自体心包隔瓣在房室通道缺损矫治手术中的应用

目的报告在房室通道缺损（AVCD）矫治术中应用自体心包片重建人工隔瓣的方法与结果。并与常规成型术式进行对比分析。方法对16例部分型与4例完全型AVCD伴隔瓣发育不良或缺如者进行矫治手术。其中按常规成型术式进行的8例作为对照组，利用修补原发孔型房间隔缺损的自体心包补片末端作成人工隔瓣，同时修补二尖瓣裂和修复房、室间隔缺损的12例作为实验组。结果全部患者无死亡或Ⅲ度房室传导阻滞。实验组术后无返流或轻度返流，心功能恢复良好；对照组术后三尖瓣返流仅3例变为轻度，其余仍为中到重度．右心功能欠佳。2组术后三尖瓣返流改善情况比较差异有统计学意义（P〈0．05）。结论利用自体心包重建人工隔瓣比较符合三尖瓣的解剖结构。能有效地消除术后三尖辩返流。     

超声心动图对Ebstein畸形解剖矫治术近期疗效的评价

目的探讨超声心动图诊断Ebstein畸形的准确性及其对解剖矫治术的近期疗效评价.方法16例行Ebstein畸形解剖矫治术的患者,术前超声心动图评价三尖瓣下移的程度、三尖瓣瓣叶及瓣下腱索发育情况、房化右室的大小、三尖瓣反流的程度,并与术中发现比较.所有患者术后均随访超声心动图,与术前结果进行比较分析.结果16例患者中,超声心动图检查三尖瓣前叶均无下移,但发育冗长,瓣叶运动异常.隔叶及后叶均下移,其瓣叶及瓣下结构发育异常.房化右室的大小为2.0cm×3.5cm至6.0cm×8.0cm.所有病例均有右房扩大、三尖瓣瓣环扩张及三尖瓣反流,其中8例中度反流,8例重度反流.术中发现2例三尖瓣前叶的后1/4下移(分别为8mm和11mm),2例后叶呈螺旋样下移,其余与术前超声心动图检查结果相同.术后1～17个月(平均7个月)随访,超声心动图显示右房明显缩小,房化右室消失,三尖瓣叶均在正常瓣环水平,重建的瓣膜均存活、启闭正常.13例三尖瓣反流消失,1例轻度反流,2例残余中度反流.结论超声心动图可全面评价Ebstein畸形三尖瓣及瓣下结构的发育情况及Ebstein畸形解剖矫治术的近期疗效.     

合并左心功能不全患者行自体心包软环三尖瓣环成形术的近期和远期疗效

目的 分析合并左心功能不全患者行自体心包软环三尖瓣环成形术的近期和远期疗效.方法 选择2014年1月至2015年1月于我院收治的三尖瓣反流合并左心功能不全患者80例,随机分为对照组及观察组,分别给予De Vega三尖瓣成形术及自体心包软环三尖瓣环成形术治疗.收集并比较两组患者一般资料.检查各组患者心功能变化.随访至术后...     

心尖右心两腔观诊断小儿三尖瓣后瓣下移的方法探讨

目的 探讨超声心尖右心两腔观诊断小儿三尖瓣后瓣下移的方法.方法 超声在心尖四腔观以隔瓣为中心,探头顺时针方向旋转至心尖右心两腔观,在旋转中动态显示三尖瓣隔瓣和后瓣的下移程度,观察三尖瓣后瓣的形态、活动和位置.彩色多普勒超声显示三尖瓣反流口位置.结果 15例三尖瓣后瓣和隔瓣下移患儿,超声在心尖右心两腔观均可清晰显示三尖瓣后瓣下移的特征.除1例超声诊断三尖瓣隔瓣和后瓣下移而手术发现前瓣也有轻度下移外,超声与手术结果均相一致.15例患儿彩色多普勒均可显示三尖瓣反流口位置明显下移.结论 心尖右心两腔观是诊断三尖瓣后瓣下移的理想切面.彩色多普勒显示三尖瓣反流口位置下移是超声诊断三尖瓣下移的重要特征.     

经食管超声心动图在三尖瓣下移手术中的应用价值

目的 探讨经食管超声心动图在三尖瓣下移手术中的应用价值.方法 回顾性分析69例三尖瓣下移畸形的术中经食管超声特征.结果 三尖瓣下移畸形体外循环前所有病例经食管超声心动图均能清晰显示三尖瓣瓣叶的解剖特性,6例发现三尖瓣前叶下移,6例三尖瓣后叶发育不良,3例后叶缺如;10例三尖瓣下移畸形成形术后经食管超声心动图显示三尖瓣中度反流,指导再次成形至满意.结论 经食管超声心动图在三尖瓣下移手术中体外循环前能够补充修正诊断,评价体外循环后手术效果,增加手术成功率.     

经食管超声心动图在三尖瓣下移手术中的应用价值

目的 探讨经食管超声心动图在三尖瓣下移手术中的应用价值.方法 回顾性分析69例三尖瓣下移畸形的术中经食管超声特征.结果 三尖瓣下移畸形体外循环前所有病例经食管超声心动图均能清晰显示三尖瓣瓣叶的解剖特性,6例发现三尖瓣前叶下移,6例三尖瓣后叶发育不良,3例后叶缺如;10例三尖瓣下移畸形成形术后经食管超声心动图显示三尖瓣中度反流,指导再次成形至满意.结论 经食管超声心动图在三尖瓣下移手术中体外循环前能够补充修正诊断,评价体外循环后手术效果,增加手术成功率.     

产前超声诊断胎儿三尖瓣下移畸形的应用价值

目的 探讨产前超声诊断胎儿三尖瓣下移畸形的临床价值。方法 回顾性分析6例经胎儿超声心动图诊断为三尖瓣下移畸形的超声诊断资料与随诊结果，总结其异常声像图特点。结果6例胎儿均经尸体解剖证实为三尖瓣下移畸形，其中三尖瓣隔叶下移2例，隔叶及后叶下移3例，隔叶、后叶及前叶下移1例。6例三尖瓣下移畸形中4例伴有功能右室缩小，合并房间隔缺损1例，肺动脉狭窄1例；4例伴有胸、腹腔及心包积液。胎儿三尖瓣下移畸形主要声像图表现为右心扩大，三尖瓣叶附着点下移，心尖到二尖瓣前叶附着点的距离与到三尖瓣隔叶附着点的距离比值≥1．8，形成典型房化右室特征；彩色血流显示三尖瓣重度反流，其反流起源点低，反流面积大，但反流速度低，平均反流速度217．0cm／s。结论 胎儿三尖瓣下移畸形具有特征性声像图表现，产前超声诊断胎儿三尖瓣下移畸形具有重要临床价值。     

经食管超声心动图在三尖瓣下移手术中的应用价值

目的 探讨经食管超声心动图在三尖瓣下移手术中的应用价值.方法 回顾性分析69例三尖瓣下移畸形的术中经食管超声特征.结果 三尖瓣下移畸形体外循环前所有病例经食管超声心动图均能清晰显示三尖瓣瓣叶的解剖特性,6例发现三尖瓣前叶下移,6例三尖瓣后叶发育不良,3例后叶缺如;10例三尖瓣下移畸形成形术后经食管超声心动图显示三尖瓣中度反流,指导再次成形至满意.结论 经食管超声心动图在三尖瓣下移手术中体外循环前能够补充修正诊断,评价体外循环后手术效果,增加手术成功率.     

功能性三尖瓣反流机制的二维及三维超声心动图探索

目的 应用超声心动图探索功能性三尖瓣反流的发生机制,并为手术治疗提供理论基础.方法 对56例左心瓣膜疾病合并三尖瓣反流的患者进行超声检查,测量三尖瓣瓣环直径、瓣叶闭合高度、闭合长度、心室三维容积和射血分数.结果 根据术前反流程度将56例患者分为轻度组(组1)和中度及以上组(组2),组2的瓣环直径、闭合高度、右室容积均明显大于组1( P＜0.01).与反流程度重要相关的因素分别是瓣环直径、闭合高度、右室舒张末容积、肺动脉高压.术前三维超声发现合并瓣叶器质性改变会加重反流.结论 合并左心瓣膜疾患的三尖瓣反流主要受瓣环扩张及肺动脉高压的严重程度影响.三维超声可以更直观地观察瓣叶结构.     

实时三维超声心动图对三尖瓣下移畸形的诊断价值

目的探讨实时三维超声心动图对三尖瓣下移畸形的诊断价值。方法超声心动图诊断为三尖瓣下移畸形的患者18例,使用实时三维超声显示三尖瓣的结构、形态、瓣膜反流情况,以及房化右心室和右心室的形态、功能,然后进行三尖瓣下移畸形和三尖瓣前叶功能（Ess）分级,并与手术结果进行对照。结果三维超声心动图显示18例患者中二维超声误诊2例,余16例患者行ESS分级：A级2例,B级11例,c级3例;三尖瓣反流轻度2例,中度9例,重度5例;合并房间隔缺损14例。手术证实三维超声诊断准确率100％。结论三维超声结合二维超声心动图能准确显示三尖瓣下移畸形,为手术医师提供更多的实用信息。     

Sonographic features of Ebstein anomaly associated with hydrops fetalis: a report of two cases

Ebstein anomaly, an abnormally low insertion of the tricuspid valve, occurs in 0.5% of patients with congenital heart disease. In rare cases, this disorder may be complicated by congestive heart failure in utero and hydrops fetalis. This article reports the prenatal sonographic features of 2 cases of Ebstein anomaly associated with hydrops fetalis. In both cases, fetal echocardiography was performed at 34 weeks of gestation. The 4-chamber view showed fetal cardiomegaly and pericardial effusion. In both cases, the annular attachment of the tricuspid valve leaflets was difficult to demonstrate and so distal that it could easily be confused with papillary muscle in the right ventricular wall. Poor fetal cardiac hemodynamics with severe tricuspid regurgitation was demonstrated by pulsed Doppler imaging. Pulmonary regurgitation was also clearly demonstrated in both cases. Preterm delivery with stillbirth occurred in both cases. Autopsies confirmed the very distal displacement of the tricuspid valve insertion, close to the apex, and enlargement of the right atrium.     

Mechanism of tricuspid regurgitation in paramembranous ventricular septal defect.

Literature has been limited in regard to the mechanisms of tricuspid regurgitation (TR) in patients with paramembranous (perimembranous) ventricular septal defect (VSD). Most observations have noted tricuspid valve clefts or dysplasia. We describe another mechanism for production of TR in association with paramembranous VSD. In 8 patients, we found significant TR produced by the VSD jet pushing the tricuspid anterior leaflet forward to open the tricuspid valve orifice. In these patients, a moderate paramembranous VSD extended slightly below the septal tricuspid leaflet with only partial obstruction of the VSD jet. All patients had restrictive VSD with low right ventricular pressure. This mechanism to produce TR was best defined by intraoperative transesophageal echocardiography, but current higher resolution imaging should allow correct diagnosis. We believe that when this mechanism for TR is found in association with a moderate VSD, surgical VSD closure is warranted.     

Ebstein anomaly in an adult presenting with wide QRS tachycardia: diagnostic and therapeutic dilemmas

A 51-year-old man presented to the emergency department with sustained hemodynamically unstable wide QRS tachycardia and was revived successfully by immediate direct current (DC) cardioversion. There was evidence of previous open heart surgery, possibly atrial septal defect closure. Transthoracic echocardiography showed severe Ebstein anomaly with severe tricuspid regurgitation, no residual atrial septal defect, but with severe right ventricular dysfunction. Subsequent electrocardiograms showed transient atrial fibrillation with no manifest Wolff-Parkinson-White (WPW) accessory pathway during sinus rhythm. The cause of wide QRS tachycardia in this patient may be WPW related or ventricular tachycardia. This case illustrates the diagnostic and therapeutic dilemmas in patients with wide QRS tachycardia and suspected WPW syndrome. In addition, this case demonstrates that unoperated Ebstein anomaly can present in late adult life with tachyarrhythmias.     

胎儿超声心动图诊断埃布斯坦综合征

目的 探讨胎儿超声心动图诊断埃布斯坦综合征及其并发症的价值。方法 2450胎接受胎儿超声心动图检查;对其中的14胎埃布斯坦综合征胎儿的超声心动图进行分析,并与大体病理结果进行对照分析。结果 14胎埃布斯坦综合征胎儿中,超声均显示三尖瓣反流,其中重度11胎(11/14,78.57%);7胎(7/14,50.00%)合并肺动脉瓣狭窄;3胎(3/14,21.43%)肺动脉内径与主动脉内径比>1.1;1胎合并心脏其他畸形;7胎伴胸腔、腹腔及心包积液共。结论 埃布斯坦综合征胎儿易并发重度三尖瓣反流、肺动脉瓣狭窄、肺动脉与主动脉比例变小、胸腔/腹腔/心包积液;胎儿超声心动图对埃布斯坦综合征胎儿的细化诊断是判断预后的重要依据。     

Left heart lesions in patients with Ebstein anomaly

OBJECTIVE: To identify the incidence of left heart abnormalities in patients with Ebstein anomaly, recognizing that left-sided lesions in this patient group have been overlooked. PATIENTS AND METHODS: According to the echocardiography database at the Mayo Clinic in Rochester, Minn, 106 consecutive patients with Ebstein anomaly underwent echocardiography between July 1, 2001, and February 28, 2003. Clinical data as well as electrocardiographic and echocardiographic reports and images were reviewed. RESULTS: Ebstein anomaly was severe in 76 patients (72%). Previous tricuspid valve surgery was reported in 46 patients (43%), and previous closure of an atrial septal defect or patent foramen ovale was reported in 34 patients (32%). Left ventricular (LV) myocardial changes resembling noncompaction occurred in 19 patients (17.9%), LV systolic dysfunction in 7 patients (7%), LV diastolic dysfunction in 34 (36%) of 95 patients, and LV dilatation in 4 patients (4%). Additional left-sided cardiac lesions included mitral valve prolapse in 16 patients (15%), bicuspid aortic valve in 8 (8%), mitral valve dysplasia in 4 (4%), and ventricular septal defect in 8 (8%). Wolff-Parkinson-White syndrome occurred in 22 patients (21%). The QRS axis tended to be different in LV noncompaction with a mean +/- SD axis of 12 degrees +/- 74 degrees vs 36 degrees +/- 66 degrees overall (P=.08). Otherwise, there were no differences in clinical or surgical data between the groups with normal and abnormal LV myocardium. CONCLUSIONS: In patients with Ebstein anomaly, left heart abnormalities involving the myocardium or valves were observed in 39% of patients. Ebstein anomaly should not be regarded as a disease confined to the right side of the heart.     

Prospective comparison of echocardiography versus cardiac magnetic resonance imaging in patients with Ebstein's anomaly

Ebstein's anomaly (EA) is primarily diagnosed by echocardiography. The purpose of this study was to compare echocardiography and magnetic resonance imaging (MRI) in EA. Data from cardiac MRI and echocardiography were prospectively collected from 16 patients with EA. Imaging data also were compared with intraoperative findings. Information provided by MRI and echocardiography were comparable for left ventricular size and function, tricuspid valve repairability, qualitative assessment of right-sided cavities, and visibility of septal and anterior tricuspid valve leaflets. The posterior tricuspid valve leaflet and tricuspid valve fenestrations were better visualized with MRI; associated heart defects were equally recognized, apart from small shunts that tended to be more readily diagnosed with echocardiography. Quantification of right-cavity size and right ventricular ejection fraction was possible only with cardiac MRI. The degree of tricuspid valve regurgitation was underestimated by echocardiography (2 patients) and by MRI (4 patients) when compared with intraoperative assessment. When evaluating EA, echocardiography and MRI provide complementary data. For visualization of the posterior tricuspid valve leaflet and quantitative assessment of right ventricular size and function, MRI is preferable. For appropriate risk stratification in EA, both MRI and echocardiography should be performed before cardiac surgery.     

室间隔缺损封堵术并发三尖瓣反流的原因分析

目的探讨经导管膜部室间隔缺损（membranous ventricular septal defect，mVSD）封堵术并发三尖瓣反流的可能原因及注意问题。方法对542例成功行mVSD封堵术患者术前、术后即刻行超声心动图检测，术后5d，1、3、6、12个月随访。结果术后14例出现三尖瓣反流，其中11例术后即刻及3d出现少量反流。22例术前超声心动图显示三尖瓣前叶腱索附着异常中3例最短术后4d、最长1个月发现三尖瓣前叶腱索断裂，出现中量反流。三尖瓣隔叶、腱索和（或）缺损周缘增生的组织粘连融合，形成缺损口右室侧多孔及不同形态，其中7例呈较大的囊袋样不规则形或瘤形，缺损口周缘部分由三尖瓣隔叶和（或）腱索构成。结论mVSD封堵并发三尖瓣反流的原因可能为导管损伤腱索、输送杆损伤腱索和封堵器磨损腱索。术前选择适应证时，除常规观察项目外，还应注意观察三尖瓣前叶腱索位置、缺损周缘构成的组织结构及缺损口右室侧形态，以减少并发症。     

多排螺旋CT在Ebstein畸形诊断中的临床应用

目的评价多排螺旋CT（MSCT）在Ebstein畸形及合并心内外畸形诊断中的临床价。方法回顾性分析本院经手术确诊的40例Ebstein畸形病例,在采用回顾性心电门控技术下行心脏增强扫描检查,并行多种CT后处理图像重组技术分析三尖瓣的形态表现、心脏各房室的形态改变以及伴发的心内外结构畸形等,同时将MSCT及心脏超声（UCG）检查诊断结果与手术结果进行对照。结果 40例Ebstein畸形中,除7例患者无合并畸形外,其余33例均合并一种或多种其他心内外畸形。40例患者中手术确诊的包括三尖瓣下移畸形在内心内畸形共87处,MSCT及UCG确诊分别为82、85处,诊断符合率分别为94.3%、97.7%,对于心内畸形部分UCG诊断符合率略高于MSCT。经手术确诊的心外大血管畸形共25处,MSCT除1例细小的PDA未能正确诊断外,其余畸形均能清楚显示,诊断符合率达96.0%,而UCG对于大血管畸形的诊断敏感性明显降低,仅准确诊断出9处,检出率为36.0%。MSCT显示Ebstein畸形患者的三尖瓣的隔瓣、后瓣附着点均不同程度向心尖部下移且二尖瓣与三尖瓣隔瓣间距超过10 mm者共32例,单纯隔瓣下移者6例;三尖瓣前瓣呈＂帆状＂共34例。所有患者均有不同程度的右侧房室环和右心房的扩大,房化的右心室肌壁变薄,心腔扩大,功能性右心室小。心脏合并畸形包括房间隔缺损、室间隔缺损、动脉导管未闭及其他复杂畸形等。结论多排螺旋CT可以清晰地显示三尖瓣形态及附着点异常,虽然对于合并的心内畸形的诊断准确率不及UCG,但两者无明显差异;而MSCT对于心外大血管畸形诊断相对于UCG具有极高的诊断准确性,是诊断Ebstein畸形且疑合并有心外大血管畸形者的有效影像学补充检查,能为外科手术治疗提供丰富的影像解剖信息。