Tricuspid valve replacement in the true annulus of Ebstein's anomaly without involvement of atrioventricular conduction disturbance

Two female patients with Ebstein's anomaly, aged 12 and 45 years, underwent new procedures of tricuspid valve replacement in the true annulus without involvement of atrioventricular conduction disturbance. The first patient had a relatively small atrialized right ventricle. A Dacron patch was sutured on the atrialized portion from the right ventricular side. Buttress stitches were placed on the whole of the atrialized ventricle except at the part of the His' bundle. A porcine valve was sutured in the true annulus of the tricuspid valve and on the top of the patch. The second patient had a fairly large atrialized ventricle, and plication was made only in the inferior part of the atrialized ventricle form the coronary sinus. A porcine valve was sutured in the true tricuspid annulus except at the part of Koch's triangle, where the porcine valve was placed on the false annulus.     

Posterior annular plication: tricuspid valve repair in Ebstein's anomaly

BACKGROUND: Nonneonatal Ebstein's anomaly is a rare congenital anomaly for which numerous operative techniques have been described to repair the aberrant tricuspid valve, exclude the atrialized right ventricle, and address right heart dysfunction. We reviewed short-term outcomes in surgical patients treated by a simplified operative technique. METHODS: Our operative approach to Ebstein's anomaly is approximation of the anteroposterior commissure to the remnant septal leaflet with closure of the cul-de-sac longitudinally. Plication of the atrialized right ventricle (1 patient) and a bidirectional cavopulmonary connection (2 patients) were performed only if necessary. All patients were followed postoperatively by their cardiac surgeon and cardiologist. RESULTS: Seven patients with a mean age of 39 years (range, 3.6 to 63.8 years) underwent repair. Preoperatively all patients had 4+ tricuspid valve regurgitation and were New York Heart Association class III. Mean postoperative hospital stay was 7 +/- 2 days (range, 4 to 11 days). Mean follow-up is 42 +/- 18 months (range, 7 to 58 months). At last follow-up 5 patients are New York Heart Association class I and average tricuspid valve regurgitation is mild. CONCLUSIONS: Plication of the posterior annulus without plication of the atrialized right ventricle, resiting the tricuspid valve, or performing prophylactic cavopulmonary connection appears to be a reasonable operative approach to nonneonatal Ebstein's anomaly. Long-term follow-up of this cohort is necessary to determine the durability of such a surgical approach.     

A case report of Ebstein's anomaly treated with Carpentier's procedure

A 42-year-old male patient was admitted with congestive heart failure. Echocardiogram and cardiac angiogram revealed Ebstein's anomaly and severe tricuspid valve incompetence. He underwent Carpentier's procedure for the treatment of Ebstein's anomaly. Carpentier's procedure consists of the following two new points; (1) the right atrium and atrialized ventricle is plicated longitudinally; (2) the anterior leaflet and the posterior leaflet of tricuspid valve are transposed with a clockwise rotation to the level of the normal tricuspid anulus. Compared with Hardy's procedure (transverse plication), longitudinal plication preserves the cavity and the function of the right ventricle and excludes atrialized chamber. In our case, there is a trivial residual regurgitation of tricuspid valve postoperatively, but the patient's clinical status has improved remarkably. We conclude that Carpentier's procedure is an effective operation for Ebstein's anomaly.     

Surgical repair of Ebstein's anomaly.

Ebstein's anomaly is a complex malformation that has been treated by various surgical techniques, with variable results, since 1958. In 1972, the authors developed a repair that consisted of plication of the free wall of the atrialized portion of the right ventricle, posterior tricuspid anuloplasty, and right atrial reduction. The repair is based on the construction of a monocusp valve by the use of the anterior leaflet of the tricuspid valve, which is usually enlarged in this anomaly. Not all patients are candidates for this procedure, however, because of significant abnormalities of the anterior leaflet such as inadequate size or attachment of the free edge of the leaflet to the ventricular wall. This report describes a ten-year experience with surgical repair of Ebstein's anomaly.     

Valve replacement for Ebstein's anomaly of the tricuspid valve.

The role of valve replacement in the treatment of Ebstein's anomaly of the tricuspid valve remains controversial. Between 1965 and 1977, five patients with Ebstein's anomaly ranging in age from 11 to 57 years (mean 29) underwent tricuspid valve replacement in our institution. All of the patients were cyanotic but one, three were in Functional Class III, and two were in Class IV (New York Heart Association classification). The valve was placed above the coronary sinus. Plication of the atrialized portion of right ventricle was required in only one patient. Four patients had additional closure of an atrial septal defect. There were no deaths. On follow-up (range 1 to 13 years), four patients are in Functional Class I and one is in Class II. None is cyanotic. Four patients are in sinus rhythm and one has a pacemaker because of postoperative complete heart block. We conclude that valve replacement and, when necessary, plication of the atrialized right ventricle produce excellent clinical improvement.     

Ebstein's anomaly in an adult

A 56-year-old female who had been diagnosed with Ebstein's anomaly was admitted with cyanosis and congestive heart failure. The echocardiogram showed severe tricuspid valve incompetence, displacement of the tricuspid valve and dilatation of the atrialized portion of the right ventricle. Atrial fibrillation was detected in the electrocardiogram. She underwent tricuspid valve replacement and right atrial maze procedure. She is released from congestive heart failure and remains in sinus rhythm 48 months after the operation.     

Tricuspid Annuloplasty and Ventricular Plication for Ebstein’s Malformation

Background. Seven patients with the diagnosis of Ebstein’s malformation of the tricuspid valve were operated on. Mean age was 12 years (range, 7 to 16 years). All were cyanotic, with severe tricuspid regurgitation. Thromboembolism was not present. No associated cardiac malformations were present.

Methods. Surgical repair included tricuspid annuloplasty associated with longitudinal plication of the atrialized portion of the right ventricle. This was attained by approximating the anterior-posterior commissure with either the posterior-septal commissure or the septal leaflet remnant. The thin atrialized ventricular wall thus excluded remained as a cul du sac and was plicated by suturing along the longitudinal axis of the heart. When present, the dysplastic posterior leaflet was included in the plication. In essence, a monocuspid right atrioventricular valve was fashioned out of the anterior leaflet. The remaining septal leaflet played a minimal functional role. No additional procedures for treatment of arrhythmia were associated with the technique described.

Results. The postoperative course was uneventful in all patients. Mean follow-up is 4.3 years (range, 1 to 10 years). Doppler echocardiographic studies reveal satisfactory monocusp valve function in all patients, with adequate coaptation of the anterior leaflet and the septal structures.

Conclusions. This technique seems applicable to most forms of Ebstein’s malformation and is reproducible. The technique relies on the adequate mobilization of the anterior leaflet. Occasionally it is necessary to free fibrous adhesions of the leaflet to the underlying ventricular surface.     

Carpentier's procedure for Ebstein's anomaly: successful and failed cases]

Two adult cases of Ebstein's anomaly underwent Carpentier's procedure. In the first case longitudinal plication limited to free wall of atrialized ventricle was performed and postoperative course was uneventful. In the second case preoperative echocardiography showed apparently restricted movement of anterior leaflet of the tricuspid valve which was compatible with intraoperative findings. That is, inferior edge of anterior leaflet was partly adherent to ventricular wall and systolic bulging of leaflet was significantly impaired which was left untouched but should be repaired by additional procedure. Six days after operation the tricuspid valve replacement was required for persistent right heart failure due to residual tricuspid regurgitation. In the same case longitudinal plication of atrialized ventricle reported by Carpentier and colleagues resulted in excessively small annulus. Therefore we had to reduce the plication and did not perform following atrial plication to avoid direct injury to conduction system or disturbing coronary venous return. In conclusion exact preoperative evaluation of anterior leaflet of the tricuspid valve especially subvalvular anatomy is essential to Carpentier's procedure, as Carpentier and colleagues emphasized, and conservative longitudinal plication of the atrialized ventricle limited to free wall is favorable when excessively small annulus might be concerned.     

Early and medium-term results for repair of Ebstein anomaly

OBJECTIVES: We evaluated the early and medium-term single-center results for primary repair of Ebstein anomaly in both adults and children. METHODS: The records were reviewed of patients undergoing repair of Ebstein anomaly at the Children's Hospital of New York from September 1990 to September 2002. Functional, demographic, and echocardiographic parameters were studied both preoperatively and postoperatively, along with functional status and adverse events. The repair technique involved vertical plication of the atrialized ventricle and valve leaflet reimplantation after clockwise rotation. RESULTS: A total of 25 patients (19 children and 6 adults) underwent repair. The average age was 14.2 +/- 15.9 years, and the average follow-up was 4.1 +/- 3.4 years. Three patients required reoperation for right ventricular overload (1 child) and progressive, severe tricuspid regurgitation (2 adults); both adults received tricuspid valve replacements, one at 4 years and the other at 8 years post-repair. Three patients had radiofrequency ablation procedures performed intraoperatively. Ten patients (40%) had moderate-to-severe tricuspid regurgitation perioperatively. However, 18 children (95%) and 5 adults (83%) demonstrated significant improvement in exercise capacity late postoperatively. Two children died suddenly 11 months and 4 years after repair. DISCUSSION: Ebstein repair has good functional outcomes in children despite residual tricuspid regurgitation, likely because of reduction in right ventricular volume loading and relative annular and ventricular plasticity. Adult patients did not demonstrate the same durability of valve repair and frequently required tricuspid valve replacement. Intraoperative radiofrequency ablation represents an important adjunctive treatment for intractable arrhythmias, which may now represent relative indications for operative intervention.     

A new procedure for Ebstein's anomaly

BACKGROUND: A new procedure for correction of Ebstein's anomaly that restores to near normal the anatomic and physiologic function of the tricuspid valve and the right ventricle is reported. METHODS: Between December 1997 and September 2002, 34 consecutive patients with Ebstein's anomaly underwent this new procedure. There were 13 male and 21 female patients aged 9 months to 48 years (mean, 17 years). Tricuspid incompetence was moderate in 12 patients and severe in 22. Our repair technique is as follows: the displaced posterior leaflet with some chordae tendineae and corresponding papillary muscle are detached from the annulus and ventricular wall, respectively. The leaflet is then reattached to the native posterior annulus with reimplantation of the papillary muscle. The displaced septal leaflet is treated in the same manner. Most of the atrialized portion of the ventricular wall is excised; the tricuspid annulus is plicated. In 8 of the patients the septal leaflet was severely hypoplastic and necessitated creation of a new leaflet using autologous pericardium. RESULTS: All patients survived and recovered uneventfully. Postoperative echocardiography showed that tricuspid incompetence disappeared in 29 patients and was mild in 5. Right ventricular size decreased significantly with complete disappearance of the atrialized segment. Follow-up of patients ranged from 1 to 55 months (mean, 25 months), with 9 patients having more than 3 years of follow-up. They are doing well and their exercise tolerance improved to normal. CONCLUSIONS: This new procedure anatomically corrects Ebstein's anomaly with the satisfactory early and midterm results.     

Tricuspid valve avulsion or excision for right ventricular decompression in pulmonary atresia with intact ventricular septum

Retrograde right ventricular decompression through the tricuspid valve, by damaging or excising the valve, was attempted in five patients with pulmonary atresia, intact ventricular septum, and severely hypoplastic right ventricle. Two patients were neonates in critical condition, one of whom had received previous palliation with a shunt; the other neonate had received pulmonary valvotomy plus shunt followed by repeat valvotomy plus shunt ligation. Three patients were infants or children who had undergone placement of isolated neonatal shunts. One procedure was performed during cardiac catheterization. Three patients survived. In one, tricuspid avulsion and a Rashkind septostomy were done by percutaneous methods; in two patients, tricuspid excision plus bidirectional cavopulmonary anastomosis was done by open operation. At restudy, all showed subsystemic pressure in the right ventricle and diminution or disappearance of sinusoids. One also showed improvement of left ventricular function. Incorporation of a small right ventricle in the right atrium at the time of the two orthoterminal palliations seemed, at least, innocuous. Right ventricular decompression by tricuspid avulsion or excision could be suggested for all patients with pulmonary atresia, intact ventricular septum, hypersystemic hypoplastic right ventricle, and major sinusoids without right ventricular dependent coronary circulation in whom the outlet portion of the right ventricle is not functional.     

Ebstein's anomaly: repair based on functional analysis.

OBJECTIVE: 'Classical' repair of Ebstein's anomaly is usually performed with transverse plication of the atrialized chamber. However, the anterior leaflet has restricted motion which is an important factor of the tricuspid valve insufficiency. We studied the long term results of mobilization of the anterior leaflet associated with longitudinal plication of the right ventricule. METHODS: From 1980 to July 2002, 191 patients (mean age 24.4+/-15 years (1-65)) were operated on. Anterior leaflet function was assessed on pre-op echocardiography and on surgical examination. Conservative surgery was possible in 187 patients (98%) and included mobilization of the anterior leaflet, longitudinal plication of the right ventricle and prosthetic annuloplasty in adults. Bidirectional cavo-pulmonary shunt was associated in 60 patients. Four patients had valve replacement. RESULTS: Hospital mortality occurred in 18 patients: 9% (95%CL: 6-15%) due to right ventricle (RV) failure in nine patients. Mean follow-up was 6.4 years (0.07-22). Actuarial survival was 82% at 20 years. Tricuspid valve insufficiency was 1 or 2+ in 80% of the cases. Reoperation occurred in 8% (16 patients). A successful second repair was obtained in ten patients. Electron beam computerized tomography (20 patients) demonstrated improved left ventricle ejection fraction 56-66% (P<0.05). Supraventricular tachycardia and pre-excitation syndromes were reduced from 23 to 5%. CONCLUSION: Conservative surgery is indicated for all symptomatic patients. The incidence of valve repair is high when leaflet mobilization is performed. Valve replacement can be avoided in most cases. Functional and hemodynamic results are excellent.     

A case of two-chambered right ventricle complicating infective endocarditis and tricuspid regurgitation

A 31-year-old male of two-chambered right ventricle with ventricular septal defect, complicating infective endocarditis and tricuspid regurgitation, was presented. Two-dimensional echocardiographic study demonstrated tricuspid vegetations and a hypertrophied, anomalous muscle bundle in the right ventricle. Cardiac catheterization revealed 58 mmHg pressure gradient between inflow chamber and outflow chamber of the right ventricle. It seems that tricuspid regurgitation was resulted from infective endocarditis. He underwent resection of anomalous muscle bundle, repair of ventricular septal defect, and tricuspid valve replacement with satisfactory result. It has not been reported in Japan so far that tricuspid valve replacement was performed for the treatment of tricuspid regurgitation due to infective endocarditis in the patient with two-chambered right ventricle. In our case, cardiac catheterization was performed after subsidence of infective endocarditis. As echocardiography can detect vegetations and anomalous muscle bundle precisely, surgical intervention would be performed without cardiac catheterization in the case of infective endocarditis intractable to medical therapy.     

Repair of septal and posterior tricuspid leaflets in Ebstein's anomaly

BACKGROUND AND AIM: In Ebstein's anomaly, the septal and posterior tricuspid leaflets are plastered to the endocardium. We postulated that tricuspid valve function could be corrected by restoring mobility of these leaflets. (Feasibility of such repair was explored by anatomical and clinical studies.) METHODS: Ten heart specimens with Ebstein's anomaly were examined to investigate the size of the tricuspid leaflets. We operated on four patients with Ebstein's anomaly: the plastered septal and posterior leaflets were mobilized from the endocardium, the atrialized right ventricle was longitudinally plicated, and the basal attachment of the mobilized leaflets was sutured (reattached) to the valve annulus. RESULTS: In heart specimens, approximately 40% of the total surface of the tricuspid leaflets was comprised of the septal and posterior leaflets. Clinically, all patients operated on returned to normal functional status after surgery. The mean cardiothoracic ratio on chest X-rays decreased from 0.70 to 0.55 (after surgery). Echocardiographic tricuspid regurgitation, graded from 0 to 4, decreased from 3.5 to 1.0, and tricuspid annular diameter ratio to the normal value reduced from 1.88 to 0.66. Angiographic right ventricular ejection fraction increased from 0.36 to 0.50, and end-diastolic volume ratio to the normal value decreased from 3.65 to 1.19. CONCLUSIONS: Repair of the septal and posterior tricuspid leaflets was found to be feasible and effective as tricuspid valvuloplasty for Ebstein's anomaly.     

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。     

A case report of Ebstein's anomaly treated with Hetzer's procedure

A 27-year-old male who had been diagnosed with Ebstein's anomaly was admitted with uncontrollable congestive heart failure. The echocardiogram revealed severe tricuspid valve incompetence and the electrocardiogram showed atrial fibrillation. He underwent Hetzer's repair procedure for tricuspid valve incompetence and Minzioni's right atrial isolation technique to restore sinus rhythm. His congestive heart failure quickly disappeared and sinus rhythm was restored after operation. He was discharged 3 weeks postoperatively and remains well 22 months after his operation. Hetzer's technique for tricuspid valve repair in Ebstein's anomaly restructures the valve mechanism at the level of the true tricuspid anulus by using the most mobile leaflet for valve closure without plication of the atrialized chamber. We conclude that Hetzer's procedure is an effective operation for Ebstein's anomaly.     

Surgical Treatment in Ebstein's Malformation

Between January, 1967, and July, 1981, 24 patients with Ebstein's malformation underwent surgical repair. Sixteen had tricuspid valve replacement; 8 did not. All interatrial communications were closed. Two patients had plication of atrialized ventricle. Twenty had dysrhythmias; these were surgically treated in 4. Four patients (17%), each with valve replacement, died in the hospital (70% confidence limits, 9-28%); 3 of these were among the 6 patients who were preoperatively in New York Heart Association Functional Class IV. There were 3 late deaths, 1 from noncardiac causes and 2 from persistent tricuspid regurgitation in patients without valve replacement. No late deaths or valve-related complications occurred in the valve replacement group. Dysrhythmias remain a problem, although most patients are symptomatically improved after operation.This experience suggests that good early and late results are obtained with replacement of incompetent tricuspid valves if this is done before advanced symptomatic deterioration, that plication rarely is necessary, and that rhythm disturbances should be recognized and appropriately managed.     

三尖瓣置换治疗Ebstein心脏畸形

目的 确定Ｅｂｓｔｅｉｎ畸形病人瓣膜转换术的手术适应证。方法 ３１例５～４６岁病人,其中１０例曾接受过修复术,全部手术均在全麻体体外循环下完成,４例心脏不停跳。分别转换生物瓣２枚,国产人工机械瓣膜１３枚和进口人工机械瓣６枚;同时对其他合并畸形进行修复。结果 体外循环转流时间５６～１３６ｍｉｎ,２７例主动脉阻断时间２９～８３ｍｉｎ。１２例病人手术结束时直视下测压,右心房压１５．８／７．５ｍｉｎＨｇ（     

矫正型大动脉转位形态三尖瓣置换术及中远期结果

目的探讨矫正型大动脉转位（cTGA）患者行形态三尖瓣置换术的手术适应证及其术后中远期结果。方法1997年9月至2007年9月,阜外心血管病医院收治cTGA患者18例,男15例,女3例;年龄16~51岁（33.3±12.8岁）,体重47~90 kg（60.9±14.7 kg）。单纯形态三尖瓣关闭不全10例,合并室间隔缺损3例,合并室间隔缺损及肺动脉瓣狭窄2例,室间隔缺损修补术后形态三尖瓣关闭不全2例,形态三尖瓣置换术后机械瓣功能不良1例。术前形态右心室射血分数为56.2%±11.6%,心功能分级（NYHA）Ⅱ级12例,Ⅲ级6例。18例cTGA患者均施行形态三尖瓣置换术。术后随访患者的心功能、形态右心室射血分数等指标。结果手术死亡1例,死于术后低心排血量综合征。术后发生瓣周漏2例,分别于术后7 d和30 d行瓣周漏修补术后治愈。随访16例,随访时间57.0±40.7个月;随访时形态右心室射血分数与术前比较差异无统计学意义（52.8%±9.2%vs.56.2%±11.6%;t=2.062,P〉0.05）,心功能分级（NYHA）Ⅰ~Ⅱ级12例,Ⅲ级4例,与术前比较心功能分级Ⅲ级者所占百分比差异无统计学意义（χ2=1.532,P〉0.05）。结论cTGA形态三尖瓣置换术能防止形态三尖瓣关闭不全对形态右心室功能的进一步损害;其中远期结果满意;随访期间,形态右心室功能基本正常。     

Technik der Trikuspidalklappenrekonstruktion

Malformations of the tricuspid valve are less commonly observed than diseases of the valves of the left ventricle. In particular, for a long time little attention was paid to the mostly secondary tricuspid valve regurgitation and the general opinion was that the secondary tricuspid valve insufficiency would be automatically improved by correction of the left ventricular pathology. After experiences from cardiac surgery of congenital defects and the long-term practice to completely or extensively resect the tricuspid valve in drug addicts with endocarditis, the assumption developed that the heart can adequately function without this valve. This line of thought was supported by the functionality of the Fontan circulation with passive perfusion without the function of the right ventricle and right ventricular valves; however, the processing of long-term results showed that most of these patients develop a relevant right heart decompensation. Currently, particular attention is paid to the “prophylactic” correction of the tricuspid valve during other cardiac surgery interventions because the number of postoperatively developing or aggravating secondary tricuspid valve regurgitations is considerable with an underlying left ventricular or pulmonary pathology. Particular problems are the technique and the indications for tricuspid valve reconstruction by high-grade impairment of right ventricular function.