心肌致密化不全的临床特征

目的分析心肌致密化不全的临床特征。方法对确诊为心肌致密化不全的18例患者的临床表现、心电图、心脏彩超、血清肌钙蛋白、血浆脑钠肽前体(NT-pro BNP)等进行总结、分析。结果心肌致密化不全患者多以胸闷、气短等心衰症状就诊,NYHA分级多Ⅳ级,NT-pro BNP、肌钙蛋白I均有不同程度的增高,心电图示合并有多种心律失常改变;均行心脏彩超检查发现心肌致密化不全的特异性改变,其中累及心尖部7例(39%),累及左室侧、后壁10例(65%)。结论心肌致密化不全患者年龄分布较广,临床表现以心衰表现多见,可合并多种心律失常。心脏彩超多以左心改变多见,为确诊的主要方法。     

扩张型心肌病与左心室心肌致密化不全临床和磁共振成像特征对比

目的 研究扩张型心肌病(DCM)和左心室心肌致密化不全(LVNC)的临床和磁共振成像(MRI)特征,探讨MRI对二者鉴别诊断的价值.方法 分析21例经心脏移植证实为DCM患者、25例临床证实为INNC患者的一般临床资料.利用心脏MRI检查,分析两组患者的左心房、室径线及左心功能,将左心室划分为17节段,对左心室致密化不全的节段进行计数,测量左心室各节段非致密化心肌和致密化心肌的厚度,计算非致密化心肌/致密化心肌比值(NC/C比值).结果 DCM患者全部都有胸闷、气短,心电图异常表现.而25例LVNC患者中21例(84%)患者有胸闷、气短、胸痛等症状,4例(16%)无明确症状.22例(88%)LVNC患者存在心电图异常,3例(12%)心电图无异常发现.心脏MRI显示DCM患者左心房、室扩张更明显,更趋向球形化[DCM球形指数(SI)=0.81±0.06,LVNC SI=0.74±0.11].DCM患者左心室射血分数[LVEF,(15.0±5.1)%]较LVNC[(32.7±14.2)%]低.DCM左心室有过度小梁化倾向,但LVNC患者受累节段即致密化不全的节段数(9±1)比DCM受累节段即过度小梁化节段(5±2)多.所有LVNC患者的心尖段(第17节段)均受累,而DCM患者心尖段未见受累.其他易受累节段在两组患者中分布大致相似,即侧壁中段、心尖段(第16、12、11节段)是最常见的受累节段,而室间隔基底段、中段(第2、3、8、9节段)均未见受累.LVNC患者最常发生致密化不全的心肌节段其致密化心肌厚度变薄,与DCM患者对应节段室壁厚度无差别.而不发生或很少发生致密化不全的节段,LVNC患者致密化心肌厚度比DCM患者对应节段厚.LVNC患者致密化不全心肌厚度与致密化心肌厚度比值(NC/C:3.3±0.6)较DCM患者(1.9±0.3)高,差异有统计学意义.结论 DCM与LVNC患者临床表现有些类似,心脏MRI能够显示二者在左心形态及功能方面的差别,NC的分布及程度对二者的鉴别有很大价值.     

心肌致密化不全研究进展

<正>心肌致密化不全(noncompaction of the ventricular myocardium,NVM)较少见,是一种以心室内异常粗大的肌小梁和交错的深隐窝为特征的与基因相关的心肌病。1932年,Bellet等首次在新生儿尸检的心脏中描述了这种心肌的特点,至1990年Chin将其命名为NVM。2006年美国心脏协会(AHA)将其归为遗传性心肌病的一种,2008年欧洲心     

心肌致密化不全研究进展

<正>心肌致密化不全(NVM)标志性特点是:心室壁内层突起的肌小梁、深陷隐窝,外层致密化心肌变薄〔1〕,可有心功能不全、心律失常及血栓栓塞等临床表现。1984年该病被认为是子宫内胚胎发育过程中心脏血窦退化异常〔2〕。1990年Chin等〔3〕认识到该病为一种"心肌病",并归因于胚胎时期心肌致密化过程停滞。因此,1996年世界卫生组织和国际心脏病学会(WHO/ISFC)工作组将该病纳入心肌病中并归类于"未定型心     

心肌致密化不全研究进展

现介绍心肌致密化不全在发病机制、病理学、流行病学、临床特点、诊断及诊断标准、治疗及预后等方面的进展,并总结了国内心肌致密化不全患者的临床特点。     

儿童左室致密化不全3例报告

<正>心肌致密化不全(noncompaction of ventricular myocardium,NVM)是较少见的先天性心肌病。由Chin于1990年首先报道〔1〕,又称海绵状心肌病,它是胚胎发育早期,网织状肌小梁致密化过程失败导致小梁化的心肌持续存在,以无数突出的肌小梁和深陷的小梁隐窝为特征〔2〕。有的患者与     

心肌致密化不全2例确诊报告

<正>心肌致密化不全(noncompaction of ventricular myocardium,NVM)疾病的概念于1990年由Chin等〔1〕提出。曾于1995年被世界卫生组织归为未分类的心肌病,现被美国心脏病学会/美国心脏学会/欧洲心脏病学会(ACC/AHA/ESC)将其归     

心肌致密化不全1例

<正>1病历摘要患者,男,50岁,因胸闷、气短、呼吸困难3年,加重2个月入院。既往:高血压病史5年。查体:血压(BP)110/80 mm Hg,颈静脉无怒张,双肺呼吸音清,双肺底可闻及湿啰音,心率100次/min,律齐,各瓣膜听诊区未闻及杂音及额外心音。肝、脾、肋下未触及,双下肢轻度浮肿。心电图示:左室肥厚,V4     

孤立性心肌致密化不全的研究现状

孤立性心肌致密化不全(isolated non-compaction cardiomyopathy,NCCM)是胚胎发育过程中心内膜以及心肌发育停止而引起的以心室内壁异常粗大交错的肌小梁和深隐窝为特征的基因异常相关性遗传性心肌病.1984年Engberding等[1]首次描述了该病的典型超声心动图表现,1995年WHO将其定义为未分类心肌病,最近美国心脏病协会(AHA)建议将其归为原发性遗传性心肌病.目前为止,临床医师对本病了解甚少,本文通过对NCCM流行病学、临床表现、病理生理学、诊断等方面研究现状作一综述,以提高临床医生对NCCM的认识及诊治水平.     

18例心肌致密化不全患者的临床特征

目的 探讨心肌致密化不全患者的临床特征。方法 回顾分析了18例心肌致密化不全患者的临床表现、心电图、超声心动图等资料,随访（11,4-5）个月。结果 心肌致密化不全占同期确诊的各类原发性心肌病的8,1％。发病年龄1．5～71岁,男性占66．7％,家族性发病2例。14例患者表现为程度不等的心力衰竭,2例患者因心律失常表现为黑喙、晕厥,1例患者发生血栓栓塞,1例患者无症状。心电图均有异常,室性期前收缩7例,心脏传导阻滞和心房颤动各4例。所有患者均通过心脏超声检查确诊,其中17例患者病变局限于左心室,1例局限于右心室,心尖部最常受累（72％）,收缩末期非致密化心肌层／致密化心肌层2．3～3．1,15例患者左室射血分数〈50％,室壁弥漫性运动障碍。随诊期间13例患者反复出现心力衰竭,1例患者于确诊后16个月发生心脏性猝死,1例患者因顽固性心力衰竭于确诊后10个月接受心脏移植,1例患者因药物难以控制的心律失常置入埋藏式自动心脏复律除颤器,口服抗凝药的患者未再发血栓栓塞。结论 心肌致密化不全的病情迁延,男性多见,有家族遗传倾向,在原发性心肌病中并不罕见。临床表现主要是心力衰竭、心律失常、血栓栓塞。心脏超声为确诊的首选检查。预后较差,顽固性心力衰竭和致死性心律失常是主要死因,心脏移植、置人埋藏式自动心脏复律除颤器能降低致死（残）率,口服抗凝药能减少血栓栓塞的发生。     

Right ventricular involvement in a patient with isolated noncompaction of the ventricular myocardium

Isolated noncompaction of the ventricular myocardium (INVM) is an unclassified cardiomyopathy and is thought to be due to arrest of myocardial morphogenesis. Although right ventricular involvement is not uncommon, the correct diagnosis is often difficult by echocardiography. In this report, we describe a patient with INVM in whom magnetic resonance imaging was useful to detect right ventricular morphological and functional abnormalities.     

Ventricular twist in isolated left ventricular noncompaction

The study aimed at assessing the rotational motion of the left ventricle around the long axis in patients affected by isolated left ventricular noncompaction (LVNC) and comparing their results with those of healthy volunteers.Patients and methodsThe study comprised eight patients with LVNC confirmed by echocardiography and magnetic resonance imaging (mean age 41 ± 19 years; four males; left ventricular ejection fraction [LVEF] 45 ± 25%). The patients were divided into Group A with an LVEF above 50% (four patients; one male) and Group B with an LVEF below 50% (four patients; three males). For both groups, age- and sex-matched volunteers were found. The focus was on myocardial twist, rotation of the LV apex and base, times to reach maximal apical and maximal basal rotation and myocardial twist, as well as correlations between systolic function, rotation of individual planes and LV twist.ResultsWhen comparing LVNC patients with healthy volunteers, there were statistically significantly decreased systolic function (p = 0.004), larger diastolic dimension (p = 0.045) and decreased apical rotation (p = 0.01). Left ventricular twist was significantly decreased in the group of patients with LVNC and systolic dysfunction (p = 0.04). A statistically significant correlation was found between systolic function and LV apical rotation.ConclusionThe study showed a significant decrease in apical rotation and twist mechanism of the LV as measured using two-dimensional speckle tracking in patients with LVNC and decreased LV systolic function. Measuring these parameters could contribute to more accurate diagnosis and risk stratification of patients affected by this condition.     

Unbiased and efficient estimation of left ventricular volumes by three-dimensional echocardiography with coaxial sections. Validation with magnetic resonance imaging

The objective of this study was to investigate the degree of bias with coaxial three-dimensional echocardiography in an experimental animal setup and to establish the minimum number of sections needed for estimation of left ventricular (LV) volume. Epicardial coaxial echocardiography and magnetic resonance imaging (MRI) was used to measure LV volume in 14 pigs, with chronic remodeled left ventricles induced by repeated intracoronary microembolizations. In addition, six animals underwent serial MRI at baseline, immediately after intracoronary microembolization, and after 119–165 days (mean 129 days). Coaxial echocardiography was performed by rotational acquisition of long-axis sections starting from an arbitrary angle. Planimetered MRI contours of LV endocardial borders were analyzed to investigate the relationship between the number of coaxial sections, and the precision of volume estimates. The mean ± 2SD of the differences between coaxial epicardial echocardiography with six sections and MRI were −2.5 ± 16.4 ml, 0.8 ± 13. 1 ml, and 2% ± 14% for end-diastolic volume (EDV), end-systolic volume (ESV), and ejection fraction (EF), respectively. Numerical analysis conducted on MRI contours of LV endocardial borders showed that with six coaxial sections the average coeffi-cient of error was <1% for the EDV and ESV. Three-dimensional echocardiography with six coaxial sections provides unbiased LV volume estimation with minimal geometric error. Received: February 10, 2000 / Accepted: May 23, 2000     

Clinical characteristics and morphologic features of isolated noncompaction of the ventricular myocardium: electrocardiogram-gated magnetic resonance imaging can elucidate more accurate biventricular structures

Isolated noncompaction of the ventricular myo-cardium (INVM) is a rare congenital disorder characterized by an arrest of myocardial morphogenesis. We experienced a case of a 23-year-old man, referred to our emergency room, whose symptoms included shortness of breath. ECG-gated cardiac magnetic resonance imaging depicted distinctive features of INVM in both the left and right ventricles. We present the clinical characteristics and morphological features of this case in comparison with some recent reports.     

Diagnosing left ventricular noncompaction by echocardiography and cardiac magnetic resonance imaging and its dependency on neuromuscular disorders

BACKGROUND: Left ventricular hypertrabeculation (LVHT), also termed noncompaction LVHT, is diagnosed by echocardiography or cardiac magnetic resonance imaging (CMRI), and associated with neuromuscular disorders (NMD). The aim of this study was to assess if LVHT can be diagnosed by CMRI applying echocardiographic definitions. METHODS AND RESULTS: The CMRI images of 19 echocardiographically diagnosed LVHT patients were re-evaluated (10 female, 14-67 y of age). Left ventricular hypertrabeculation was diagnosed by CMRI in 9 cases. Patients with CMRI-diagnosed LVHT were more often females (67% versus 40%), experienced heart failure more often (100% versus 50%), had an LV end diastolic diameter > 57 mm (67% versus 40%), had an LV fractional shortening < 25% (89% versus 40%), and had a larger extension of LVHT than patients without CMRI-diagnosed LVHT. The prevalence of NMD (87%) did not differ between both groups. CONCLUSIONS: Echocardiographic definition for CMRI yielded the diagnosis of LVHT in only 47%. When looking for LVHT by CMRI, LV size, function, and extension of LVHT have to be considered. Copyright (c) 2008 Wiley Periodicals, Inc.     

心肌致密化不全11例的临床与彩色多普勒超声心动图

目的 探讨彩色多普勒超声心动图诊断心肌致密化不全的临床价值.方法 心肌致密化不全11例,年龄11个月～75岁,用GE VIVID7,SEQUOIA 256彩色超声仪,探头频率2.5～4.5MHz,分析其超声特点及临床表现.结果 11例患者左心室不同程度扩大,心肌收缩功能减退,心尖部心肌由两层构成,外层致密心肌边薄,内层心肌呈小梁网状海绵样结构,有丰富的隐窝,海绵样心肌厚度与致密心肌厚度比值为2.5～5.4,彩色多普勒显示隐窝内低速血流与左心室腔相通.结论 左心室心肌致密化不全有明显的超声特征,彩色多普勒超声心动图是惟一的诊断手段.     

Comprehensive contrast and 3-dimensional echocardiographic imaging of left ventricular noncompaction cardiomyopathy

A 62-year-old woman with a history of heart failure, hypertensionand stroke presented to hospital with dyspnea. Transthoracicechocardiography revealed significant left ventricular dysfunction;with contrast-enhanced 2- and 3-dimensional echocardiography,extensive hypetrabeculation of the left ventricular myocardiumwas visualized, leading to a diagnosis of noncompaction cardiomyopathy.Apical thrombi were excluded with contrast imaging; however,the patient was systemically anticoagulated owing to the presenceof noncompaction cardiomyopathy in the setting of prior stroke.This case report demonstrates remarkable imaging of the leftventricular myocardium achieved with contrast-enhanced 3-dimensionalechocardiography in the setting of noncompaction cardiomyopathy.