体征、心电图检查及超声心动图检查等.所有患者均接受心脏核磁共振检查.依据9节段分析法分析受累节段范围、程度等.结果 肥厚型心肌病患者中男163例,女62例,平均年龄(50.4±14.5)岁.28例肥厚型心肌病患者临床无症状,系通过体检发现.197例肥厚型心肌病患者临床症状明显,其中11例伴发晕厥.216例肥厚型心肌病患者心电图异常,73例患者有明确家族史.126例肥厚型心肌病患者可闻及收缩期杂音.超声心动图发现95例患者合并左心室流出道梗阻,32例患者伴发二尖瓣关闭不全.肥厚型心肌病患者合并高血压50例,合并冠心病14例,合并糖尿病5例.225例患者左心房前后径平均为(39.4±8.3)mm,左心室舒张末期横径平均为(47.8±5.5)mm.依据9节段分析法,32.1%的节段受累.非对称性肥厚患者222例,其中室间隔受累198例,对称性肥厚患者3例.心尖受累67例,其中单独心尖受累24例.98例患者室间隔和左心室前壁同时受累.所有室间隔肥厚患者室间隔平均厚度为(23.0±5.2)mm.其中伴发左心室流出道梗阻患者室间隔平均厚度为(24.3±5.3)mm,高于无梗阻患者(21.6±4.6)mm,P＜0.05.所有心尖肥厚患者心尖平均厚度为(15.6±3.4)mm.结论 我国肥厚型心肌病患者男性比例较高,临床发病时间较晚.心脏磁共振能很好地评估肥厚型心肌病各个亚型的病理解剖学特征,是准确诊断肥厚型心肌病的有效方法.     

肥厚型心肌病患者心肌纤维化范围的相关因素分析

目的探讨肥厚型心肌病患者心肌纤维化范围的相关因素。方法该研究为横断面分析性研究,入选2016年1月至2020年5月在云南省第一人民医院住院的肥厚型心肌病患者。通过病案管理系统收集入选患者的一般临床资料。入选者均行心脏磁共振(CMR)检查,以CMR钆对比剂延迟强化(LGE)识别是否存在心肌纤维化及其部位,应用视觉分析法计算LGE范围(LGE%)。根据是否存在LGE分为LGE阳性组和LGE阴性组,进一步根据左心室舒张末期最大室壁厚度(LVMWT)将LGE阳性组患者分为轻度肥厚组、中度肥厚组和重度肥厚组。入选患者均测定外周血N末端B型利钠肽原(NT-proBNP)和心肌肌钙蛋白I(cTnI)水平。结果该研究共入选患者48例,年龄(46.4±14.3)岁,其中男性42例(87.5%)。CMR检查示LGE阳性患者34例(LGE阳性组)占70.8%,阴性者14例(LGE阴性组)。与LGE阴性组比较,LGE阳性组患者较为年轻(P=0.038),纽约心脏协会心功能Ⅲ/Ⅳ级者占比较高(P=0.00)。与LGE阴性组比较,LGE阳性组患者LVMWT较厚(P=0.008),左心室质量指数(LVMI)较大(P=0.001),左心室舒张末期容积(LVEDV)较大(P=0.043),左心室射血分数(LVEF)和心脏指数(CI)均较低(P均<0.05)。LGE阳性组患者血NT-proBNP和cTnI水平均明显高于LGE阴性组[分别为2760.5(1503.4,3783.6)ng/L比861.3(552.2,1092.8)ng/L,P=0.002;0.970(0.448,1.684)μg/L比0.147(0.033,0.251)μg/L,P=0.041]。LGE阳性组患者心肌轻度肥厚者15例(轻度肥厚组)、中度肥厚者10例(中度肥厚组)、重度肥厚者9例(重度肥厚组),3个亚组间LGE%以及NT-proBNP和cTNI均随着心肌肥厚程度增加而增加(P均<0.05)。LGE%与年龄呈负相关性(r=-0.618,P=0.011),与NT-proBNP、cTnI水平呈正相关(分别为,r=0.271,P=0.010;r=0.111,P=0.013),与LVEDV、LVMWT及LVMI均呈正相关(分别为,r=0.438,P=0.09;r=0.735,P=0.001;r=0.532,P=0.034)。结论肥厚型心肌病患者心肌纤维化范围与年龄呈负相关,与血NT-proBNP、cTnI水平以及LVEDV、LVMWT、LVMI均呈正相关。     

延迟增强心肌磁共振定量分析对缺血性心肌病患者室性心律失常预测价值

目的探索延迟增强心脏磁共振成像(DE-CMR)定量分析对缺血性心肌病患者发生室性心律失常的预测价值。方法 41例缺血性心肌病伴有左心室射血分数≤35%的患者在植入心脏转复除颤器(ICD)前进行DE-CMR检查,对心肌延迟增强定量分析,并对ICD定期程控记录室性心律失常发生情况。结果在平均(441±209)d随访中,12例(29%)患者ICD)记录到自发或治疗终止的持续性室性心动过速或心室颤动。发生室性心律失常患者的增强心肌质量及其占左心室心肌质量百分比均显著高于无室性心律失常组[(60.1±24.4)g比(40.9±20.1)g,P=0.01)及(51.8%±20.0%)比(37.8%±15.2%),P=0.02]。受试者工作特征曲线(ROC)分析显示增强心肌质量或其百分比对室性心律失常预测价值高于射血分数。在多因素分析中,增强心肌质量(HR 1.54/10 g;95%CI1.06～2.45,P=0.02)或其百分比(HR 1.65/10%;95%CI 1.05～2.58;P=0.03)是惟一的发生室性心律失常预测因子。结论 DE-CMR定量分析是预测缺血性心肌病患者发生室性心律失常的独立危险因子。     

肥厚型心肌病的心律失常与猝死

肥厚型心肌病的心律失常与猝死的发生率较高。近年来对肥厚型心肌病的心律失常与猝死的认识有进展,本文就此问题做一综述。肥厚型心肌病严重心律失常的发生率随着动态心电图和平板运动试验的广泛应用,肥厚型心肌病的心律失常检出率有所增加。Savage等报告100例肥厚型心肌病经24小时动态心电图监测,均有多型性或复发性室性早搏(室早)。而室性心动过速(室速)的检出率为19%。Goodwin等报告室性心律失常的发生率为50%,无症状性室速发生率为19～36%,后来Mulron等发现,随着动态心电图监测时间的延长,心律失常的检出率有所增加。一年内监测48～168小时(平均72小时)室早检出率为2～17695次/日,室速检出率为108阵,平均每日1.5阵,而监测24小时室速检出率仅为     

新型磁共振对典型及早期致心律失常性右室心肌病诊断价值的探讨

目的 用有黑血技术的新型磁共振（MRI）对典型致心律失常性右室心肌病（ARVC）进行检查，以确定新型MRI诊断ARVC的特异性和敏感性，并通过对确诊的ARVC患的一级亲属行MRI检查，以探讨MRI对早期ARVC的诊断价值。方法 10例ARVC患（除1例猝死首诊外）及其7个家系的54名成员全部接受询问病史，体检，心电图，心脏超声等检查；10例临床患均接受MRI检查，分析和确定其影响特征及诊断条件，在此基础上对部分家系成员行MRI检查以发现早期ARVC患。结果 临床患有阵发性室性心动过速（8／8），晕厥（9／10），心力衰竭（3／10）和猝死（3／10）。心电图均有左束支传导阻滞型阵发性室性心动过速，心室晚电位（VLP）均阳性（8／8）。MRI检查显示临床患均有明显右心室（RV）扩大及室壁广泛强信号，经压脂处理后心肌信号呈岛状或连续中断，为特征性纤维脂肪替代影像，患均有RV运动减低或室壁瘤形成，部分伴左心室受累（3／8）。家系筛选发现8例异常，拟诊为早期ARVC，2例有心电图异常，2例VLP阳性。MRI显示，8例心室壁均有局限性纤维脂肪病的影像改变，4例有RV扩大，2例可疑扩大，6例RV心尖部血流淤滞现象。结论 带黑血技术的新型MRI是目前诊断ARVC和早期ARVC的最具特异性和敏感性的检查手段。     

肥厚型心肌病患者心律失常的研究进展

本文综述了近年来肥厚型心肌病(HCM)患者心律失常方面的研究情况,HCM患者心律失常的发生率很高,与猝死、晕厥和心功能的下降有关,它的发生有其病理学和电生理基础,对HCM患者抗心律失常治疗最有效的药物是胺碘酮。     

肥厚型心肌病研究的进展

肥厚型心肌病(HCM)是最常见的遗传性心肌病,其特征是在无异常负荷的情况下心室壁异常增厚,主要累及室间隔。HCM目前主要的治疗目的是缓解症状,包括药物治疗和减少室间隔肥厚的治疗。本文将对HCM的临床诊断方法、药物及手术治疗等方面研究的进展作一综述。     

Interpretation of electrocardiographic abnormalities in hypertrophic cardiomyopathy with cardiac magnetic resonance.

AIMS: To clarify the mechanisms of electrocardiographic abnormalities in hypertrophic cardiomyopathy, 102 patients were examined with cardiac magnetic resonance. Distribution and magnitude of hypertrophy and late-enhancement were correlated with electrocardiographic abnormalities. METHODS AND RESULTS: Abnormal Q waves were associated with greater upper anterior septal thickness (22+/-7 mm vs. 18+/-5 mm, P=0.001) and increased ratios of upper anterior septum to mean inferolateral (P=0.01), anterolateral (P=0.002), apical (P=0.001), and right ventricular (P=0.001) wall thickness. There was no relation between abnormal Q waves and late-enhancement, except for Q waves >/=40 ms (P=0.02). Conduction disturbances and absent septal Q waves were associated with late-enhancement (89 vs. 45%, P=0.01 and 75 vs. 39%, P=0.002, respectively). The depth of negative T waves was related to an increased ratio of the mean thickness between apical and basal level (P=0.01), and to the presence of apical late-enhancement (P=0.03). CONCLUSION: Abnormal Q waves reflect the interrelation between upper anterior septal thickness and other regions of the left and right ventricles, and wider Q waves are associated with late-enhancement. Conduction disturbances and absent septal Q waves are associated with late-enhancement. The depth of negative T waves is related to craniocaudal asymmetry and apical late-enhancement.     

心脏磁共振对肥厚型心肌病应用价值的研究

肥厚型心肌病(HCM)是最常见的遗传性心肌病,在普通人群中其发病率为1/500,HCM临床表现多样,主要发病机制是心肌细胞肥厚、排列紊乱、心肌纤维化及胶原沉积以及不稳定的心电活动容易导致恶性心律失常所致。近年来心脏磁共振(CMR)被广泛应用于HCM的诊断以及与其他疾病的鉴别诊断,CMR不仅能够准确评估心室功能、心室壁厚度,并且钆延迟强化(LGE)能够无创检测心肌纤维化及瘢痕。重要的是越来越多的研究证明LGE与HCM等多种心血管病的发生相关,并可以预测其发生。本文将回顾相关文献,总结CMR对HCM的应用价值,使CMR更好的应用于临床。     

Value of real time three-dimensional echocardiography in patients with hypertrophic cardiomyopathy: comparison with two-dimensional echocardiography and magnetic resonance imaging

Real time three-dimensional echocardiography (RT3DE) has been demonstrated to be an accurate technique to quantify left ventricular (LV) volumes and function in different patient populations. We sought to determine the value of RT3DE for evaluating patients with hypertrophic cardiomyopathy (HCM), in comparison with cardiac magnetic resonance imaging (MRI). Methods: We studied 20 consecutive patients with HCM who underwent two-dimensional echocardiography (2DE), RT3DE, and MRI. Parameters analyzed by echocardiography and MRI included: wall thickness, LV volumes, ejection fraction (LVEF), mass, geometric index, and dyssynchrony index. Statistical analysis was performed by Lin agreement coefficient, Pearson linear correlation and Bland-Altman model. Results: There was excellent agreement between 2DE and RT3DE (Rc = 0.92), 2DE and MRI (Rc = 0.85), and RT3DE and MRI (Rc = 0.90) for linear measurements. Agreement indexes for LV end-diastolic and end-systolic volumes were Rc = 0.91 and Rc = 0.91 between 2DE and RT3DE, Rc = 0.94 and Rc = 0.95 between RT3DE and MRI, and Rc = 0.89 and Rc = 0.88 between 2DE and MRI, respectively. Satisfactory agreement was observed between 2DE and RT3DE (Rc = 0.75), RT3DE and MRI (Rc = 0.83), and 2DE and MRI (Rc = 0.73) for determining LVEF, with a mild underestimation of LVEF by 2DE, and smaller variability between RT3DE and MRI. Regarding LV mass, excellent agreement was observed between RT3DE and MRI (Rc = 0.96), with bias of − 6.3 g (limits of concordance = 42.22 to − 54.73 g) . Conclusion: In patients with HCM, RT3DE demonstrated superior performance than 2DE for the evaluation of myocardial hypertrophy, LV volumes, LVEF, and LV mass.     

Impact of cardiac magnetic resonance imaging in nonischemic cardiomyopathies

Non-ischemic cardiomyopathies include a wide spectrum of disease states afflicting the heart, whether a primary process or secondary to a systemic condition. Cardiac magnetic resonance imaging(CMR) has established itself as an important imaging modality in the evaluation of non-ischemic cardiomyopathies. CMR is useful in the diagnosis of cardiomyopathy, quantification of ventricular function, establishing etiology, determining prognosis and risk stratification. Technical advances and extensive research over the last decade have resulted in the accumulation of a tremendous amount of data with regards to the utility of CMR in these cardiomyopathies. In this article, we review CMR findings of various non-ischemic cardiomyopathies and focus on current literature investigating the clinical impact of CMR on risk stratification, treatment, and prognosis.     

Left ventricular structure and diastolic function by cardiac magnetic resonance imaging in hypertrophic cardiomyopathy

ObjectiveDiastolic dysfunction is common in hypertrophic cardiomyopathy (HCM) and hypertensive heart disease (HHD), but its relationships with left ventricular (LV) parameters have not been well studied. Our objective was to assess the relationship of various measures of diastolic function, and maximum left ventricular wall thickness (MLVWT) and left ventricular mass index (LVMI) in HCM, HHD and normal controls using cardiac magnetic resonance imaging (CMR). We also assessed LV parameters and diastolic function in relation to late gadolinium enhancement (LGE) and right ventricular (RV) hypertrophy in HCM.Methods41 patients with HCM, 21 patients with HHD and 20 controls were studied. Peak filling rate (PFR), time to peak filling (TPF), MLVWT and LVMI were measured using CMR. LGE and RV morphology were assessed in HCM patients.ResultsMLVWT correlated with TPF in HCM (r = 0.38; p = 0.02), HHD (r = 0.58; p = 0.01) and controls (r = 0.54; p = 0.01); correlation between MLVWT and TPF was weaker in HCM than HHD. LVMI did not correlate with diastolic function. In HCM, LGE extent correlated with MLVWT (τ = 0.41; p = 0.002) and with TPF (τ = 0.29; p = 0.02). The HCM patients with RV hypertrophy had higher MLVWT (p < 0.001) and TPF (p = 0.03) than patients without RV hypertrophy.ConclusionMLVWT correlates with diastolic function (TPF) in HCM, HHD and controls. LVMI did not show significant correlation with TPF. The diastolic dysfunction in HCM is not entirely explained by wall thickening. LGE and RV involvement are associated with worse LV diastolic function, suggesting that these may be markers of more severe underlying myocardial disarray and fibrosis that contribute to diastolic dysfunction.