Ann Thorac Surg 2001;71:582-586
© 2001 The Society of Thoracic Surgeons
Original article: cardiovascular
Tricuspid valve supra-annular implantation in adult patients with Ebstein’s anomaly
Masashi Tanaka, MDa,
Toshihiro Ohata, MDa,
Sachito Fukuda, MDa,
Ikutaro Kigawa, MDa,
Yoichi Yamashita, MDa,
Yasuhiko Wanibuchi, MDa
a Division of Cardiovascular Surgery, Mitsui Memorial Hospital, Tokyo, Japan
Accepted for publication June 5, 2000.
Address reprint requests to Dr Tanaka, Division of Cardiovascular Surgery, Mitsui Memorial Hospital, 1 Kanda Izumi-cho, Chiyoda-ku, Tokyo 101-0024, Japan
e-mail: masashi{at}omiya.jichi.ac.jp
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Abstract
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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.
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Introduction
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Ebstein’s anomaly is a complex malformation that has been treated by various surgical techniques with variable results [1–3]. Although the standard surgical treatment for patients with Ebstein’s anomaly is tricuspid valve repair or replacement [3], tricuspid repair and replacement are not feasible in some cases because of anatomical factors or late problems caused by prosthetic valves and arrhythmias [4, 5]. We selected a different procedure for these patients to avoid the complications related to tricuspid annuloplasty and classical valve replacement. Tricuspid valve supra-annular implantation (TVSI) has been performed for adult patients with Ebstein’s anomaly at our hospital since 1974. The aim of the present study was to investigate the clinical efficacy and long-term results of this procedure.
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Patients and methods
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Patients
Between January 1974 and December 1998, 10 adult patients with Ebstein’s anomaly underwent TVSI at our hospital. There were 5 men and 5 women aged from 16 to 54 years (33 ± 4, mean ± standard error). The average follow-up period was 12 years and 4 months ± 5 years and 6 months (range 3 to 19 years) after surgery. The preoperative New York Heart Association functional class was III in 7 patients and IV in 3 patients. Eight patients had associated cardiac lesions, including 6 with atrial septal defect, 1 with combined mitral stenosis, regurgitation, and aortic regurgitation, and 1 with Wolff-Parkinson-White (WPW) syndrome (type B) (Table 1). Preoperative electrocardiography showed sinus rhythm in 5 patients, atrial fibrillation in 4, and complete atrio-ventricular block in 1. All patients had severe tricuspid regurgitation (TR) and right atrial enlargement on echocardiography. The preoperative left ventricular ejection fraction ranged from 50% to 82% (62 ± 12%), the right ventricular diameter ranged from 30 to 82 mm (63 ± 11 mm), and cardio-thoracic ratio (CTR) on plain chest x-ray films ranged from 61% to 78% (71 ± 6%). Seven patients had liver dysfunction due to hepatic congestion induced by severe TR, and the total billirubin ranged from 0.8 to 3.4 mg/dL (1.5 ± 0.8 mg/dL).
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Table 1. Preoperative Characteristics From Patients Who Underwent TVSI (Mitsui Memorial Hospital, From December 1974 to December 1998)
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Operative technique
After median sternotomy, cardiopulmonary bypass was established under moderate hypothermia (25°C to 28°C) using two venous cannulas. Cardiac protection was performed in an antegrade fashion with cold crystalloid cardioplegia. After the right atrium was opened and the tricuspid valve was thoroughly examined, 2-0 Ticron interrupted sutures reinforced with small hollow tubes made by expanded Polytetra-fluoroethylene (ePTFE) were placed above the annulus. Several sutures are placed at the wall of right atrium to avoid Koch’s triangle and care was taken to not injure the atrioventricular node or the bundle of His. After measuring the diameter of the tricuspid annulus, a bioprosthesis was implanted at the supra-annular position, while preserving the native tricuspid valve (Fig 1). Hancock (Medtronic Inc, Minneapolis, MN), Carpentier-Edwards, and Carpentier-Edwards Pericardial valves (Baxter Healthcare Corp, Irvine, CA) were used. No plication, either of the atrium or the right ventricle, was performed in all patients. Closure of the right atriotomy was performed before aortic declamping. We performed four direct closures and two patch closures in the patients with associated atrial septal defects, and mitral valve replacement was done for the patient with mitral stenosis and regurgitation.
Follow-up
All patients were reevaluated by annual clinic visits, and physical exam, electrocardiography, echocardiography, and laboratory tests were performed. Echocardiography was performed every year. Anticoagulation therapy was also performed for all patients. Two patients were lost to follow-up and 2 patient died (Table 1).
Measurements
TR, right ventricular diameter (RVD), left ventricular ejection fraction (EF), cardio-thoracic ratio (CTR), New York Heart Association functional class (NYHA), and total billirubin (T-bil) were analyzed preoperatively, at discharge, 1 year after the operation, and after long-term follow-up. RTR, RVD, and EF were measured by echocardiography, and RTR was classified into five grades of severity. RVD was measured as the maximum end-diastolic diameter on M-mode echocardiography.
Statistics
The Kaplan-Meier method was used to estimate the actuarial survival rate, the freedom from structural valve deterioration rate, and the reoperation free rate at 19 years after surgery. All results are expressed as the mean ± standard deviation of the mean. Data were compared by the repeated-measures analysis of variance (ANOVA), and p less than 0.05 was regarded as statistically significant. All analyses were performed using the Statview v4.5 statistical package (Abacus Concepts Inc, Berkeley, CA).
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Results
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The aortic cross-clamping time was 69 ± 22 minutes, and the extracorporeal circulation time was 108 ± 24 minutes. No hospital mortality occurred, but 2 patients died in the late postoperative period. One patient died of low output syndrome and multiple organ failure on the 18th day after emergency repeat mitral valve replacement at 9 years after the first operation. The other died of low output syndrome on the third day after coronary artery bypass grafting and re-TVSI for coronary artery disease and severe TR due to paravalvular leakage at 17 years after the first operation. Intraoperatively, detachment of the bioprosthesis from the annulus was encountered after the passage of a Swan-Ganz catheter, but there was no evidence of valvular deterioration such as major pannus formation, cusp tears, calcification, or dissection of the valvular stent.
Residual tricuspid regurgitation
Preoperative TR was severe in all patients, but RTR improved to a trace at the time of discharge. At 1 year after the operation, mild regurgitation was encountered in 1 patient. Over the long-term, moderate to severe recurrent TR was observed in 2 patients at 7 and 9 years, respectively, after the operation (Fig 2). The cause of recurrent TR was paravalvular leakage induced by the passage of a Swan-Ganz catheter.
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Fig 2. Changes in residual tricuspid regurgitation before and after the operation. *Paravalvular leakage: this patient resulted in operative death during coronary artery bypass grafting, which was performed 17 years after the first operation. **Paravalvular leakage: it appeared at 7 years after the operation. We did not select a surgical intervention because he had no symptoms.
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Right ventricular diameter
Marked right ventricular enlargement was detected in all patients preoperatively by echocardiography. However, the RVD at discharge was significantly smaller than preoperatively (63 ± 11 vs 37 ± 9 mm, p < 0.01), and no reenlargement was observed during the postoperative period (1 year and long-term: 40 ± 7 and 33 ± 9 mm, respectively) (Fig 3).
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Fig 3. Changes in diameter of right ventricle before and after the operation. Vertical bars indicate standard errors.
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Left ventricular ejection fraction
There was no significant change of EF postoperatively (preoperative, at discharge, 1 year, and long term: 62 ± 12%, 70 ± 11%, 62 ± 15%, and 65 ± 9%, respectively).
Cardiothoracic ratio
The cardiothoracic ratio was significantly smaller at discharge than preoperatively (71 ± 6% vs 65 ± 5%, p < 0.001), and no reenlargement was observed during the postoperative period (1 year and long term: 64 ± 6% and 64 ± 7%, respectively).
Total bilirubin level
Total bilirubin was significantly lower at discharge than preoperatively (1.5 ± 0.8 vs 0.8 ± 0.3 mg/dL, p < 0.001), and there was no significant change subsequently (1 year and long term: 0.9 ± 0.5 and 0.8 ± 0.4 mg/dL, respectively).
New York Heart Association functional class
Seven patients were in class I and 3 were in class II at discharge. Five patients were in class I, 4 in class II, and 1 in class III at long-term follow-up.
Actuarial survival, structural valve deterioration, and reoperation
The actuarial survival rate at 19 years was 76 ± 15% (Fig 4). The freedom from structural valve deterioration rate and the reoperation rate at 19 years were both 100%. No cusp tears or calcification were detected on echocardiography.
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Fig 4. The actuarial survival rate, freedom from structural valve deterioration, and reoperation at 19 years after the operation. Solid line = actuarial survival rate; dashed line = freedom from structural valve deterioration and reoperation rate.
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Arrhythmia
Although ventricular tachycardia was observed in 2 patients postoperatively, one was converted to sinus (Table 2) rhythm after open heart massage and the other after direct cardioversion. Atrial flutter was observed in 1 patient postoperatively, and was abolished by direct cardioversion. Sick sinus syndrome was observed in 2 patients at 1 and 10 years, respectively, after the operation, and implantation of permanent pacemakers was needed. Neither atrioventricular block nor fatal complications related to the cardiac conduction system occurred in this series.
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Comment
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Ebstein’s anomaly was originally described by William Ebstein in 1866 [1]. In 1949, Tourniaire stated that Ebstein’s anomaly occurs as a result of defective attachment of the tricuspid valve leaflets during formation of the atrio-ventricular valve from the endocardial cushions at 5 to 6 weeks of gestation. This anomaly accounts for 0.1% to 1.0% of the congenital heart disease and there have been reports of familial clustering [2, 3].
The standard surgical treatment for Ebstein’s anomaly is tricuspid valve plication and replacement [3]. Although many plication methods have been reported [4–9], surgical correction is difficult and fatal complications associated with arrhythmia are common, because valve deformity is severe in this disease [3, 10, 11]. On the other hand, tricuspid valve replacement with a mechanical valve can prevent RTR completely, but postoperative atrio-ventricular block caused by injury to the conduction system and thromboembolism due to the low pressure gradient remain as problems [3, 12, 13]. Pannus formation, endocarditis, and structural valve deterioration are the main late complications of TVR with a bioprosthesis. To overcome these complications, we performed TVSI in adult patients with Ebstein’s anomaly and severe tricuspid regurgitation. As a result, regurgitation was improved in most cases over the short term. No transvalvular leakage due to structural deterioration occurred over the long term, although 2 patients developed paravalvular leakage. The durability of the bioprosthesis at the supra-annular tricuspid position was also demonstrated to be excellent and was acceptable for our long-term results.
Nakano and colleagues reported that freedom from structural valve deterioration of the Carpentier-Edwards pericardial valve in the tricuspid position was 100% and structural dysfunction was 73 ± 21% at 9 years [14], and Ohata reported that freedom from structural valve deterioration was 100% and reoperation was 88 ± 9.4% at 14 years [15]. On the other hand, Kawachi and associates reported that freedom from structural deterioration of Hancock’s porcine valve in the tricuspid position was 94 ± 6% at 10 years [16]. Although it is not controversial whether bioprosthesis should be used in the tricuspid position rather than mechanical valve, its long-term results are thought to be excellent. These data coincide with our data. This excellent results of bioprosthesis may support the good long-term outcome of this procedure.
The other main problem with surgical treatment for Ebstein’s anomaly is complications and mortality due to fatal arrhythmias. According to some reports, fatal arrhythmias occur in 7% to 9% of patients after plication and in 6% to 18% of patients after valve replacement [6, 10, 17]. The incidence of death related to arrhythmia is relatively high. We theorized that complications related to arrhythmia occurred as a result of surgical manipulation around Koch’s triangle. In this series, there were 2 patients with sick sinus syndrome at long-term follow-up, but arrhythmias were transient during the early postoperative period. No atrio-ventricular block was observed, and there was no mortality related to arrhythmia.
Critical issues at long-term follow-up include the weakness of the right atrium wall at the implantation site, bioprosthesis-related thromboembolism in the pulmonary circulation, and the durability of bioprosthesis in the tricuspid position. Our data indicate that this procedure largely resolves these problems. On the other hand, this study includes only 10 cases and this procedure might have limited surgical indication among all patients of Ebstein’s anomaly. However, TVSI might be recommended as a selective procedure for patients with adult Ebstein’s anomaly because of its good long-term results.
By performing TVSI, we could avoid complications related to arrhythmia and RTR over the long-term. RVD was also reduced in this series. In summary, we examined the outcome of TVSI in 10 adult patients with Ebstein’s anomaly. In conclusion, these results demonstrate that TVSI is a very simple and useful procedure in adult patients with Ebstein’s anomaly in spite of their small number because of the few complications related to fatal arrhythmia and minimal RTR.
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References
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Abstract
Introduction
