超声心动图诊断心肌致密化不全22例

心肌致密化不全（noncompactionofventficularmyocardium,NVM）是一种罕见的先天性心脏疾病,系胚胎发育过程中心内膜及心肌停止发育而导致的心肌疾病,有家族遗传倾向。该病可单独存在,也可与其他先天性疾病并存,临床及超声医师对该病容易误诊和漏诊,我院2008-01至2012—10超声检查发现心肌致密化不全患者22例。     

超声心动图对心肌致密化不全的声像图分析

目的分析心肌致密化不全的声像图表现并评价其诊断意义。方法对我院9例超声诊断为心肌致密化不全患者的受累心肌部位和程度及腔室大小变化进行回顾性分析。结果 9例患者中5例受累心肌部位为左室乳头肌水平以下大部分心肌,较厚处约18 mm,3例局限于心尖部,1例合并右心室心尖部受累并心尖部血栓形成,其中8例患者出现左房、室腔扩大,其中2例合并右心扩大。结论超声心动图检查心肌致密化不全有较高的诊断价值,早期诊断对预后有重要意义。     

心肌致密化不全的超声心动图诊断（附5例分析）

目的 :观察心肌致密化不全的心内结构特点及心功能情况。材料和方法 :回顾性分析 5例心肌致密化不全患者的超声心动图及临床表现。结果 :4例患者致密化不全的心肌受累范围偏小 ,局限在整个心尖部或左室侧壁心尖部 ,左心室内径正常 ,左室整体收缩功能正常 ,舒张功能轻度减低 2例 ;1例患者致密化不全的心肌受累范围较大 ,伴前间壁心梗 ,室壁明显变薄 ,左室收缩功能减低。结论 :超声心动图对心肌致密化不全可以做出明确诊断。     

孤立性左室心肌致密化不全的超声诊断

孤立性左室心肌致密化不全以往又称为海绵状心肌病，是由于在胚胎形成过程中心肌致密化过程失败所造成的一种罕见的先天性心脏畸形，其解剖学特征是受累的心腔内可见无数突起的粗大肌小梁及小梁间深陷的隐窝。现将我们应用超声心动图诊断的5例孤立性左室心肌致密化不全总结分析如下。     

心肌致密化不全1例

患者女,57岁。因＂劳力性胸闷、气短2年,呼吸困难,夜间端坐呼吸5天＂入院。入院查体：入院查体：BP 100/60mmHg,心律不齐,心界左下扩大,二尖瓣、三尖瓣、主动脉瓣膜区可闻及2/6级收缩期样杂音。双下肢轻度浮肿,呈凹陷性。心电图示：心房纤颤（偶见心室融合波）起搏器心律,I、V6～V9导联异常Q波,心室内传到阻滞,Q-T间期延长,心超示：全心增大,肺动脉轻度高压,左室后壁基底段至左心室心尖段肌小梁增多增粗,呈海绵状,肌小梁与陷窝明显（图1）,左心整体收缩无力,射血分数约为25．6％,彩色多普勒显示可探及隐窝内有血流与心腔相通（图2）。     

成人孤立性心室心肌致密化不全

孤立性心室心肌致密化不全是一种胚胎期心肌发育异常的心肌病。就大组病例从历史、WHO分类、临床表现、诊断、病理解剖、遗传学、自然病史及预后和治疗选择等几方面加以讨论。     

成人孤立性心室心肌致密化不全

孤立性心室心肌致密化不全是一种胚胎期心肌发育异常的心肌病.就大组病例从历史、WHO分类、临床表现、诊断、病理解剖、遗传学、自然病史及预后和治疗选择等几方面加以讨论.     

心肌致密化不全1例

病例男，56岁，因“反复心累、气紧2年，加重2w”入院。查体：血压138／72mmHg，端坐位，口唇紫绀，颈静脉怒张，肝颈征阳性，双肺呼吸音低，双侧中下肺闻及少许细湿哕音，心界左下扩大，心尖搏动位于第5～6肋间左锁骨中线外2．0cm，未闻及病理性杂音，肝肋下可触及约3cm，双下肢轻度水肿。     

成人孤立性左室心肌致密化不全的少见MRI表现

【摘要】目的：探讨成人孤立性左室心肌致密化不全（ILVNC）的少见MR表现,提高对该病的诊断及鉴别诊断能力。方法：回顾性分析本院2009年8月-2017年12月经心脏超声及MRI共同诊断的20例成人ILVNC的MR影像资料（诊断标准为舒张末期致密化不全节段心肌厚度与致密心肌厚度比值大于2.3）,所有患者均行心肌短轴快速自旋回波TSE-T1WI及TSE-T2WI脂肪抑制序列及全心短轴电影序列,其中14例行增强扫描,6例行胸部CT平扫检查。1例最终行心脏移植。20例ILVNC中男13例,女7例,年龄15～68岁,平均年龄（39±3）岁。结果：同时合并右室心肌致密化不全者5例。左室心肌致密化不全心尖段受累最多,共17个节段（85.0%,17/20）,3例心尖未受累,第2、3、8、9节段所有患者均未受累;3例特殊表现病例（2例致密化不全节段单独累及心尖且伴心尖室壁瘤样膨出,表现为数个粗大肌小梁突入腔内,1例单独累及心底下壁第4节段,同样表现为数个粗大肌小梁突入腔内）;1例ILVNC于胸部CT见散在心内膜下钙化影;2例ILVNC出现心尖部血栓,1例血栓位于心尖部心内膜下,1例血栓位于致密化不全心肌的粗大小梁间隙内,增强后电影序列得以显示清晰; 14例增强扫描中,6例共84个节段出现延迟强化（致密化不全节段29个,正常节段55个）,强化以心内膜下分布为主,部分呈透壁样,部分位于右室插入点。心脏移植病理符合心肌致密化不全改变。结论：除典型的心肌两层结构之外,ILVNC可以出现其他不典型MR表现及伴随征象,心脏MR能够很好地显示并诊断此病。     

成人孤立性心肌致密化不全1例

患者,男,33岁。活动后心悸,气促1年,伴双下肢浮肿6个月就诊。患者于1年前无明显诱因出现活动后心悸,呼吸困难。6个月前出现双下肢浮肿,腹胀,纳差,乏力,在当地县医院诊断为“扩张型心肌病”予强心利尿等治疗稍好转,但时有反复,故转诊我院。体检：血压100／65mmHg。无紫绀。无颈静脉怒张。右下肺呼吸音减低。未闻及干湿性哕音。心界扩大,心率96次／min,律齐,未闻及杂音。肝肋缘下2cm,移动性浊音弱阳性。双下肢无水肿。心电图：频发室性早搏。胸片：双肺纹理增粗,右侧少量胸腔积液;心影增大。腹部B超：肝肿大。少量腹水。彩色超声心动图（图1,图2）：左室舒张末径7．17cm,左房内径4．60cm,右心大小正常。主、肺动脉内径正常。室间隔厚度正常。在左室腔内可探及无数突起增大的肌小梁状结构和深陷的肌小梁间隐窝,相互交错呈海绵状,彩色多普勒可探及隐窝内有血流与心腔相通。乳头肌水平以下至心尖部肌小梁状结构逐渐增多,占据大部分心尖区心腔。左室腔内未见附壁血栓声像。     

孤立性心肌致密化不全的MRI诊断

目的探讨孤立性心肌致密化不全(INVM)的MRI表现及其对该病诊断的价值。方法12例INVM患者均经超声心动图诊断。采用MRI不同脉冲序列，分析INVM心室结构、运动功能及心肌灌注的MRI表现。结果(1)左心室单独受累9例，双室受累3例，未见右室单独受累者。左心室心尖段及前壁中间段为最常见受累部位(12例)；(2)心肌增厚并能分层，非致密化心肌信号不均匀，由多发、粗大、交错排列的肌小梁及小梁隐窝组成，致密心肌变薄，信号均匀；(3)小梁隐窝内血栓2例；(4)心肌灌注成像首过期，6例14段(14／99段)可见灌注缺损；延迟期左心室心肌强化4例23段(23／99段)，其中1例伴右室心尖心肌强化；(5)左心室普遍收缩功能不良4例，节段性功能不良5例。结论MRI能清晰显示INVM的形态学改变、心室节段性及整体运动功能异常、心肌血流灌注及纤维化的程度和范围，对INVM诊断及预后评估具有重要价值。     

An aviator with idiopathic dilated cardiomyopathy

The clinical presentation, evaluation and diagnosis of an aviator with dilated cardiomyopathy of determined etiology are discussed. The aeromedical disposition and prognosis of this patient are also presented.     

Quantification of enhancement of left ventricular myocardium in patients with dilated cardiomyopathy using delayed enhanced MR imaging

The purpose is to evaluate delayed enhancement (DE) of the myocardium in patients with dilated cardiomyopathy (DCM), compared with control subjects. We also evaluated the interrelationships of DE and contractile function.DCM patients (n = 42) and 14 control subjects were evaluated by DE MR imaging, acquired using a two-dimensional segmented inversion-recovery prepared gradient-echo sequence (TI = 250 ms), 15 min after intravenous administration of 0.2 mmol/kg gadolinium.For the myocardium of left ventricle (LV), we traced epicardial and endocardial borders, and regions of interest (ROIs) were placed in each slice. For analysis of DE images, the signal-to-noise ratio (SNR) and the contrast-to-noise ratio (CNR) of the LV myocardium were calculated. The averaged SNR (aSNR) and averaged CNR (aCNR) per slice of the LV myocardium were calculated. In the DCM group, we also evaluated the interrelationship of DE and the contractile function of the LV.Mean aSNR was not significantly different between the studied groups; however, mean aCNR was significantly higher in the DCM group (3.5 ± 3.1) than in control subjects (−4.1 ± 2.1).In the DCM group, aCNR was moderately related to LV ejection fraction (LVEF) (r = 0.52, P < .0001). Mean aCNR was significantly higher in the DCM group with low LVEF (<25%) (6.0 ± 2.8) than in the DCM group with high LVEF (≥25%) (2.0 ± 2.3).In DE MR imaging, the LV myocardium of DCM usually has high aCNR, which may suggest fibrosis. Quantification of aCNR may contribute to the diagnosis of DCM. The level of aCNR seems to correlate with LVEF. Using this technique, quantification of aCNR is objective and very useful for the diagnosis of DCM and contractile function of LV.     

孤立性左心室心肌致密化不全患者的心肌灌注显像研究

目的 分析孤立性左心室心肌致密化不全（ILVNC）患者的心肌灌注受损情况,及其与心脏MRI结果之间的关系.方法 前瞻性入选19例（男14例,女5例,年龄15 ～ 76岁）经心脏MRI确诊的ILVNC患者,同期行99Tcm-MIBI SPECT MPI.采用标准的17节段法分析MRI及SPECT图像.计算MRI所示各节段心肌非致密层（NC）与致密层（C）厚度比值（NC/C）,NC/C＞2.3者诊断为心肌致密化不全.计算并比较致密不全节段和致密化节段延迟强化（DE）出现率及心肌灌注异常的出现率.两样本率的比较采用χ^2检验;lgLVEF（LVEF由MRI测定）和心肌灌注受损节段数、致密化不全节段数和DE节段数的关系采用Pearson或Spearman法分析.结果 19例患者中16例（84.2％） MPI出现灌注受损;MRI示19例共有致密化不全节段107个和致密化节段216个,灌注异常出现率分别为33.6％（36/107）和31.9％（69/216）,差异无统计学意义（χ^2=0.09,P＞0.05）.共31个节段出现DE,致密化不全节段和致密化节段DE出现率分别为5.6％（6/107）和11.6％ （25/216）,差异也没有统计学意义（χ^2=2.94,P＞0.05）.DE节段灌注异常出现率高于非DE节段[54.8％（17/31）与30.1％（88/292）;χ^2=7.80,P＜0.01].lgLVEF与致密化不全节段数、DE节段数、灌注受累节段数间相关系数均无统计学意义（r=-0.35、0.15和-0.34,均P＞0.05）.结论 大部分ILVNC患者存在不同程度的心肌血流灌注受损,MRI所示致密化不全和非致密化不全心肌均可出现;心肌灌注受损在该病的发生及进展中的作用尚需进一步研究.     

孤立性左室心肌致密化不全的MRI特征

目的:研究经临床综合诊断确诊为孤立性心肌致密化不全(IVNC)的患者,分析MRI特征。方法:利用心脏MRI对30例IVNC患者的左心房、室径线及左心功能进行分析,将左心室划分为17节段,对致密化不全的节段计数,测量左室各节段致密化心肌(C)和非致密化心肌(NC)的厚度,计算NC/C比值。并分析延迟增强扫描后左心室各节段心肌的强化特点。22名成人健康者纳入对照组进行对比研究。结果:心脏MRI显示IVNC患者左心房、室扩张,有球形化的趋势〔球形指数(SI)=0.74±0.11〕;左室射血分数(LVEF=32.8±13.6)下降;IVNC患者致密化不全的节段数为9±2。所有IVNC患者的心尖段(第17节段)均受累,其他最常见的受累节段为侧壁中段、心尖段(第16、12、11节段),而室间隔基底段、中段(第2、3、8、9节段)未见受累。IVNC患者最常发生致密化不全的节段其致密化心肌厚度变薄,而不发生及很少发生致密化不全的节段,其致密化心肌厚度与正常对照组对应节段室壁厚度无差别。IVNC患者NC/C比值为3.2±0.8。延迟增强扫描发现11例IVNC患者共68个节段室壁有延迟强化。结论:心脏MRI既能清楚显示左室各节段(尤其是心尖部)的非致密化心肌,又能准确测量NC/C比值,是诊断IVNC的理想检查方法。     

Myocardial defect detected by123I-BMIPP scintigraphy and left ventricular dysfunction in patients with idiopathic dilated cardiomyopathy

The present study examined the role of myocardial fatty acid in patients with idiopathic cardiomyopathy (DCM) by means of¹²³I-β-methyl-p-iodophenyl pentadecanoic acid (¹²³I-BMIPP) scintigraphy. Thirteen patients underwent¹²³I-BMIPP imaging,²⁰¹Tl imaging and echocardiography. All patients showed defective myocardial uptake of¹²³I-BMIPP and²⁰¹Tl. The left ventricular end-diastolic dimension (64.1 ± 7.3 mm vs. 55.6 ± 1.5 mm, p < 0.05) and end-systolic dimension (52.4 ± 8.0 mm vs. 40.6 ± 2.1 mm, p < 0.01) were significantly larger in the large defect group (¹²³I-BMIPP defect score (DS) > 8) than the small defect group (DS < 7). The % fractional shortening (%FS) was also significantly smaller (18.6 ± 3.8% vs. 27.0 ± 3.3%, p < 0.01) in the large defect group. The¹²³I-BMIPP DS correlated statistically with %FS (r = 0.75, p < 0.01), while the²⁰¹Tl DS did not (r = 0.41, ns). We conclude that the patients with DCM revealed a¹²³I-BMIPP uptake defect and the defect reflected the degree of left ventricular dysfunction.     

Assessment of right ventricular oxidative metabolism by PET in patients with idiopathic dilated cardiomyopathy undergoing cardiac resynchronisation therapy

Purpose Right ventricular (RV) performance is known to have prognostic value in patients with congestive heart failure (CHF). Cardiac resynchronisation therapy (CRT) has been found to enhance left ventricular (LV) energetics and metabolic reserve in patients with heart failure. The interplay between the LV and RV may play an important role in CRT response. The purpose of the study was to investigate RV oxidative metabolism, metabolic reserve and the effects of CRT in patients with CHF and left bundle brach block. In addition, the role of the RV in the response to CRT was evaluated.Methods Ten patients with idiopathic dilated cardiomyopathy who had undergone implantation of a biventricular pacemaker 8±5 months earlier were studied under two conditions: CRT ON and after CRT had been switched OFF for 24 h. Oxidative metabolism was measured using [¹¹C]acetate positron emission tomography (K_mono). The measurements were performed at rest and during dobutamine-induced stress (5 g/kg per minute). LV performance and interventricular mechanical delay (interventricular asynchrony) were measured using echocardiography.Results CRT had no effect on RV K_mono at rest (ON: 0.052±0.014, OFF: 0.047±0.018, NS). Dobutamine-induced stress increased RV K_mono significantly under both conditions but oxidative metabolism was more enhanced when CRT was ON (0.076±0.026 vs 0.065±0.027, p=0.003). CRT shortened interventricular delay significantly (45±33 vs 19±35 ms, p=0.05). In five patients the response to CRT was striking (32% increase in mean LV stroke volume, range 18–36%), while in the other five patients no response was observed (mean change +2%, range –6% to +4%). RV K_mono and LV stroke volume response to CRT correlated inversely (r=–0.66, p=0.034). None of the other measured parameters, including all LV parameters and electromechanical parameters, were associated with the response to CRT. In responders, RV K_mono with CRT OFF was significantly lower than in non-responders (0.036±0.01 vs 0.058±0.02, p=0.047).Conclusion CRT appears to enhance RV oxidative metabolism and metabolic reserve during stress. Patients responding to CRT appear to have lower RV oxidative metabolism at rest, suggesting that the RV plays a significant role in the response to CRT.     

Cardiac magnetic resonance imaging findings in a patient with noncompaction of ventricular myocardium

Noncompaction of ventricular myocardium (NCVM) is a rare cardiomyopathy characterized by numerous prominent trabeculations in the ventricular wall and deep intertrabecular recesses communicating with the ventricular cavity. This article reports a 33-year-old female with a familial history of cardiovascular disease, who presented with shortness of breath and palpitations. Transesophageal echocardiography and cardiac magnetic resonance imaging (MRI) were consistent with the diagnosis of NCVM. The advantages of MRI in depicting both the morphological features and pathological characteristics of NCVM were presented.