小儿心内膜弹力纤维增生症的超声诊断

本文对32例小儿心内膜弹力纤维增生症容易误诊的原因进行分析,并提出了二维超声心动图在本病的特点,为早期诊断本症提供了重要的客观依据,对其疗效评价,降低死亡率均具有重要的临床意义。     

超声血管增强技术联合断层超声显像技术在胎儿先天性心脏病诊断中的应用

目的探讨超声血管增强技术(VET)联合断层超声显像技术(TUI)在胎儿先天性心脏病诊断中的应用价值.方法应用VET-TUI技术对二维超声筛查诊断为先天性心脏病的17例孕22~30周中晚孕期胎儿进行检查,在获取满意的胎儿心脏容积数据后通过TUI模式,对胎儿心脏进行断层分析,并与产后临床检查结果进行对照分析.结果产前应用VET-TUI技术17例胎儿均获得满意的心脏容积数据及胎儿心脏断层成像图像,17例先天性心脏病胎儿中单一性先天性心脏病3例,复合性先天性心脏病14例.17例胎儿心脏畸形包括心内膜垫缺损5例、永存左位上腔静脉7例(其中1例下腔静脉离断合并异常连接)、右心室双出口4例(包括左侧心室解剖右心室双出口)、肺动脉狭窄6例、室间隔缺损8例、主动脉弓缩窄2例、大动脉转位1例、主动脉闭锁2例、右位主动脉弓3例、左心室发育不良2例、右心室发育不良1例、单心室1例、三房心1例、镜面右位心1例.产前超声诊断与引产后尸检结果相符合10例(10/17),与产后超声心动图复查结果相符合5例(5/17),误诊2例(1例超声诊断永存动脉干,尸检证实为升主动脉闭锁,1例超声诊断单心室,尸检证实为左心室发育不良).结论应用VET-TUI技术可增加超声图像的清晰度,使胎儿心脏与大血管容积图像清晰直观,易于辨别,较常规二维超声心动图能提供更多胎儿心脏细微解剖结构的诊断信息.     

Two-dimensional echocardiographic features of double outlet left ventricle

In a cyanotic newborn infant, the diagnosis of double outlet left ventricle was made from the two-dimensional echocardiographic examination. The diagnosis was later confirmed at cardiac catheterization and surgery. The parasternal and subcostal views were especially useful for identification of the origin of both great arteries from the morphologic left ventricle. A review of the medical literature since 1967 revealed 77 cases of double outlet left ventricle, most of which were diagnosed only at surgery or postmortem examination. The anatomic features demonstrated with two-dimensional echocardiography in this case are representative of the findings cited most often in the cases reported in the medical literature.     

Late-diastolic forward flow in the aorta induced by left atrial contraction.

Late-diastolic forward flow is a well-described phenomenon detectable by Doppler echocardiography in the pulmonary trunk. It is supported by a restrictive right ventricular diastolic function and by a low end-diastolic pulmonary artery pressure. A similar phenomenon for the left ventricle and the aorta has not been described. We report a case of a preterm infant with aortic stenosis and endocardial fibroelastosis, who underwent balloon valvuloplasty. Restrictive left ventricular diastolic filling led to high left atrial pressure (27 mm Hg) and a very pathologic ratio of early-to-late peak velocities (2.6) for an infant of 29 weeks' gestation. In combination with a low diastolic aortic pressure (24 mm Hg) caused by moderate aortic regurgitation after intervention, a late-diastolic forward flow was detectable in the aorta during left atrial contraction with pulsed Doppler echocardiography.     

Assessment of cardiac performance by first pass radionuclide angiocardiography in infants and children with normal heart and endocardial fibroelastosis

Peak to peak time from the right and to the left ventricle (PPT), left ventricular ejection fraction, left ventricular peak ejection rate and left ventricular peak filling rate were measured by first pass radionuclide angiocardiography in 27 infants and children with normal heart and in 8 patients (18 studies) with endocardial fibroelastosis. In normal subjects, the PPT significantly correlated with heart rate (r = -0.87, p less than 0.001). A PPT corrected by the heart rate (cPPT) was calculated by rotation of the regression equation relating the variables: cPPT = PPT + 0.018 X (heart rate)-2.1. The cPPT averaged 3.0 +/- 0.0 (mean +/- S.E.) sec. Consequently, there was no significant correlation between the cPPT and heart rate, but the cPPT and body surface area are significantly correlated (r = 0.41, p less than 0.05). Left ventricular ejection fraction, peak ejection rate and peak filling rate averaged 68 +/- 2%, 4.2 +/- 0.3/sec and 4.8 +/- 0.3/sec, all of which were independent of the heart rate and body surface area. In patients with endocardial fibroelastosis, the cPPT was prolonged (4.7 +/- 0.4 sec), and left ventricular ejection fraction, peak ejection rate and peak filling rate were all reduced (28 +/- 4%, 1.7 +/- 0.2/sec and 1.8 +/- 0.2/sec). These results indicate that parameters obtained from the radionuclide angiocardiography are useful for evaluating cardiac performance in patients with endocardial fibroelastosis.     

超声心动图诊断婴幼儿心内膜弹力纤维增生症

目的 探讨超声心动图在婴幼儿心内膜弹力纤维增生症（EFE）诊断与鉴别诊断中的价值.方法 对30例 EFE患儿（EFE组）进行超声心动图检查,超声测量房室内径、心内膜厚度、瓣膜血流及心功能指数.另选同期健康婴幼儿30例为对照组.结果 超声显示EFE组有心内膜增厚、回声增强,二尖瓣瓣膜反流,左室收缩功能明显减低;左室舒张末内径（LVDd）扩大,而射血分数（EF）、心室短轴缩短率（FS）、每搏指数（SI）、心脏指数（CI）均降低,与对照组比较差异均有统计学意义（P＜0.05）;峰值速度比（E/A）、等容舒张时间（IVRT）两组间比较差异均无统计学意义（P＞0.05）.结论 超声心动图对EFE的早期诊断及疗效评价具有重要的临床价值.     

左冠状动脉异常起源于肺动脉的超声诊断与手术矫治分析

目的结合左冠状动脉异常起源于肺动脉（ALCAPA）患者的CT表现及术中肉眼观察情况,探讨超声心动图对左冠状动脉起源于肺动脉的诊断价值。 方法回顾分析2008年1月至2015年5月武汉亚洲心脏病医院经手术证实的11例ALCAPA患者的超声心动图表现,并与术前CT和术中诊断比较。 结果11例患者CT检查与术中肉眼观察诊断结果相符,超声心动图表现与CT检查结果、术中肉眼诊断结果比较,8例诊断符合,3例诊断不符合,其中2例超声检查分别误诊为心内膜弹力纤维增生症（EFE）和右冠状动脉-肺动脉瘘（CPF）,1例术前超声诊断为左冠状动脉扩张,二尖瓣前叶裂合并重度关闭不全,而CT检查及术中诊断发现存在2支冠状动脉左前降支,1支起源于左冠状动脉,另一支起源于肺动脉。 结论超声心动图可及时、准确诊断ALCAPA,但对明确心腔外的血管走行、起源有一定局限性,需结合CT等其他影像检查方法,进一步明确冠状动脉的走行及发育,以评估手术适应证及预后,为临床提供更为精确的诊断依据。     

不同切面超声心动图诊断胎儿心脏畸形的临床研究

目的探讨不同切面超声心动图诊断胎儿心脏畸形的价值。方法对1341例孕妇行胎儿超声心动图检查,采用二维、彩色及频谱多普勒从常规取胎儿四腔心、五腔心、左心室流出道及右心室流出道长轴、大动脉短轴、主动脉弓及动脉导管弓长轴等切面观察胎儿心脏解剖结构及血流状况。并与引产后的尸检结果进行对照。结果本组共检出胎儿先天性心脏畸形30例,经引产后胎儿尸体解剖和产后新生儿随访证实与产前完全相符合28例,占93．33％（28／30）,有2例复杂性畸形与产前诊断基本相符合,占6．67％（2／30）。在出生后的新生儿中发现2例室间隔缺损,1例房间隔缺损漏诊,漏诊率为9．09％（3／33）。畸形类型包括室间隔缺损3例,房间隔膨胀瘤3例,心内膜垫缺损1例,单心室4例,大动脉转位2例,右室双出口2例,永存动脉干2例,左心室发育不良3例,右心室发育不良2例,三尖瓣闭锁2例,三尖瓣下移畸形2例,肺动脉闭锁1例,严重心律失常3例。产前超声诊断符合率为90．91％（30／33）。结论不同切面超声心动图可用于产前胎儿心脏畸形的诊断。     

超声心动图诊断胎儿心肌病

目的 探讨胎儿超声心动图诊断胎儿心肌病(FCM)及其并发症的价值.方法 对11 360胎胎儿进行超声心动图检查;对其中诊断为胎儿心肌病或可疑心肌原发病变者进行分析,并与大体病理结果及出生后超声心动图表现进行对照分析.结果 11 360胎中检出FCM 19胎,检出率为0.167%(19/11 360).其中扩张型心肌病10胎(累及双心室4胎、右心室4胎、左心室1胎、右心房1胎)、肥厚型心肌病2胎(累及双心室1胎,室间隔1胎)、心内膜弹力纤维增生症4胎(原发型1胎、继发型3胎)、心肌致密化不全3胎(累及双心室2胎、右心室1胎),FCM累及心室中以双心室为著.结论 胎儿超声心动图可对FCM做出诊断及分型,且可评估胎儿心脏功能及血流动力学的变化,并判断FCM的预后.     

Delineation of intracardiac shunts using contrast echocardiography.

Contrast echocardiography is useful to visualize the endocardial borders of the left ventricle and improve the signal intensity of spectral Doppler signals. It can also help to define intracardiac flow dynamics in complex situations. We report 2 cases where contrast echocardiography improved the delineation of complex shunts and also provided new information not available by conventional echocardiography.     

超声心动图对心内膜弹力纤维增生症患儿血流动力学变化的分析研究

目的 应用二维多普勒超声心动图技术对心内膜弹力纤维增生症(EFE)患儿的血流动力学变化进行分析研究.方法 超声心动图检查EFE患者32例,平均年龄(1.5±0.6)岁,正常对照(NC)组30例.结果 EFE组与NC组比较,各指标均有显著性差异(P<0.001),超声参数的特点为舒张期左房内径(LADd)、左室内径(LVDd)、室间隔厚度(IVSTd)、左房射血力(LAF)、室壁应力指数(ws I～Ⅲ)、峰值速度A(A峰)及等容舒张时间(IRTs)增大;但射血分数(EF)、心脏指数(CI)、心室短轴缩短率(FS)、峰值速度E(E峰)、峰值速度比(E/A)、舒张早期快速充盈加速度(AC)、1/3及1/2充盈分数(1/3FF、1/2FF)及标准化充盈分数(NPFR)降低;将EFE组治疗前后比较除IVSd、A峰及IRTs无显著性差异(P>0.05)~b,其余各指标均有显著性差异(P<0.01-0.001).结论 EFE患儿可出现房室腔扩大、心脏收缩和舒张功能降低、室壁应力增大及左房射血力代偿性增加;而二维多普勒超声心动图对于EFE患儿的早期诊断、疗效评价及预后估测都有极为重要的临床价值.     

Electron beam computed tomography appearance of endocardial fibroelastosis EBCT appearance of endocardial fibroelastosis

Recent reports of endocardial fibroelastosis (EFE) have not reported the disease to be correctly diagnosed during the patients' life spans. Our purpose in this communication is to provide some feasible approaches toward correct diagnosis at the primitive stage and possible correlations to the prognosis. We analyzed five cases of EFE from 1997 to 2001. Four had pathology proven EFE. Data were sampled from the clinical symptoms, eletrocardiography, echocardiography, electron beam computed tomography (EBCT), management, and prognosis. A case of anomalous left coronary artery originating from main pulmonary artery diagnosed EFE correctly before death by utilizing an EBCT. The second case was double outlet of the right ventricle with severe calcification and fibrosis shown on EBCT studies, while the third case had severe calcification over both apices. Both patients required heart transplantation. The fourth case, with a decreasing ejection fraction, was idiopathic hypertropic subaortic stenosis with mild calcification and fibrosis on the EBCT images. The last stationary case had severe aortic stenosis with trivial fibrotic change and calcification. We propose that EBCT may accurately help to diagnose EFE before pathology confirmation. The magnitude of calcification and fibrotic thickness in the myocardium of the EBCT imaging may predict the outcome of EFE.     

Modern methods for the instrumental diagnosis of intracavitary thrombosis of the left ventricle in myocardial infarct

To study the sensitivity and specificity of two-dimensional echocardiography in the diagnosis of left ventricle thrombosis which often attends myocardial infarction and to define diagnostic potentialities of the new research methods (digital subtraction ventriculography and MR tomography), 449 patients with acute transmural myocardial infarction were examined. Based on the comparison of the echocardiographic readings and morbid anatomy data it has been shown that the sensitivity of two-dimensional echocardiography in revealing left ventricle thrombosis amounts to 89%, specificity to 88%. Digital subtraction ventriculography has a lower (77%) sensitivity, with the specificity being satisfactory enough (88%). Meanwhile MR tomography enables a highly precise diagnosis of left ventricle thrombosis and can be used as a supplementary diagnostic method in questionable cases and in thrombi small in size or in unsatisfactory ultrasonic visualization of the heart structures.     

时空关联成像检测胎儿复杂心脏畸形的应用价值

目的 评价时空关联成像(spatio-temporal image correlation,STIC)在诊断胎儿复杂心脏畸形中的应用价值.方法 选择二维超声筛查疑似复杂心脏畸形的25例中晚孕期胎儿,胎龄20～30周,平均(25.88±8.3)周.进行心脏和胸部的自动扫查,应用STIC技术后处理获得动态三维图像,对获得的容积数据应用正交三平面模式、表面成像模式、反转模式和最小模式离线分析进行心脏复杂畸形的诊断,并与尸解或出生后彩色多普勒超声复查的结果对照分析.结果 25例胎儿通过STIC技术均可获得满意的胎儿心脏容积数据.发现胎儿复杂心脏畸形中.右室双出口1例,永存动脉干1例,完全型大动脉转位3例,矫止型大动脉转位1例,完全性心内膜垫缺损4例,右室发育不良1例,左室发育不良2例,三尖瓣闭锁1例,肺动脉闭锁1例,法洛四联症3例,单心房1例,两腔心畸形4例,法洛五联症1例,房间隔缺损、室间隔缺损并冠状静脉窦扩张1例.23例与尸体解剖或产后复查结果相一致,敏感性为92%(23/25).结论 STIC技术能快速准确地检测出胎儿复杂心脏畸形,对妊娠预后具有重要的指导意义.     

超声心动图对胎儿心脏结构畸形的诊断价值

目的：探讨超声心动图检测胎儿心脏结构异常的诊断价值及临床意义.方法：应用彩超显示六个标准切面,筛查不同孕期高危胎儿心脏,并与产后作超声心动图对比,个别经尸体解剖验证.结果：230例高危胎儿中检出先天性心脏病19例,其中包括永存动脉干2例,完全性心内膜垫缺损3例,单心房1例,大动脉转位2例,单心室2例,左室憩室1例,右室双出口2例,主动脉缩窄1例,法洛氏四联症1例,肺动脉狭窄1例,室间隔缺损2例,左心室肿瘤1例.结论：胎儿超声心动图的应用有助于早期检出心脏结构异常并指导患胎的处理.     

Doppler color flow imaging in detection and mapping of left coronary artery fistula to right ventricle and atrium

Two patients who had left coronary artery fistulas that drained into the right ventricle (case 1) and right atrium (case 2) were studied with combined two-dimensional echocardiography and Doppler color flow imaging. The origin of the fistulas from the left coronary artery, their course, and drainage sites were readily identified. These cases illustrate the enhanced identification of left coronary artery fistulas and the drainage sites with the addition of Doppler color flow imaging to two-dimensional echocardiography.     

先天性冠状动脉畸形的超声心动图诊断

目的探讨超声心动图诊断小儿先天性冠状动脉畸形的意义。方法对本院诊治的30例先天性冠状动脉畸形的超声心动图结果与心血管造影、手术结果进行分析比较。结果冠状动脉异位开口于肺动脉4例,占13%;单支冠状动脉畸形3例,占10%;冠状动脉瘘23例,占77%,累及右冠状动脉12例（52%）,其中多个瘘口1例,左冠状动脉11例（48%）,瘘入右侧心腔11例（48%）,瘘入肺动脉11例（48%）,瘘入左侧心腔1例（4%）。冠状动脉畸形中伴有其他先天性畸形6例（26%）。超声心动图诊断敏感性93%,特异性100%。其中对冠状动脉瘘及瘘口的形态、数目、瘘入的部位准确性达100%,对冠状动脉异位开口于肺动脉及单支冠状动脉畸形各有1例误诊为弹力纤维增生症。结论超声心动图对先天性冠状动脉畸形有较高的诊断价值,可作为首选诊断方法。对心脏扩大、心内膜增厚、心功能弥漫性降低的弹力纤维增生症样超声心动图改变必须保持对冠状动脉畸形的警惕性,应仔细探测左右冠状动脉及分支,并结合心电图等临床资料进行分析。     

Enhanced endocardial visualization with noncontrast harmonic imaging during stress echocardiography.

The diagnostic value of echocardiography hinges on the reader's ability to adequately visualize the endocardium of the left ventricle. This study was designed to evaluate the potential benefit of noncontrast harmonic imaging to enhance endocardial visualization. Eighty consecutive outpatients who underwent treadmill stress echocardiography were randomly assigned to either fundamental or harmonic imaging. The echoes were interpreted by 2 experienced readers. Compared with fundamental imaging, harmonic imaging of tissue improved the overall endocardial visualization score by 35% and 21% for readers 1 and 2, respectively (P <.001). Harmonic imaging also reduced the percentage of nondiagnostic segments by one half (P <.01). In patients undergoing treadmill stress echo, harmonic imaging offers a clinically significant improvement in endocardial visualization.     

左室中段室壁缩短率早期评价高血压患者左室舒张功能

目的　评价左室中段室壁缩短（ｍｉｄｗａｌｌｆｒａｃｔｉｏｎａｌｓｈｏｒｔｅｎｉｎｇ,ＭＦＳ） 率评价高血压患者左室舒张功能的临床价值。方法　对２９２ 例原发性高血压患者进行二维及多普勒超声心动图观察,比较常规测量左室射血分数（ＥＦ）,短轴缩短率（ＦＳ）及左室中段室壁缩短率与左室舒张功能的关系。结果　分别以左室肥厚和左室向心性重构分组,发现左室中段室壁缩短率在肥厚组（Ｐ＝０ ．０１１３） 和向心性重构（ Ｐ＝ ０．００００１） 组明显降低,与舒张功能的改变一致,ＥＦ,ＦＳ在分组比较中无显著差异。多元逐步回归分析结果显示：左室中段室壁缩短率ＭＦＳ较ＥＦ,ＦＳ与舒张功能的关系更为密切。结论　超声测量左室中段室壁缩短率对于早期评估高血压患者舒张功能降低具有重要价值。     

产前超声诊断胎儿主动脉缩窄

目的 探讨产前超声诊断胎儿主动脉缩窄的临床价值.方法 回顾分析10例经尸体解剖证实的主动脉缩窄胎儿的超声心动图资料,总结其异常声像图特点.结果 10例胎儿主动脉缩窄中,7例主动脉弓失去正常柔和的弯曲状,呈细窄僵直状,2例足月胎儿主动脉峡部直径小于2.6mm,1例主动脉弓部血管显示不清;10例均显示右心房室增大,肺动脉及动脉导管增宽,4例动脉导管呈瘤样扩张,3例伴左心发育不良,4例伴室间隔缺损,2例合并右室双出口,1例合并二尖瓣闭锁;产前超声诊断胎儿主动脉缩窄9例,误诊主动脉弓离断1例.结论 产前超声检查对诊断胎儿主动脉缩窄,具有重要的临床价值.