Ebstein's Anomaly—An Overview

Aim: To present the various echocardiographic spectrum of Ebstein’s mal-formation in adolescence and adults. Introduction: Ebstein’s anomaly has different anatomic and hemodynamic variables with clinical manifestations of cyanosis, right-sided heart failure and arrhythmias. The leaflet tethering and dysplasia, together with dilatation of the tricuspid valve ring, constitute the anatomic cause of tricuspid regurgitation observed in this condition. Case Reports: The spectrum of leaflet tethering from mild to extreme with varying degrees of tricuspid regurgitation were documented by echocardiography in a 16-year-old cyanotic male with Ebstein’s anomaly associated with an atrial septal defect and mild low tricuspid regurgitation (TR), 22-year-old acyanotic male with right-sided heart failure due to severe tricuspid regurgitation and an intact atrial septum, 55-year-old asymptomatic female with moderate high tricuspid regurgitation. The extreme form was described as an “atretic” mem-brane in a 28-year-old cyanotic male and as a “blanket” of leaflet tissue in a 30-year-old cyanotic male. Conclusion: Ebstein’s anomaly has to be sus-pected clinically in presence of cyanosis with a WPW (Wolf-Parkinson-White) or atrioventricular (AV) block pattern of ECG and its management is complex and must be individualized. RV (right ventricular) exclusion procedures are preferred in advanced cases.

Corrected Transposition of the Great Arteries with Ebstein’s Anomaly, Dysplasia of the Mitral Leaflets and Persistence of Left Superior Vena Cava in an Adult

Introduction: Congenitally corrected transposition of the great arteries (cc-TGA) is a congenital heart disease rarely described among adult population, especially if it is associated with other abnormalities such as Ebstein’s anomaly and abnormal origin of coronary arteries. Case presentation: Twenty-two-year-old woman admitted to intensive care unit with acute decompensated heart failure. The transthoracic echocardiography demonstrated atrioventricular and ventriculoarterial discordance. The left-sided atrioventricular valve, in this case, the tricuspid valve, showed apical displacement of the septal valve suggesting Ebstein’s anomaly. The computed tomography corroborated these findings and additionally showed a left superior vena cava and an abnormal origin of the coronary arteries. The patient is in NYHA functional class II, receiving treatment with diuretic, digitalis, beta-blockers and angiotensin-converting enzyme (ACE) inhibitor. Conclusion: This is a very interesting case of an adult in the third decade of life with cc-TGA associated with abnormalities not described in the literature that had reached adulthood. Noninvasive images play an important role in its diagnosis.

1(1/2)心室修补手术治疗Ebstein畸形

目的 总结 1 心室修补手术治疗Ebstein畸形的经验。方法 1997年 1月至 2 0 0 2年 8月对 9例中、重度Ebstein畸形病人 ,行 1 心室修补手术 ,其中合并房间隔缺损 (右向左分流 ) 5例 ,三尖瓣为轻至重度反流 ;所有病人术前均有症状 ,其中心功能Ⅱ级 5例 ,心功能Ⅲ级 4例。除行三尖瓣成形及闭合房间隔缺损外 ,均行上腔静脉右肺动脉双向分流术。结果 本组无手术死亡。 1例 5岁重度三尖瓣下移患儿术后出现右侧胸腔积液 ,经穿刺治疗半月后胸腔积液消失。随访 3个月至 5年 ,心功能恢复到Ⅰ级 ,复查心脏超声提示三尖瓣轻度反流。结论 1 心室修补手术治疗可能是治疗中、重度Ebstein畸形较好的方法。其远期疗效有待于进一步观察。

射频导管消融治疗伴发于Ebstein畸形的房室折返性心动过速

目的：为治疗伴发于Ｅｂｓｔｅｉｎ畸形的阵发性室上性心动过速，我们对４例患者进行了射频导管消融。方法：４例伴发于Ｅｂｓｔｅｉｎ畸形的阵发性室上性心动过速患者，年龄２５～３４岁，药物治疗效果不佳或不能耐受，遂进行电生理检查和射频导管消融。结果：４例患者经心内电生理检查均证实为右侧旁路房室折返性心动过速。理想的消融靶点位于房室环、心内ＡＶ波大致相等处，其ＡＶ波间距较长。全部患者的射频导管消融均获成功。结论：射频导管消融通过阻断右侧旁路而治愈伴发于Ｅｂｓｔｅｉｎ畸形的室上性心动过速。正确认识其解剖和电生理特点，是消融成功的关键。附图射频导管消融治疗Ｅｂｓｔｅｉｎ畸形的旁路心内电生理图。图ａ：射频导管消融靶点图，消融导管远端（ＬＡＢｄ）所描记到的ＡＶ波振幅基本相等并有距离，在此进行消融。图ｂ：射频导管消融成功后ＡＶ间距明显增大。ＨＢＥ：希氏束电图ＲＶＡ１～２：右心室心尖部ＣＳ１～１０：冠状静脉窦ＣＳ９～１０：冠状静脉窦近端ＣＳ１～２：冠状静脉窦远端ＬＡＢｐ：消融电极近端３讨论Ｅｂｓｔｅｉｎ畸形是最常伴有预激综合征的先天性心脏病（可达１６．１％），且有１２．９％的患者有阵发性室上性心动过速［１］；与心动过速有关的晕厥及?

Ebstein畸形合并右侧旁道的射频消融及靶点图特征探讨

报道６例Ｅｂｓｔｅｉｎ畸形合并右侧房室旁道患者（１例为外科手术切断未成功），其中顺向型房室折返性心动过速（ＡＶＲＴ）５例、逆向型１例。６例中有心房颤动和电击复律史者３例。经导管射频消融均成功地阻断了旁道。与心内结构正常的１５例右后侧壁显性旁道消融结果比较，平均消融时程（１３９±７４ｍｉｎｖｓ１１３±４６ｍｉｎ）、Ｘ线曝光时间（２８±１７ｍｉｎｖｓ２３±１２ｍｉｎ）均无显著性差异（Ｐ＞０．０５）。平均随访８±５月，１例术后６个月复发，经再次消融成功。其余病例未服用任何抗心律失常药物无心动过速复发。无并发症发生。提出掌握Ｅｂｓｔｅｉｎ畸形旁道消融的有效靶点图特征及其消融的特殊性。

14例成人Ebstein心脏畸形的外科治疗

目的 分析14例成人Ebstein心脏畸形的外科治疗。方法 常规体外循环下Danielson方法行房化心室折叠加三尖瓣环环缩9例;三尖瓣置换5例;房间隔缺损修补12例;右房减容成形术14例。结果 牛心包瓣置换术后低心排综合征死亡1例;长期存活13例。随访9～109月,无晚期死亡,无严重心律失常及血栓栓塞和心内膜炎。成形术组心功能I级5例,Ⅱ级4例,5例术后有轻-中度三尖瓣返流;瓣膜置换术组无瓣周漏,心功能I级3例,Ⅱ级1例。结论成形术适用于三尖瓣前瓣叶可形成单叶瓣的病人,但术后返流率较高;瓣膜置换术后三尖瓣返流即消失;右房减容成形术可消除巨大右房引起的房扑或房颤及减少血栓形式的机会。

利用自体心包片修复三尖瓣治疗Ebstein畸形

目的介绍一种利用自体心包片修复三尖瓣治疗Ebstein畸形的新方法和临床经验。方法 2008年1月至2012年6月手术治疗8例Ebstein畸形患者,根据Carpentier病理分型,B型1例,C型6例,D型1例。术中在Carpentier术式的基础上,应用戊二醛处理过的自体心包片重建前瓣、后瓣,增加瓣叶对合面积。结果围术期无死亡。三尖瓣成形术后超声心动图示三尖瓣反流明显减轻。随访3～48个月,超声心动图表现明显改善,所有患者心功能均恢复至Ⅰ～Ⅱ级。结论利用自体心包片修复三尖瓣治疗Ebstein畸形拓宽了瓣膜成形的适应证,能够更好地恢复三尖瓣的解剖形态及功能。

48例Ebstein畸形患者手术的麻醉处理研究

目的:探讨埃博斯坦(Ebstein)畸形患者手术治疗的麻醉处理方法。方法:对1999-01至2008-05在我院手术治疗的Ebstein畸形患者共48例进行了总结。全部病例采用静吸复合全身麻醉。术中除常规监测外,经食管超声心动图是围术期必要的监测手段。麻醉维持以中、大剂量芬太尼为主,辅以静脉泵入异丙酚和吸入低浓度的异氟醚或七氟醚。术中控制适当容量,使用血管活性药物,调整内环境至稳定,避免肺血管阻力增加,避免心律失常。结果:全组患者35例治愈,9例好转,4例死亡。出院患者症状均有明显改善,围术期血流动力学基本平稳。结论:术前正确评估患者病情,选择正确麻醉方式,术中维护心血管功能稳定和维持低状态肺血管阻力,避免心律失常是患者安全渡过围术期的关键。

Strategy and technique for surgical treatment of Ebstein’s anomaly

Background:Ebstein’s anomaly(EA)is a rare and complex congenital heart anomaly,and the effect of surgical treatment is not ideal.This study aims to introduce our experience in management strategies,surgical techniques,and operative indications for patients with Ebstein’s anomaly.Methods:A retrospective study of 258 operations was performed in 253 patients by the same cardiac surgeon in The First Hospital of Tsinghua University between March 2004 and January 2020.32 patients had previously received cardiac surgery in other hospitals.The clinical data including diagnosis,operative indications,techniques,pathological changes,and survival rates were collected and analyzed.Results:Anatomical correction was performed in 203(78.7%)operations,1½ventricle repair in 38(14.7%)operations,tricuspid valve repair only in four operations(1.6%),tricuspid valve replacement in ten(3.9%),total cavopulmonary connection(TCPC)in two(0.8%),and Glenn operation in one operation(0.4%).Reoperation was performed in five patients(2.0%)during hospitalization.Among them,tricuspid valve replacement was performed in one patient,1½ventricle repair in two patients,and tricuspid valve annulus reinforcement in two patients.Five patients died with an early mortality rate of 2.0%.Complete atrioventricular conduction block was complicated in one patient(0.4%).A total of 244 patients was followed up(four in the 253 patients lost)with a duration of 3.0-168.0(87.6±38.4)months.Cardiac function of 244 patients improved significantly with mean New York Heart Association(NYHA)functional class recovery from 3.5 to 1.1.The mean grade of tricuspid valve regurgitation improved from 3.6 to 1.5.Three late deaths(1.2%)occurred.The survival rates at five and ten years after surgery were 98.6%and 98.2%,respectively.Reoperation was performed in five patients(2.0%)during the follow-up period.Conclusion:Based on our management strategies and operative principles and techniques,anatomical correction of EA is capable of achieving excellent long-term results,and low rates of TCPC,1½ventricle repair and valvular replacement.

Rheumatic “Taussig-Bing Heart”: A Case Report

Aim: To report a case of acyanotic Taussig-Bing heart, anatomically consistent with L-transposition and rheumatic Left AV valve regurgitation associated with complete AV block in an adolescent male. Introduction: Taussig-Bing heart is one of the conotruncal malformation, characterized by double-outlet right ventricle (DORV) and a subpulmonary VSD. Embryologically, abnormal cardiac looping with malalignment of conotruncal septum result its complexity and great artery relationships. Case Report: A 14-year old acyanotic boy presented with severe left-sided AV valve regurgitation and bradycardia. ECG revealed left sided morphologic right ventricular hypertrophy (RVH) as evidenced by a loss of septal Q waves in left precordial leads suggesting ventricular inversion. X-ray chest revealed a straight upper right cardiac border due to loss of normal relationship of great vessels and cardiomegaly due to both left atrial and morphologic right ventricular enlargements suggesting a left-sided regurgitant lesion. Echocardiography revealed the ventricular inversion, primary origin of both L-transposed great arteries from the left-sided morphologic right ventricle suggesting a “double-outlet morphologic right ventricle” with “double discordance” and a subpulmonary VSD of Taussig-Bing type. The left-sided morphologic tricuspid valve is severely regurgitant due to rheumatic process resulting in heart failure which was improved with anti-failure measures and penicillin prophylaxis. Conclusion: The presence of left-sided regurgitation associated with bradycardia is a suspicion of “double discordance” and “double switch” procedure remains the mainstay of its surgical correction. Left AV valve replacement with intraventricular repair is preferred in this child since the malformation is consistent with Taussing-Bing type of DORV with L-transposition.

Clinical analysis of arrhythmia in 297 Ebstein＇s anomaly patients

Ebstein＇s anomaly is a kind of congenital heart disease ＇with part or all leaflets of tricuspid valve notnormally attaching to the tricuspid annulus in the normal position, but spirally shifting downward and abnormally attaching to the right ventricular wall. Ebstein＇s anomaly patients often suffer from arrhythmias, featured by paroxysmal fibrillation and supraventricular tachycardia, atrial atrial flutter, etc.

Anesthetic considerations of an emergency decompressive craniotomy complicated with Ebstein＇s anomaly and atrial septal defect

Despite considerable published papers regarding Ebstein＇s anomaly （EA） patients receiving open-heart tricuspid valve replacement, non-cardiac emergency surgeries were rarely reported. We report a case of emergency decormpressive craniotormy in a patient with EA. Anesthesiologists should pay special attention to the complications and anesthetic management during the non-cardiac surgeries performed in EA patients.hile papers regarding Ebstein＇s anomaly （EA）patients receiving open-heart tricuspid valve replacement are numerous, the reports of non-cardiac emergency surgeries have not been to the same degree.We report a case of emergency decompressive craniotomy in a patient with EA. This case report describes the symptoms and anesthetic management during the surgery.

Intraoperative Transesophageal Echocardiography in the Operation of Ebstein＇s Anomaly： A Retrospective Study

Background： Ebstein＇s anomaly （EA） has various spectrums in clinical and anatomic features. This study aimed to report the experience of two-dimensional intraoperative transesophageal echocardiography （2D-ITEE） during the EA surgery and to analyze the characteristics of the tricuspid valve （TV） by comparing the data from 2D-ITEE with the results from the surgery. Methods： 2D-ITEE data of 164 patients with EA who were operated in the First Hospital of Tsinghua University between July 2004 and April 2014 were retrospectively analyzed in this study. 2D-ITEE was applied in all patients. Downward displacement distances were measured, and the numbers of downward displacement or absent leaflets were compared with that of the surgery and with that of the two-dimensional-transthoracic echocardiogram （2D-TTE）. Data comparison was peribrmed using the Chi-square test. Results： The anterior leaflet partial or total downward displacement was 37.76 ±17.50 mm in 54 cases, absent in one patient; septal leaflet downward displacement was 29.07 ± 12.34 mm in 134 cases, absent in 17 cases; and posterior leaflet downward displacement was 43.18 ± 19.16 mm in 115 cases, absent in 34 cases. Statistically, there was no significant difference between the results from 2D-ITEE and that of 2D-TTE. The consistency rates of 2D-ITEE with operation for septal and posterior leaflets were 93.2% and 96.1%, respectively, while the rate for anterior was only 40.1%o, which was significantly different. Color Doppler flow image showed severe regurgitation in 150 cases and moderate in 14 cases. After surgical correction, moderate regurgitation of TV was found in 58 cases with 2D-ITEE, repair was performed again until the effect was satisfied. No complication occurred relating to the use of 2D-ITEE. Conclusions： 2D-ITEE could help diagnose anterior leaflet, evaluate the effect of TV repair, increase operational success rate, and reduce complication.

Managements of Ebstein's anomaly with Hetzer technique

Background Ebstein’s anomaly is a rare and complex congenital malformation of the tricuspid valve and the right ventricle with variable pathological anatomy. The surgical treatment of this anomaly has long been controversial. We reviewed cases corrected with Hetzer technique to analyze the uni/biventricular strategy,timing of surgery and results. Methods Between October 2009 and December 2013, 21 patients with Ebstein’s anomaly were admitted to Guangdong General Hospital and underwent surgical correction. There were 11 males and 10 females. Type A, 3 cases; Type B, 16 cases; Type C, 1 case, Type D, 1 case. The 2 patients of Type C and Type D undergoing Starnes procedure were excluded, all other 19 patients who underwent surgical repair with Hetzer technique in this time frame were included in the analysis. In this “Hetzer group”, the age at the time of the repair ranged from 13 months to 42 years（median： 8 years） and weight ranged from 11.3 to 55kg（median： 19.5 kg）. Combined anomalies include： 2 of 3 Type A combined with secondum atrial septal defect（ASD）, 14 of 16 cases of Type B combined with secondum ASD, both of 2 cases of Type C and D combined with secondum ASD. 2 of 21（9.5%） of these patients have Wolff-Parkinson-White（WPW）syndrome. Results There was no early, or late mortality. One 13-month infant boy, after valvuloplasty, ASD closure and bidirectional Glenn（BD-Glenn） procedure, presented with low cardiac output, depression of the left ventricle, severe tricuspid regurgitation（TR） with massively dilated right ventricle, the patient underwent emergent redo procedure, however, even after re-valvuloplasty, Transesophageal echocardiography（TEE）showed moderate to severe TR, we decided to reopen and enlarge the ASD, sewed a 5 mm fenestrated pericardial patch to close the tricuspid orifice partially, after 7 days, the patient weaned successfully from the ventilator. Another two 1-year old infants after valvuloplasty, BD Glenn and partial ASD closure showed no improvement of TR, but postoperative oxygen saturation increased, symptoms diminished and exercise tolerance improved significantly. Other 16 patients, whose age ranged from 5-42 years, had satisfactory results.Echocardiography showed： 14 of the 16 patients’ TR improved from severe to mild, 2 of them improved from moderate to trace or mild. Except the redo case, the pulse oxygen saturation at rest goes up to over 95%without cyanosis; 15 of 18 patients shows 85%-90% O2 saturation after exercise in room air. Conclusions Hetzer technique is a duplicable surgical approach to Ebstein’s anomaly with satisfactory results for older patients, especially for adults. However, we notice a trend that this technique may be not suitable for small infants, and further evaluation will be needed.

Ebstein畸形解剖纠治术

目的 总结 2 0例Ebstein畸形解剖纠治术的近期效果。 方法 1997年 12月至 2 0 0 0年 9月 ,对 2 0例Ebstein畸形患者行解剖纠治术。其中男 8例 ,女 12例 ,年龄 3～ 32岁 ,平均 11岁。Carpentier分型 :A型 2例 ,B型 4例 ,C型 14例。手术方法 :梯形或三角形切除绝大部分房化右心室 ,沿瓣环切下下移的后瓣及其相连的乳头肌 ,必要时切下部分或全部隔叶 ,折叠缝合瓣环 ,使其恢复正常大小。将瓣叶根部移植于正常瓣环水平 ,并对瓣叶组织进行修复 ,重新移植或固定乳头肌 ,处理合并病变。合并手术包括 :Rastalli手术 1例、房间隔缺损修补术 5例、缝闭卵圆孔 4例、室间隔缺损修补术 2例、动脉导管未闭直视缝闭术 1例。 结果 患者术后均恢复顺利 ,心功能均恢复Ⅰ级。术前 12例重度三尖瓣关闭不全患者 ,术后 8例三尖瓣返流消失 ,4例三尖瓣轻度返流 ;8例中度关闭不全患者 ,术后 7例三尖瓣返流消失 ,1例轻度三尖瓣返流 ;三尖瓣叶均在正常水平 ,房化心室基本消失。术后随访时间 1～ 17个月 ,平均 6个月。患者活动量恢复正常。超声心动图检查 :17例三尖瓣返流消失 ,2例中度三尖瓣返流 ,1例轻度三尖瓣返流。 结论 Ebstein畸形解剖纠治术的近期效果良好 ,可能使绝大多数患者免于三尖瓣替换术 。

改良Carpentier法治疗三尖瓣下移畸形32例病例分析

目的：总结32例Ebstein畸形经改良Carpentier法手术治疗的疗效与经验.方法：回顾性分析自2003年6月至2013年8月采取改良Carpentier法手术治疗的32例Ebstein畸形（儿童23例,成人9例）的临床资料,其中男性15例,女性17例；年龄1～60岁,体质量8.5～ 68 kg.手术方式为改良Carp.ntier法,先将所有有用瓣叶切下,纵向折叠房化右心室,将瓣叶顺时针转位缝合至新瓣环根部,同期矫治合并畸形.其中6例因右心室发育差,三尖瓣瓣环小,加行双向格林术.结果：1例患儿因术后早期心律失常并发持续性低氧血症而死亡,余均存活,顺利出院.其中1例术后出现Ⅲ.房室传导阻滞,3个月后回转窦性心律.存活31例均获随访,随访时间1～72个月,其中术后复查超声心动三尖瓣反流情况轻度21例,轻中度5例,中度5例,重度1例.结论：运用改良Carpentier法手术治疗Ebstein畸形,较好保留了右心室形态,三尖瓣整形好,术后心功能恢复良好,效果满意.

三尖瓣下移畸形的外科治疗策略

目的：分析39例三尖瓣下移畸形（Ebslein畸形）的外科治疗,探讨不同分型的三尖瓣下移畸形的外科治疗策略.方法：回顾性分析2010年7月至2013年2月,我院小儿心脏外科收治的39例Ebstein畸形患者,其中男性23例,女性16例,年龄0.8～35岁.5例（12％）术前经ECG诊断合并预激综合征.手术方式包括：双心室矫治26例,一个半心室矫治10例,双向Glenn术1例,肺动脉环缩术2例.结果：围手术期死亡1例,病死率2.5％.其余患者无严重并发症,均顺利康复出院.复查心脏超声心动图等,提示三尖瓣关闭不全程度明显减轻,心功能改善明显.结论：根据三尖瓣瓣叶发育情况、瓣环大小及房化右心室与功能右心室的比例,选择适当的术式,矫治三尖瓣下移畸形能够获得较为满意的临床治疗效果.

圆锥法矫治三尖瓣下移畸形的近中期效果

目的:通过探讨圆锥法矫治三尖瓣下移畸形,总结其早、中期临床效果。方法:2012年1月至2016年6月我院应用圆锥法矫治三尖瓣下移畸形16例,其中男7例、女9例,平均(28.21±11.88)岁,Carpentier B型12例、C型4例,心功能Ⅱ级2例、Ⅲ级9例,Ⅳ级5例,三尖瓣反流2级2例、3级10例、4级4例,通过分析其临床资料,观察早、中期效果。结果:住院及随访过程中无死亡,其中1例术后第1天出现低心排血综合征、1例术后第3天出现室早二联律,经治疗后好转,均恢复顺利,出院时三尖瓣反流由术前的(3.13±0.62)级下降到(1.75±0.45)级(P<0.001),心功能由术前的(3.19±0.66)级提高至(2.00±0.52)级(P<0.001),平均随访(24.36±10.71)个月,较出院时三尖瓣反流进一步下降至(1.31±0.48)级(P=0.016),心功能提高至(1.50±0.52)级(P=0.031)。结论:圆锥法矫治三尖瓣下移畸形能够有效改善三尖瓣功能、右室几何形态及心功能状态,提高患者生活质量,取得满意的早、中期效果,但仍需扩大病例数及严格掌握适应证,远期效果尚需进一步观察。

三尖瓣下移畸形认识与治疗研究进展

三尖瓣下移畸形(EA)又称Ebstein畸形,是一种罕见且复杂的先天性心脏病。EA由右心室心肌病和三尖瓣病变组成,从而导致患儿右心室功能障碍和心律失常的发病率升高。目前先天性心脏病方面的专家对EA的理解和认知有所提高。但疾病异质性和复杂的治疗方案仍然是巨大挑战。本文对EA的认识与治疗最新进展进行综述。