51例Ebstein畸形的外科治疗

目的总结Ebstein畸形的外科治疗经验。方法回顾性分析2002年1月至2011年8月河南省胸科医院51例Ebstein畸形患者经外科手术治疗的临床资料,其中男22例,女29例;平均年龄15.8(0.8～48.0)岁;平均体重35.3(7.0～68.0)kg。Carpentier分型A型12例、B型34例、C型5例。超声心动图(UCG)提示:三尖瓣轻度反流7例,中度反流8例,重度反流36例。手术中应用褥式缝合上提隔瓣和后瓣,平行折叠房化右心室加三尖瓣环缩术21例,Carpentier法8例,二瓣化法11例,纵向折叠房化心室加三尖瓣成形术4例,三尖瓣机械瓣置换术3例,生物瓣置换术2例;另有2例因右心室发育不良仅做双向格林手术。17例患者于三尖瓣成形术后加做双向格林手术。结果围术期死亡2例,均死于低心排血量。术后随访49例,平均随访时间32(2～102)个月。随访期间心功能分级(NYHA)Ⅰ级30例、Ⅱ级19例;三尖瓣中度反流8例,轻度反流6例,无反流35例。3例三尖瓣机械瓣置换术患者的机械瓣、2例生物瓣置换术的生物瓣功能均正常。患者恢复正常的生活或工作,无需再次手术患者。结论对Ebstein畸形要根据不同的病情采取相应的手术治疗方法,可以获得良好的手术效果。     

三尖瓣置换术治疗Ebstein氏畸形的术后监护

Ebstein氏畸形是一种罕见的三尖瓣先天性畸形，约占先天性心脏病的0．5％［1］，预后不佳，手术病死率高达40％～50％[1]。该畸形的病理解剖有三个特征：①三尖瓣隔叶和后叶下移至右心室内；②有一个房化的右心室部分，位于三尖瓣环与隔叶、后叶附着处之间；③右心室畸形（三尖瓣以下的功能性右心室腔显著减小），室壁薄，收缩功能减弱。本病因其畸形特点不同，手术方法各异如三尖瓣成形术或三尖瓣置换术，目的是矫治三尖瓣功能障碍和折叠房化右心室部分。我科于1992～1997年对5例Ebstein氏畸形的患者施行三尖瓣置换术，均治愈，无1例发生…     

44例Ebstein畸形的外科治疗

目的：观察各种Ｅｂｓｔｅｉｎ畸形矫治手术的疗效以及手术前后左室功能的变化。方法：手术治疗４４例Ｅｂｓｔｅｉｎ病人,其中Ｈａｒｄｙ手术２８例,改良Ｄａｎｉｅｌｓｏｎ手术７例,Ｃａｒｐｅｎｔｉｅｒ手术２例,三尖瓣替换手术７例。对５例进行了压力－容积环测量,同时观察了左室室壁运动。     

改良术式矫治Ebstein畸形

目的探讨改良Carpentier方法矫治Ebstein畸形的手术疗效。方法Carpentier法手术修复的基础上，采用自制涤纶环代替人工环；后瓣叶边缘对合缝在隔瓣叶或前瓣，以加大面积；必要时Devega成形环缩三尖瓣环。同期矫治合并畸形。结果手术死亡1例，死亡率4％。随访22例，随访期2～65个月，无远期死亡。结论改良Carpentier方法矫治Ebstein畸形，方法新颖，可以不用成形环，费用降低，临床实用，疗效满意。     

Ebstein综合征的外科治疗

目的总结Ebstein综合征的外科治疗经验。方法回顾性分析2006年2月至2010年6月济宁医学院附属医院23例Ebstein综合征患者经外科手术治疗的临床资料,其中男5例,女18例;年龄3～40(15.0±7.6)个月。行单纯三尖瓣环成形术1例,Danielson式折叠术5例,Carpentier式折叠术16例(其中5例患者三尖瓣和功能右心室发育差,加行双向Glenn手术),心脏复跳后三尖瓣关闭不全,有大量反流,再次阻断主动脉行三尖瓣置换术1例。结果无手术死亡,术后出现频发性室性早搏4例,室性心动过速2例,Ⅲ°房室传导阻滞1例,均于术后1周内恢复。术后1～2周复查心脏超声心动图提示:三尖瓣有微量至轻度反流19例,中度反流3例。随访19例,随访时间0.5～4.0(3.3±1.6)年,随访期间因右心功能衰竭死亡1例,再次手术行三尖瓣置换术1例;心功能分级(NYHA)Ⅰ级12例,Ⅱ级4例,Ⅲ级2例。失访4例。结论三尖瓣修复术是治疗Ebstein综合征有效的手术方式,可有效地缓解患者的临床症状,改善血流动力学,促进右心室功能的恢复,提高患者的生活质量。     

Ebstein畸形的外科治疗

目的总结15例先天性三尖瓣下移（Ebstein）畸形的手术治疗经验，以提高手术疗效。方法对2002年4月至2007年8月收治的15例Ebstein畸形患者采用三尖瓣成形和房化右心室折叠术，其中8例隔瓣后瓣发育不全或缺如的患者采用自体心包矫正。结果全组无死亡。术后1例发生低心排血量综合征，经使用正性肌力药物（洋地黄）和利尿剂控制心力衰竭，术后第3d好转；其余患者恢复良好，心功能有明显改善。随访13例，2例失访，随访时间1～42个月，其中11例患者心功能恢复至Ⅰ级，2例心功能恢复至Ⅱ级；紫绀和心脏杂音消失；复查超声心动图提示：12例三尖瓣水平反流消失，1例仍有轻度至中度反流。结论对右心室病理改变的正确认识，完善的三尖瓣功能修复和房化右心室折叠是手术成功的关键；自体心包三尖瓣隔瓣后瓣再造，保持了右心室几何形态和功能，减少了并发症的发生，能提高手术成功率。     

Ebstein 畸形解剖纠治术

目的 总结20例Ebstein畸形解剖纠治术的近期效果。方法 1997年12月至2000年9月,对20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,远期结果有待进一步观察。     

Ebstein畸形的外科治疗进展

Ebstein畸形是一种较少见的先天性心脏畸形,可明显影响患者的心功能,随着病情的进展最终诱发右心衰竭甚至全心衰竭,而导致患者死亡。近年来,Ebstein畸形手术主要在针对病变瓣膜的处理、右心室功能完善及相关并发症的预处理上已取得较大的进展。但由于其病理解剖及临床表现的复杂多样性,目前尚未有一个"金标准"来治疗所有病理解剖类型的畸形患者,也一直未有一种手术策略来解决临床所遇到的实际问题,所以术后疗效不尽如人意。我们通过文献复习,对Ebstein畸形的外科治疗进展及其相关并发症防治等进展进行综述。     

Ebstein畸形手术前后的心电图表现

Ｅｂｓｔｅｉｎ畸形手术前后的心电图表现柳克晔陈英淳孔晴宇Ｅｂｓｔｅｉｎ畸形是三尖瓣下移出现房化右心室和右心室发育不良的先天性心脏病，发病率占先天性心脏病的０．５％～１％，常合并房间隔缺损，有效的治疗方法是外科手术。１９８４年９月～１９９６年１２月我院...     

Ebstein畸形的麻醉处理

反流 Ebstein畸形即三尖瓣下移畸形,并非是一种罕见的先天性心脏畸形,因1866年由Ebstein首先报道而命名。Ebstein畸形在先心病中的发病率大约为0．5％～1％。尽管其病理解剖多样,但此类病人共同表现为三尖瓣位置下移,以隔瓣和后瓣基部的下移为主,瓣叶常发育不全。Ebstein畸形病人病变轻微,可无临床表现,如果病变严重,病人会很快死亡。由于该畸形的特殊性,在此有必要对Ebstein畸形的麻醉处理进行讨论。     

埃布斯坦综合征的外科治疗

目的 为了改善埃布斯坦综合征（Ｅｂｓｔｅｉｎ ａｎｏｍａｌｙ）的手术疗效,总结３６例埃布斯坦综合征患者的手术治疗经验。方法 ３６例手术中,三尖瓣置换术６例,Ｄａｎｉｅｌｓｏｎ手术２８例,Ｃａｒｐｅｎｔｉｅｒ手术２全;其中５例同时行右心房室旁路切断术。结果 术后早期右侧心力衰竭死亡４例,其中心瓣膜置换术１例,成形术２例,１例成形术并右心房室旁路切断术死于右心室壁血肿。２５例患者随访３个月～１１年,１     

45例成人Ebstein畸形的外科治疗

目的 回顾分析45例成人Ebstein畸形外科手术方法及疗效.方法 45例Ebstein畸形患者,男14例,女31例;年龄18 ～61岁,平均39岁.行改良 Carpentier 术23例,改良Danielson术15例,三尖瓣置换术7例(二次手术4例).结果 三尖瓣成形术后超声心动图示三尖瓣反流明显减轻.早期死亡2例(4.4％),死因为术后低心排血量综合征和右心功能衰竭.随访2～ 46个月,生存者中41例(95％)恢复心功能Ⅰ～Ⅱ级.结论 根据Ebstein畸形的病变程度选择合适的手术方法是手术成功的关键.手术方式要个体化,尽量行成形术.     

Thrombosed tricuspid valves presenting some 20 years after the surgical correction of Ebstein's anomaly: Report of two cases

We present herein the cases of two patients who were diagnosed as having thrombosed tricuspid valves by electrocardiography after undergoing valve replacement with Starr-Edwards caged ball valves for Ebstein's anomaly 19 and 27 years earlier. In both patients, the valves were found to be attached to the right ventricular septum and covered with massive thrombus, the balls being completely jammed in a half-open position in the cages. Re-replacement of the valves was successfully performed with Hancock II porcine heterograft prostheses and both patients are now classified as being in Functional Class I or II.     

Enlarged coronary sinus thrombosis after repair of Ebstein's anomaly

A 58-year-old woman was referred to our hospital with progressively increasing breathlessness. She reported a history of bioprosthetic valve implantation for tricuspid valve replacement and direct closure of an atrial septal defect for Ebstein's anomaly, 31 years before presentation. Transthoracic echocardiography revealed prosthetic valve failure, an enlarged coronary sinus, and severe mitral regurgitation. Computed tomography revealed a giant coronary sinus with thrombosis and persistent left superior vena cava. She underwent successful mitral and tricuspid valve replacement; however, severe hemodynamic deterioration necessitated mechanical ventilatory support with extracorporeal membrane oxygenation.     

风湿性三尖瓣重度关闭不全外科治疗远期疗效分析

目的总结风湿性三尖瓣重度关闭不全外科治疗远期疗效,探讨理想的三尖瓣重度关闭不全外科治疗方法。方法对78例风湿性三尖瓣重度关闭不全患者采用不同的三尖瓣处理方法,随访6个月~4年,根据超声心动图比较中远期疗效。结果成形组复发三尖瓣重度关闭不全56例,复发率77.5%,三尖瓣置换组效果满意。结论风湿性三尖瓣重度关闭不全根据肺动脉压力及三尖瓣腱索病变程度选择适宜的三尖瓣手术是提高术后生活质量及生存时间的关键。     

Surgical management of Ebstein anomaly: impact of the adult congenital heart disease anatomical and physiological classifications

OBJECTIVESOur goal was to evaluate the impact of the adult congenital heart disease anatomical and physiological (ACHD AP) classification system on the surgical management of Ebstein anomaly (EA) in adult patients.METHODSFrom February 2000 through August 2017, data of patients aged at least 16 years, who underwent primary EA surgery, were retrospectively evaluated. The cohort was divided in 2 groups according to their ACHD AP classification: the moderate EA group (IIB, IIC) and the severe EA group (IID). Survival, freedom from reoperation and freedom from occurrence of major adverse advents were estimated.Open in a separate windowRESULTSThere were 33 patients (21 women, 12 men). Eighteen belonged to the moderate group, 15 to the severe group. There were 12 female patients (80%) in the severe group. Patients in the moderate group were younger than those in the severe group (P = 0.02): 32 ± 12 vs 44 ± 15 years old. Thirty tricuspid valve repairs and 3 replacements were performed. Repair was mainly performed in the moderate group (P = 0.02). Overall survival was 90.1 ± 5.4% at 9 months after the operation and did not change in the later follow-up period. It was 100% for patients in the moderate group and 80.0 ± 10.3% in the severe group (P = 0.07), and 75.0 ± 12.5% for female patients of in the severe group compared to 100% for the remaining patients (P = 0.025). Survival free from major adverse events, including reoperation, at 10 years was 60.0 ± 12.6% in the moderate and 38.1% ± 12.9% in the severe group (P = 0.03). No patient in the moderate group evolved to be in the severe group at late follow-up.CONCLUSIONAdult EA patients should undergo surgery earlier when they are still in the moderate ACHD AP classification.     

腔镜不停跳术对左心瓣膜置换术后远期三尖瓣重度反流疗效分析

目的 总结左侧心脏瓣膜置换术后远期出现孤立性重度三尖瓣关闭不全患者的腔镜辅助不停跳三尖瓣手术治疗经验。方法 11例心脏瓣膜疾病患者行左心瓣置换术后远期发生重度三尖瓣关闭不全并右心衰竭,8例出现心脏恶病质综合征及肝肾功能不全,予行再次三尖瓣手术,包括人工瓣环成形术2例,行三尖瓣置换术9例,其中置换生物瓣5例,双在叶机械瓣4例;在腔镜辅助下行心脏不停跳再次手术5例,常规再次心脏停搏手术6例。结果 2例围术期死亡,均为停跳组瓣膜置换病人。术后心包引流液量心脏不停跳组明显少于停跳组（P<0.05）。停跳组术后严重低心排血量综合征4例,不停跳组1例。两组术后1月复查超声心动图,右心房、室均明显缩小,三尖瓣无或少量反流,两组间无明显差异。获长期随访5例、随访时间25~86月、心功能Ⅱ级3例、Ⅲ级2例。结论 左心瓣膜置换术后远期孤立性重度三尖瓣关闭不全合并右心衰的再次手术死亡率高、合理掌握手术指征、手术时机、积极开展微创不停跳手术和良好的围术期治疗是手术成功的关键。对于终末期病例,手术死亡率高,应积极开展针对右心系统的心脏超声及磁共振检测指标,综合评估手术风险,常规换瓣手术指征需慎重,必要时可考虑微创经皮导管瓣膜植入术。     

One and a half ventricle repair for Ebstein’s anomaly

The surgical strategy for patients having Ebstein’s anomaly and hypoplastic right ventricle is controversial. An 11-year-old boy patient having such condition, with estimated end-diastolic volume index of the atrialized and functional right ventricle being 70% of normally expected values, underwent biventricular repair. Immediately after the surgery, however, he developed right heart failure with the central venous pressure of 11 mmHg. He consequently underwent additional bidirectional cavopulmonary anastomosis, thereby converting the biventricular repair into one and a half ventricle repair. He recovered uneventfully and is doing well 2 years after the surgery.