Ann Thorac Surg 2004;77:470-476
© 2004 The Society of Thoracic Surgeons
Original article: cardiovascular
A new procedure for Ebstein's anomaly
Qingyu Wu, MD*a,
Zhixiong Huang, MDa
a Department of Cardiovascular Surgery, Cardiovascular Institute, Fu Wai Hospital, Chinese Academy of Medical Science, Peking Union Medical College, Beijing, China
Accepted for publication July 1, 2003.
* Address reprint requests to Dr Wu, Department of Cardiovascular Surgery, Cardiovascular Institute, Fu Wai Hospital, A 167 Beilishi Rd, Fuchengmenwai, Beijing 100037, China.
e-mail: wuqingyu{at}pulic.bta.net.cn
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Abstract
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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.
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Introduction
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Ebstein's anomaly refers to a condition with downward displacement of the septal and posterior leaflets of the tricuspid valve into the right ventricle, leaving an atrialized portion of the right ventricle [1]. Operations for Ebstein's anomaly include tricuspid valve repair and replacement. The long-term results of tricuspid valve replacement are not satisfactory because of anticoagulant complications concerning mechanical prosthetic valves or calcification and degeneration of bioprosthetic valves. Between December 1997 and September 2002, 34 consecutive patients with Ebstein's anomaly underwent the new procedure, involving excision of the atrialized ventricular wall, tricuspid annuloplasty, detachment of the displaced leaflets with their papillary muscles, and their reattachment to a new and appropriate position. All patients survived, the repaired tricuspid valve functioned well, and the right heart function improved significantly. There were no significant postoperative complications and none of the patients needed a tricuspid valve replacement.
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Material and methods
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There were 13 male and 21 female patients aged 9 months to 47 years (mean, 17 years) (Fig 1).
Patients were in the New York Heart Association functional class II (n = 14) or functional class III (n = 20). Their hemoglobin ranged from 119 to 208 g/L (mean, 142 g/L). Six patients had cyanosis during exercise and 7 had cyanosis at rest, with arterial oxygen saturation values between 73% and 91% (mean, 84%). Electrocardiograms indicated right ventricular hypertrophy in 9 patients, complete right bundle branch block in 6, atrial fibrillation in 2, and Wolff-Parkinson-White syndrome in 3; 1 patient had undergone successful catheter ablation of his abnormal pathway before the operation. The preoperative cardiothoracic ratio ranged from 0.50 to 0.75 (mean, 0.60).
The diagnosis was established by preoperative echocardiography in 33 patients. In 1 patient associated with double outlet right ventricle and pulmonary stenosis, the diagnosis was confirmed according to the operative findings. The patient was a 3-year-old girl with cyanosis. On physical examination, a grade II systolic murmur in the second to third left intercostal space and clubbing were found. Electrocardiography showed right ventricular hypertrophy and atrial enlargement. The chest radiography demonstrated decreased pulmonary vascularity and an upturned cardiac apex. Transthoracic echocardiography revealed double outlet right ventricle with subaortic ventricular septal defect, and pulmonary valve stenosis. During the operation the typical pathology of double outlet right ventricle with subaortic ventricular septal defect was found, and pulmonary stenosis included a combination of valvular and infundibular stenosis. Apart from these, Ebstein's anomaly was found. The anterior leaflet of the tricuspid valve developed well. The posterior leaflet displaced 2 cm from annulus. Half of the septal leaflet near to the antero-septal commissure was severely hypoplastic, which made this erea to be absent from valve tissue. The remainder of the septal leaflet was displaced 1.5 cm from the annulus. The size of the atrialized ventricle was moderate. Associated complicated lesions masked the Ebstein's anomaly in this patient.
According to Carpenter's classification, 2 patients were type A, 7 were type B, and 25 were type C. Tricuspid incompetence was moderate in 12 patients and severe in 22.
Surgical procedure
All operations were done under general anesthesia with total cardiopulmonary bypass using aortic and bi-caval cannulation and systemic hypothermia (28°C). The mean aortic cross-clamp time was 68 ± 21 minutes, and the mean bypass time was 109 ± 36 minutes. After the aorta was cross clamped, cardioplegia was administered through the aortic root. The right atrium was opened parallel to the atrioventricular groove, and the tricuspid valve was inspected (Figs 2–4).
The displaced posterior leaflet with some chordae tendineae and papillary muscle was detached from the annulus and ventricular wall, respectively (Figs 4, 5).
The displaced septal leaflet was treated in the same manner as well. Most of the atrialized portion of the ventricular wall was excised in the shape of a trapezoid or triangle (Figs 6–8).
In 5 patients, the big branch of the right coronary artery or the posterior descending coronary artery was present over the external surface of the atrialized ventricular wall. In this situation, the atrialized ventricular wall containing the coronary artery was left as a strip of muscle, whereas the rest of the atrialized portion was excised. The cut edges were then sutured together with continuous 4-0 or 5-0 Prolene (Ethicon, Somerville, NJ), with the preserved strip of muscle containing the coronary artery positioned underneath the wall of the ventricular chamber. Meanwhile the tricuspid annulus was plicated (annuloplasty) by running a suture from the anteroposterior commissure to the postero-septal commissure (Fig 9).
The resulting annulus size was calibrated with a valvular probe with measurements corresponding to the predicted tricuspid valve size based on the patient's body surface area, thereby avoiding stenosis. In 18 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 (Fig 10).
In 8 patients the septal leaflet was severely hypoplastic, appearing like tiny, membranous tissue remnants of the original leaflet. For these patients, a piece of freshly prepared autologous pericardium was fashioned as the "new septal leaflet" by suturing one end of the pericardium to a position just below the natural annulus (Fig 11).
Meanwhile a chordae from the anterior leaflet was transferred and sutured to the free edge of this piece of pericardium. The membranous remnants of the hypoplastic septal leaflet, the chordae from the posterior leaflet, and even a piece of pericardial tissue also can be used to fashion a new chordae. There were abnormal muscular bands attaching the anterior leaflet to the infundibulum in 3 patients. Some of the chordae of the anterior leaflet even arose from these abnormal muscular bands narrowing the outflow tract of the right ventricle. These muscle bands were resected with satisfactory relief of the obstruction. Associated procedures included atrial septal defect repair in 7 patients, closure of patent foramen ovale in 4, ventricular septal defect repair in 2, closure of patent ductus arteriosus plus ventricular septal defect repair in 2, closure of patent ductus arteriosus plus relief of right ventricular outflow tract obstruction in 1, Rastelli operation in 1, and bi-directional Glenn shunt in 1. The patient associated bi-directional Glenn shunt was a 9-year-old girl. She had severe downward displacement of the septal leaflet and posterior leaflet of the tricuspid valve, along with a huge atrialized ventricle (3.5 cm x 4 cm), a very hypoplastic right ventricle, and an atrial septal defect. Considering that the function of the right ventricle may be too poor and because of the high central venous pressure (19 mm Hg), the decision to proceed with an off-pump bi-directional cavopulmonary anastomosis was made.
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Fig 2. The anatomic characteristics of Ebstein's anomaly: downward displacement of septal and posterior leaflets of tricuspid valve, atrialized chamber, and dilatation of annulus of tricuspid valve.
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Fig 3. The displaced posterior and septal leaflets with their chordae tendineae and papillary muscle are detached from the annulus and ventricular wall, respectively.
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Fig 4. The septal leaflet was hypoplastic and displaced downward. The posterior leaflet was also hypoplastic and displaced downward to the apex of the right ventricle (not shown). The atrialized ventricle was huge.
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Fig 5. The displaced posterior and septal leaflets with their chordae tendineae and papillary muscle are detached from the annulus and ventricular wall, respectively.
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Fig 9. The tricuspid annuloplasty is completed, and the detached septal and posterior leaflets are reattached to a position just below the natural annulus.
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Fig 11. If the septal leaflet is severely hypoplastic, a piece of freshly prepared autologous pericardium is fashioned as a "new septal leaflet" by suturing one end of the pericardium to a position just below the natural annulus.
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An intraoperative transesophageal echocardiogram was carried out to assess the tricuspid valve and ventricular function after the repair.
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Results
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There were neither early deaths nor significant postoperative complications. No patient had transient or permanent third degree atrio-ventricular block and none needed tricuspid valve replacement. Thirty-two 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 29 patients and was mild in 5. There was no tricuspid valve stenosis either. All these findings were confirmed on follow-up echocardiography 7 days postoperatively, before the patient's discharge. Postoperative reduction in heart size was considerable; the cardiothoracic index on chest roentgenogram had significantly decreased (mean, 0.60 ± 0.07 preoperatively vs 0.53 ± 0.04 postoperatively, p < 0.05). Echocardiography before discharge showed right ventricle cavity dimension was remarkably reduced (mean antero-posterior diameter, 41.0 ± 8.8 preoperatively vs 22.4 ± 4.63 postoperatively, p < 0.01), the atrialized chamber had vanished, and the three leaflets of the tricuspid valve were at the level nearing the natural annulus (Figs 12–15).
After surgery, all patients were in New York Heart Association functional class I and their functional capacity had improved from a preoperative mean of 2.6 to a postoperative value of 1.0 (p < 0.05). Improvement in New York Heart Association functional level was achieved by one class in 14 patients and by two classes in 20 patients.
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Fig 12. Preoperative echocardiography shows downward displacement of anterior and septal leaflets of tricuspid valve. (AL = anterior leaflet; MV = mitral valve; RA = right atrium; RV = right ventricle; SL = septal leaflet.)
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Fig 13. Preoperative echocardiography demonstrates downward displacement of the posterior leaflet of the tricuspid valve. (PL = posterior leaflet; RA = right atrium; RV = right ventricle.)
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Fig 14. Postoperative echocardiography shows that the anterior and posterior leaflets are near the level of the natural annulus; the atrialized ventricle vanished. (AL = anterior leaflet; PL = posterior leaflet; RA = right atrium; RV = right ventricle.)
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Fig 15. Postoperative echocardiography: anterior and septal leaflets are near the level of the natural annulus; the atrialized ventricle vanished. (AL = anterior leaflet; MV = mitral valve; RA = right atrium; RV = right ventricle; SL = septal leaflet.)
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Follow-up in all 34 patients ranged from 1 to 55 months (mean, 25 months). There were 9 patients with more than 3 years of follow-up. The exercise tolerance of all 34 patients improved to normal, with 32 patients in sinus rhythm. At last follow-up echocardiograph, the tricuspid incompetence was still absent in 28 patients, mild in 3, and moderate in 3.
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Comment
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The new procedure presented in this article for the correction of Ebstein's anomaly was successfully applied in 34 consecutive patients. Their excellent early and midterm results allow extension of the scope of repair, even for patients previously deemed to undergo tricuspid valve replacement.
It is well known that Ebstein's 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.
The new technique of treating Ebstein's anomaly differs from previous techniques [2–8] in some important ways:
- The atrialized ventricular wall is excised longitudinally. Therefore, the right ventricular geometry is restored, the atrialized ventricular chamber is completely obliterated, and the load of the right ventricle is alleviated. In patients with a large atrialized chamber, performing a transverse plication or longitudinal plication can not completely obliterate the abnormal area, and this may even cause undue tension leading to suture tear. The complete longitudinal excision of the atrialized ventricular wall performed in this series of patients avoids this complication. After surgery, the right ventricle cavity dimensions are restored to near normal, which is beneficial for the recovery of both right ventricular and left ventricular function.
- The displaced leaflets with their subvalvular structures are detached from their abnormal positions and reattached to a position near the true tricuspid annulus. This technique makes "the new leaflets" function better in term of their competence and durability. Previous techniques of suspending the displaced leaflets do not move the displaced leaflets to the normal position, thus the distance from the displaced leaflets to the apex of the right ventricle does not change.
- There is less likelihood for the tricuspid annulus to enlarge after the annuloplasty because of the effective size-reduction of the tricuspid annulus and the restoration of the valve leaflets to a near normal position, leading to competence of valve function and eventual decrease of the right atrial and ventricular pressures.
It is important to avoid injury to the atrio-ventricular bundle during annuloplasty and leaflet reattachment. In this series of patients, none had transient or permanent third degree atrio-ventricular block. Our experience is to place the suture just below the position of the atrio-ventricular bundle.
The indication of the new procedure for Ebstein's anomaly is the same as that of previous procedures. Most patients with Ebstein's anomaly can be free from tricuspid valve replacement, except those very few patients with very hypoplastic anterior leaflet and severe dysfunction of the right ventricle who may need to have a bidirectional Glenn shunt plus a tricuspid valve repair [9].
Because of complete excision of the atrialized ventricular wall, a tri-leaflet mechanism covering the entire orifice of the tricuspid valve and the restoration of the tricuspid annulus to its appropriate position and size, the tricuspid valve competence is achieved. The incidence of right ventricle dysfunction and arrhythmia decreased significantly, thus attaining satisfactory early and midterm results after this procedure.
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References
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- Schmidt-Habelmann P., Meinser H., Struck E., et al. Results of valvuloplasty for Ebstein's anomaly. J Thorac Cardiovasc Surg 1981;29:155-157.
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Abstract
Introduction
