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J Thorac Cardiovasc Surg 2007;134:1438-1442
© 2007 The American Association for Thoracic Surgery

Surgery for Congenital Heart Disease

Early and midterm results in anatomic repair of Ebstein anomaly

^a Heart Center, First Hospital of Tsinghua University, Beijing, China
^b Department of Cardiovascular Surgery, Cardiovascular Institute, Fu Wai Hospital, Chinese Academy of Medical Science, Peking Union Medical College, Beijing, China.

Read at the Eighty-seventh Annual Meeting of The American Association for Thoracic Surgery, Washington, DC, May 5-9, 2007.

Received for publication May 7, 2007; revisions received August 22, 2007; accepted for publication August 30, 2007.

^_* Address for reprints: Qingyu Wu, MD, Heart Center, First Hospital of Tsinghua University, Beijing, China. (Email: wuqingyu{at}mail.tsinghua.edu.cn).

	Abstract

Top Abstract Introduction Patients and Methods Surgical Procedure Results Discussion References

 
Objective: We report the results of surgical treatment of Ebstein anomaly during a 10-year period, especially with an anatomic repair technique that restores to near normal anatomic and physiologic function of the tricuspid valve and the right ventricle.

Methods: Between December 1997 and December 2006, 83 consecutive patients aged 9 months to 52 years underwent operation for Ebstein anomaly. Among them, 78 underwent the anatomic repair technique (male 39). Tricuspid incompetence was moderate in 22 patients and severe in 56 patients. The main surgical technique includes excision of the atrialized right ventricle, detachment and repair of the leaflet, transposition of the leaflet, and anulus plication of the tricuspid valve. In some patients in whom the septal leaflet was severely hypoplastic or absent, a new leaflet was created with autologous pericardium. Biventricular repair was not performed in 5 patients with very severely hypoplastic tricuspid valves and poor right ventricular function.

Results: All patients survived and recovered uneventfully. For the 78 patients who underwent anatomic repair, postoperative echocardiography showed that tricuspid incompetence disappeared in 65 patients and was mild in 13 patients. Right ventricular function was good. Patients have been doing well during a mean follow-up of 46 months (2 months to 9 years). The latest follow-up echocardiograms showed that tricuspid incompetence was eliminated in 55 patients and was mild in 17, moderate in 3, and severe in 3. The 5 patients with a total cavopulmonary connection procedure and one-and-one-half-ventricle correction procedure are also doing well.

Conclusions: Satisfactory early and midterm results can be achieved with the anatomic repair technique to correct Ebstein anomaly. Tricuspid valve replacement can be avoided in most patients.

	Introduction

Top Abstract Introduction Patients and Methods Surgical Procedure Results Discussion References

Dr Wu

Ebstein anomaly refers to a condition with downward displacement of the septal and posterior leaflets, occasionally with isolated anterior leaflet of the tricuspid valve into the right ventricle, leaving an atrialized portion of the right ventricle. Operations for Ebstein anomaly include tricuspid valve repair and replacement. The long-term results of tricuspid valve replacement are not satisfactory because of anticoagulant-related complications concerning mechanical prosthetic valves or calcification and degeneration of bioprosthetic valves. Several kinds of repair techniques with good results have been reported.^1-4 We report our experience in the surgical treatment of Ebstein anomaly with the anatomic repair technique.

	Patients and Methods

Top Abstract Introduction Patients and Methods Surgical Procedure Results Discussion References

 
Between December 1997 and December 2006, 83 consecutive patients with Ebstein anomaly underwent operation, and 78 of them were treated with the anatomic repair technique. There were 40 male and 43 female patients aged 9 months to 52 years (17.9 ± 7.2 years). Patients were in New York Heart Association functional class II (n = 24) or functional class III (n = 59). Six patients had cyanosis during exercise and 7 had cyanosis at rest. Electrocardiograms indicated right ventricular hypertrophy in 9 patients, complete right bundle branch block in 18, atrial fibrillation in 2, and Wolff–Parkinson–White syndrome in 5; 1 patient had undergone successful catheter ablation of his abnormal pathway before the operation. The preoperative cardiothoracic ratio ranged from 0.46 to 0.76 (mean, 0.63). The diagnosis was established by preoperative echocardiography in all patients. Tricuspid incompetence was moderate in 22 patients and severe in 56. In 1 patient the diagnosis of double-outlet right ventricle and pulmonary stenosis was made by ultrasonic cardiography and cardioangiography. The diagnosis was confirmed and an extra diagnosis of Ebstein anomaly was added on the basis of the operative findings. Associated complicated lesions masked the Ebstein anomaly in this patient.

	Surgical Procedure

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All operations were done with the patient under general anesthesia with total cardiopulmonary bypass, aortic and bicaval cannulation, and systemic hypothermia (25°C-30°C). The mean aortic crossclamp time was 60 ± 21 minutes, and the mean bypass time was 100 ± 34 minutes. After the aorta was crossclamped, cardioplegic solution was administered through the aortic root. The right atrium was opened parallel to the atrioventricular groove, and the tricuspid valve was inspected. The main surgical technique includes excision of the atrialized right ventricle, detachment and repair of the leaflet, transposition of the leaflet, and anulus plication of the tricuspid valve, as described before.⁵ In 22 patients with adequate-sized posterior and septal leaflets, the detached leaflets were reattached to a position just below the natural annulus with their corresponding papillary muscle reimplanted. In 30 patients, a "new leaflet" was made by using autologous maldeveloped posterior and septal leaflet tissue. In 26 patients the septal leaflet was severely hypoplastic, appearing like tiny membranous tissue remnants of the original leaflet. For these patients, a piece of fresh autologous pericardium was fashioned as the "new septal leaflet" by suturing the base of the "new leaflet" to a position just below the natural annulus. Autologous tissue and a pericardiac strip were used to make a new chordae, and the abnormal muscle band was freed to substitute for papillary muscle if necessary. Part of the right atrial wall was also resected. There were 2 patients with extremely uncommon conditions: the isolated anterior leaflet was displaced downward and the large atrialized right ventricle was positioned anteriorly, whereas the septal and posterior leaflets were reasonably developed and in the normal position. These conditions were also corrected with the anatomic repair technique. Associated procedures included atrial septal defect repair in 18 patients, closure of a patent foramen ovale in 13, ventricular septal defect repair in 3, closure of a patent ductus arteriosus plus ventricular septal defect repair in 2, relief of right ventricular outflow tract obstruction in 8, closure of a patent ductus arteriosus plus relief of right ventricular outflow tract obstruction in 1, and Rastelli operation in 1. Biventricular repair was not performed in 5 patients owing to severe hypoplasia of all three leaflets of the tricuspid valve and very poor right ventricular function. Among them, a total cavopulmonry connection procedure was performed in 4 patients and one-and-one-half-ventricle repair in 1.

An intraoperative transesophageal echocardiogram was obtained routinely to assess the tricuspid valve and ventricular function after the repair.

	Results

Top Abstract Introduction Patients and Methods Surgical Procedure Results Discussion References

 
There were neither early deaths nor significant postoperative complications. No patient had transient or permanent third-degree atrioventricular block and none needed tricuspid valve replacement or pacemaker insertion. Additionally, no patient had postoperative ventricular arrhythmia. Eighty-one patients were in sinus rhythm; the other 2 patients were in atrial fibrillation. Intraoperative transesophageal echocardiography after bypass showed that the tricuspid incompetence disappeared in 65 patients and was mild in 13. There was no tricuspid valve stenosis either. All these findings were confirmed on follow-up echocardiograms 7 days postoperatively, before the patient’s discharge. Postoperative reduction in heart size was significant; the cardiothoracic ratio on chest radiograph had significantly decreased (mean, 0.63 ± 0.07 preoperatively vs 0.59 ± 0.06 postoperatively; P <.01). Echocardiography before discharge showed that right ventricular cavity dimension was remarkably reduced (anteroposterior diameter, 41.3 ± 8.6 mm preoperatively vs 24.3 ± 5.3 mm postoperatively; P < .01), the atrialized chamber had vanished, and the three leaflets of the tricuspid valve were at the level nearing the natural annulus. After surgery, significant improvement in cardiac function was achieved in all patients.

Follow-up time ranged from 2 months to 9 years (46 ± 12.5 months) in all patients. The improved cardiac function was maintained; 64 patients were in New York Heart Association functional class I, and the rest were in functional class II. Seventy-six patients were in sinus rhythm. At the most recent follow-up echocardiograms, tricuspid valve competence was sustained in 54 patients, and tricuspid incompetence was mild in 18, moderate in 3, and severe in 3. Right ventricular cavity dimension had a further decrease (21.2 ± 3.5mm vs 24.3 ± 5.3 mm postoperatively; P < .01). The 5 patients with the total cavopulmonary connection procedure and one-and-one-half-ventricle correction procedure are also doing well.

	Discussion

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The anatomic repair technique for the correction of Ebstein anomaly was successfully applied in 78 consecutive patients. Their excellent early and midterm results allow extension of the scope of repair, even for patients previously deemed to require tricuspid valve replacement.

Ebstein anomaly is a complex congenital defect involving the tricuspid valve and right ventricle. The clinical presentation is closely related to the degree of tricuspid incompetence, right ventricular dysfunction, and associated lesions.

This technique of treating Ebstein anomaly differs from previous techniques^6-12:

1 The atrialized ventricular wall is excised in the shape of a trapezoid or triangle. Therefore, the right ventricular geometry is restored, the atrialized ventricular chamber is completely obliterated, and the load on the right ventricle is alleviated. After surgery, the right ventricular cavity dimensions are restored to near normal, which is beneficial for the recovery of right ventricular function.

2 The displaced leaflets with their partial subvalvular structures are detached from their abnormal positions and reattached to a position near the true tricuspid annulus, and the new leaflet is made with autologous tissue if necessary. This technique improves the function and durability of "the new leaflets." It is important to reattach the septal leaflet strongly enough to avoid suture tear. Although fresh pericardial tissue is not a very satisfactory material to use for the reconstruction of septal leaflet, the leaflet was mobile and functioned well in most patients in this group during follow up.

3 There is less likelihood for the tricuspid annulus to enlarge after the annular plication with a 4-0 suture line because of the effective size reduction of the tricuspid annulus and the restoration of the valve leaflets to a near normal area and position, leading to competence of valve function.

It is important to avoid injury to the coronary arteries and the atrioventricular bundle during septal leaflet reattachment. In this series of patients, none had transient or permanent third-degree atrioventricular block. Our experience is to place the suture just below the atrioventricular bundle. The membranous interventricular septum can be viewed clearly.

For patients with a confirmed diagnosis of Ebstein anomaly, the indications for this procedure are as follows: the patient has symptoms, compromised heart function, moderate and above tricuspid regurgitation, and significantly enlarged right atrium and right ventricle. Most patients with Ebstein anomaly can avoid tricuspid valve replacement. Very few patients with very hypoplastic leaflets and severe dysfunction of the right ventricle may need one-and-one-half-ventricle repair¹³ or some other procedures.

Because of complete excision of the atrialized ventricular wall, a trileaflet mechanism covering the entire orifice of the tricuspid valve and the restoration of the tricuspid annulus to its appropriate position and size, tricuspid valve competence is achieved. The incidence of right ventricular dysfunction and arrhythmia decreased significantly, thus yielding satisfactory early and midterm results after this procedure.

	References

Top Abstract Introduction Patients and Methods Surgical Procedure Results Discussion References

 

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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Lei Li Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Wu, Q. Articles by Xue, H. Search for Related Content PubMed Articles by Wu, Q. Articles by Xue, H. Related Collections Congenital - cyanotic Valve disease Related Article