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心脏畸形139例手术治疗经验

目的：总结Ebstein心脏畸形手术治疗的经验。方法：回顾性分析1980年6月至2000年1月手术治疗139例Ebstein心脏畸形病人。其中行三尖瓣成形、房化右心室折叠和三尖瓣环DeVega成形111例，三尖瓣置换术27例，右心室发育不全行心外管道全腔静脉-肺动脉连接术（ETCPC）1例。结果：全组手术死亡12例（8.6％）；近10年手术死亡率降至3.3％。成形组中10例再次行瓣膜置换术，均生存。结论：对Ebstein心脏畸形应根据其美丽解剖特征选择手术方案，轻型可选用三尖瓣成形术；中间A型可选用成形术，中间B型应慎重选择成形或瓣膜置换术；重型选用瓣膜置换术。     

An unusual first presentation of Ebstein's anomaly in a 72-year-old patient

We report the unusual case of a 72-year-old woman with a primary presentation of right heart failure in the setting of recently diagnosed Ebstein's anomaly with concomitant atrial fibrillation (AF). The patient had New York Heart Association (NYHA) class III dyspnea for 12 months, with refractory right heart failure prior to undergoing surgical management in the form of a tricuspid annuloplasty ring and plication of the atrialized ventricle. The patient had an uneventful postoperative recovery and enjoyed an improvement in her exercise tolerance (NYHA class I) with minimal echocardiographic evidence of tricuspid regurgitation (TR) at the latest follow-up.     

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.     

Long-term follow-up after repair of Ebstein's anomaly.

OBJECTIVE: We studied the long-term results of vertical plication repair of Ebstein's anomaly according to Carpentier. METHODS: Between 1988 and 2007, 28 patients (mean age 28.8+/-15.7 years, range 4-58 years) underwent vertical plication repair of Ebstein's anomaly. At operation the anomaly was classified according to Carpentier. In three patients (11%) a cavopulmonary shunt was added at the repair on the indication of impaired right ventricular function. RESULTS: There was no operative mortality. Early mortality was 3.6% (one patient). Actuarial survival and actuarial freedom from reoperation at 19 years were 96% (95% CI; 96-97%) and 72% (95% CI; 53-92%), respectively. Six patients required reoperation, with a successful re-repair in three patients. Mean duration of follow-up was 10.7+/-6.5 years. One year postoperatively, tricuspid incompetence had decreased significantly (p<0.001), as had New York Heart Association (NYHA) functional class (p<0.001). In addition, exercise tolerance had increased (70+/-19% to 92+/-9% of predicted values, p<0.05). Both tricuspid function and NYHA functional class remained essentially unchanged at the end of follow-up, indicating durable haemodynamic and functional results. CONCLUSION: This study demonstrates favourable long-term results following vertical plication repair of Ebstein's anomaly with low mortality, acceptable morbidity and good haemodynamic and functional results.     

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.     

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.     

Repair of Ebstein's anomaly in an elderly patient; report of a case

The operative repair of Ebstein's anomaly is performed usually during the younger age. On the other hand, the operative indication of asymptomatic Ebstein's anomaly in adult patients has not been clearly defined. We encountered a 71-year-old female patient with asymptomatic Ebstein's anomaly. Because of severe tricuspid regurgitation (TR) and right ventricular dilatation, we repaired the tricuspid valve configuration. The operation was successful and medium term result was excellent. We believe that severe TR with moderate right ventricular dysfunction can be the operative indication in adult patients with asymptomatic Ebstein's anomaly especially when tricuspid valve repair is possible.     

Tricuspid valve replacement with a mitral homograft in children with rheumatic tricuspid valvulopathy

OBJECTIVE: Tricuspid valve replacement in children is associated with a nonnegligible complication rate because of specific disadvantages of mechanical or biologic prostheses. The objective of this study was to examine the midterm clinical outcomes of tricuspid valve replacement with a mitral homograft in 8 children with unreparable rheumatic tricuspid valve involvement. METHODS: Between 1993 and 2003, tricuspid valve replacement with a mitral homograft was performed in 8 patients (2 male and 6 female patients; mean age, 14.2 years) with rheumatic tricuspid valve disease. All patients were in New York Heart Association class III or IV. In all patients with rheumatic valve disease, conservative operations had previously been performed on the tricuspid valve during concomitant left-sided surgical intervention. Mean follow-up was 56 +/- 12 months and was complete. RESULTS: There were no operative or late deaths. All patients were alive at the most recent follow-up contact and were in New York Heart Association functional class I or II. None of the patients required homograft-related reoperation. At the most recent echocardiograhic examination, 6 patients had trivial residual tricuspid regurgitation, and 2 had mild tricuspid regurgitation. None of the patients had maximal transvalvular tricuspid gradients greater than 2 mm Hg during their yearly follow-up visits. CONCLUSION: On the basis of our midterm results, tricuspid valve replacement with a mitral homograft in children seems to be a valuable alternative surgical option.     

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.     

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.     

Ebstein心脏畸形外科矫治39例

目的　总结Ebstein心脏畸形外科治疗经验。方法　回顾近 18年收治Ebstein畸形 39例 ,其中重型 8例 ,中间型 2 7例 ,轻型 4例。采用改良Danielson法矫正畸形 30例 ,其中 19例同时行瓣环成形 ,3例行瓣叶修补 ,6例行瓣交界成形 ;单纯三尖瓣成形 3例 ;Minale法修复 1例 ;双向上腔静脉—肺动脉连接和三尖瓣成形 1例 ;4例行三尖瓣置换。结果　 2例 (成形和换瓣各 1例 )术后早期死于右心衰竭(5 1% )。成形术后三尖瓣功能正常 2 7例 (77 1% ) ,轻至中度关闭不全 5例 (14 3 % ) ,中至重度关闭不全 3例 (8 6 % )。生存者随访 0 5～ 18年 ,平均 8 6年。 33例心功能I级 ,3例心功能II级 (成形 2例、瓣膜替换 1例 ) ,1例行人工瓣置换者术后顽固性右心衰竭 ,于 1年后失访。结论　Ebstein畸形应尽早外科治疗。修复成形多能矫正畸形 ,重症者可同时行双向腔—肺动脉连接术     

Management of Ebstein's anomaly and pure tricuspid insufficiency in the neonate

Because the pulmonary vascular resistance is very elevated at birth, severe tricuspid regurgitation is poorly tolerated and even life-threatening in the newborn. The etiology may be tricuspid valve papillary muscle rupture or the more ominous Ebstein's anomaly, with its associated dysfunctional right ventricle. After the diagnosis is established and the patient is supported with prostaglandin infusion and nitric oxide, definitive surgical management is undertaken with the expectation of excellent outcomes for isolated tricuspid valve regurgitation. For neonates with Ebstein's anomaly, therapy is tailored to the severity of the malformation and the degree of right ventricular outflow tract obstruction, assessed in the context of declining pulmonary vascular resistance. The surgical approach may involve ligation of a patent ductus arteriosus, placement of a systemic to pulmonary shunt, establishment of functional tricuspid atresia, or tricuspid valve repair. With the application of these various approaches, the outlook for neonatal Ebstein's anomaly has improved remarkably.     

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.     

Combination of a Hetzer operation and a Sebening stitch for Ebstein's anomaly

OBJECTIVE: In order to improve the function of the anterior leaflet as a monocusp valve in tricuspid valve repair for Ebstein's anomaly, a single-stitch technique after Sebening, creating a hinge point of the anterior leaflet on the ventricular septum on the opposite side, was newly incorporated into the Hetzer operation (either posterior annulorrhaphy or the double-orifice technique). We describe the operative technique of the modified operation and present the initial results. METHODS: Eleven patients who underwent the modified operation were involved in the study. Tricuspid valve function was assessed by echocardiography preoperatively and 3 months after the operation. Its improvement was studied, and compared with that after the conventional Hetzer operation (n=15). RESULTS: After a mean follow-up period of 18.4 months (range 8.2-32.6 months), there were no early deaths and no cases of reoperation on the tricuspid valve in patients who had undergone the modified operation. At the last follow-up, 7 patients were in New York Heart Association class I, and 4 patients were in class II. The function of the tricuspid valve was significantly improved 3 months after the modified operation (P < 0.01, Wilcoxon signed-ranks test). Among the patients with Carpentier's classification type A or B, 85.7% of patients showed a tricuspid valve regurgitation (TR) grade of less than 2 after the modified operation, and this rate was significantly higher than that of 30.0% of patients in the group who underwent the conventional operation, (P < 0.05, Fisher's exact test). CONCLUSION: The initial results of the new modification are satisfactory. However, a further follow-up and an accumulation of operative cases are necessary.     

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.     

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 supra-annular implantation in adult patients with Ebstein's anomaly

BACKGROUND: Tricuspid valve supra-annular implantation (TVSI) has been performed for adult patients with Ebstein's anomaly at our hospital for several decades. TVSI is characterized by reliable reduction of tricuspid annulus size without affecting the conduction system; by prevention of residual tricuspid regurgitation (RTR) through preservation of the native tricuspid valve; and by implantation of the bioprosthesis at a supra-annular site. METHODS: Ten adult patients with Ebstein's anomaly underwent TVSI. The right ventricular diameter and residual tricuspid regurgitation were evaluated by echocardiography preoperatively, at discharge, 1 year after the operation, and over the long term (12.4 +/- 5.5 years). Actuarial survival rate, actuarial freedom from structural valve deterioration rate, and postoperative occurrence of arrhythmia were also evaluated. RESULTS: The actuarial survival rate at 19 years was 76 +/- 15%. Tricuspid regurgitation disappeared in 8 patients just after operation. Right ventricular diameter was significantly smaller at discharge than preoperatively (63 +/- 11 vs 37 +/- 9, p < 0.01), and there were no significant differences between values at discharge and at follow-up. The actuarial freedom from structural valve deterioration rate and the reoperation rate were both 100%. There were no fatal complications related to arrhythmia or thromboembolism. CONCLUSIONS: TVSI is useful for adult patients with Ebstein's anomaly. The absence of complications related to fatal arrhythmia and thromboembolism, good durability of the bioprosthesis, and a simple operative procedure are merits of this therapy.     

Surgical assessment of tricuspid valve replacement for severe tricuspid regurgitation without stenosis.

Eight patients undergoing tricuspid valve replacement (TVR) for severe tricuspid regurgitation (TR) without stenosis were subjected to the study. Four patients had primary TR due to trauma, infective endocarditis, or Ebstein's anomaly, and the other 4 had secondary TR associated with mitral valve lesions. The right heart pressure as well as clinical manifestations due to right heart failure showed deteriorated values in both the primary and secondary TR groups, though pulmonary arterial pressure was higher in the latter group. In 2 patients, initial annuloplasty procedures could not reduce TR sufficiently and were instantly abandoned for TVR. These 2 patients, one with congenital and one with secondary TR, died of intractable right heart failure early postoperatively, while the remaining 6 patients are alive to date, in the New York Heart Association (NYHA) functional class I, during the follow-up period of 6-192 (mean, 104) months. The 2 patients who died had shown a longer morbid duration and a lower preoperative right ventricular systolic function indicated by stroke work to pulmonary artery resistance. This paper might suggest that an earlier surgical intervention in severe TR is recommended before the right ventricular function deteriorates.     

Surgical management of Ebstein's anomaly in the adult

Ebstein's anomaly is a rare cardiac malformation that affects the tricuspid valve, right ventricle, and right atrioventricular junction. These anatomical and functional abnormalities cause important tricuspid regurgitation that results in right atrial and right ventricular dilatation and atrial and ventricular arrhythmias. Diagnosis is made by echocardiography. Operation includes tricuspid valve repair or replacement, closure of any interatrial communications, and appropriate antiarrhythmia procedures. Repair of Ebstein's anomaly eliminates right-to-left intracardiac shunting, improves exercise tolerance and functional class, and reduces supraventricular arrhythmias. In addition, quality of life and longevity are improved.