HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Abstract 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 Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Norbert Augustin Hans Meisner Fritz Sebening Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Augustin, N. Articles by Sebening, F. Search for Related Content PubMed PubMed Citation Articles by Augustin, N. Articles by Sebening, F.

Ann Thorac Surg 1997;63:1650-1656
© 1997 The Society of Thoracic Surgeons

---

Original Article: Cardiovascular

Results After Surgical Repair of Ebstein's Anomaly

Department of Cardiovascular Surgery, German Heart Center Munich, Munich, Germany

Accepted for publication December 12, 1996.

	Abstract

Top Footnotes Abstract Introduction Material and Methods Operative Technique Results Intraoperative Echocardiography Follow-up Comment References

 
Background. Ebstein's anomaly of the tricuspid valve is a complex malformation. Various operations have been undertaken with varying results. Because valve replacement yielded poor results, surgical treatment has focused on valvuloplasties.

Methods. Between April 1974 and February 1995, 60 patients with Ebstein's anomaly underwent surgical repair. Age ranged from 5 months to 54 years. In 56 patients (93.3%), tricuspid valvuloplasty was feasible, mainly by creating a monocusp valve with the single-stitch technique. The other 4 patients had valve replacement with a bioprosthesis. Six reoperations were necessary (10.0%): four valve replacements and two repeat valvuloplasties.

Results. There were two hospital deaths (3.3%) and a late mortality rate of 10.0% (6 patients). Forty-nine (94.2%) of 52 survivors were followed for 5 months to 18.6 years (median follow-up, 5.0 years; mean follow-up, 6.9 years). The actuarial survival rate (Kaplan-Meier) was 96.5% ± 2.4% at 1 year and 83.3% ± 5.6% at 18 years. At follow-up evaluation, nearly all patients showed substantial improvement (93.9% were in functional class I or II) compared with their preoperative status. Doppler echocardiographic studies demonstrated good tricuspid valve function in most patients.

Conclusions. Valvuloplasty using the single-stitch technique is a rewarding operation. It yields good long-term results with substantial improvement in functional performance and clinical status.

	Introduction

Top Footnotes Abstract Introduction Material and Methods Operative Technique Results Intraoperative Echocardiography Follow-up Comment References

 
See also page 1656.

Ebstein's anomaly of the tricuspid valve is an unusual congenital cardiac lesion with an extraordinarily variable natural history. The clinical manifestation can become evident at any age, depending on the severity of the abnormality. If the malformation is distinctly developed, neonatal or even intrauterine death can occur [1, 2]. In contrast, patients with a mildly deformed tricuspid valve can remain asymptomatic until late in life [3].

Until the middle of the 1970s, valve replacement was the standard treatment of Ebstein's anomaly but was associated with serious complications and high mortality [4–10]. The results were substantially improved only with the development of various techniques of valve repair [10–14]. This report presents a 21 year experience with surgical therapy for Ebstein's anomaly.

	Material and Methods

Top Footnotes Abstract Introduction Material and Methods Operative Technique Results Intraoperative Echocardiography Follow-up Comment References

 
Patient Population
From April 1974 to February 1995, 60 consecutive patients with Ebstein's anomaly underwent operation. The diagnosis was based on transthoracic echocardiography and cardiac catheterization. Indications for operation included New York Heart Association functional class III or IV, paradoxical emboli, increased cyanosis, progressive arrhythmias, worsening tricuspid regurgitation, and right atrial enlargement. Age at operation ranged from 5 months to 54 years (median age, 17.5 years; mean age, 20.0 years). Most of the patients were 11 through 20 years of age (Fig. 1). Preoperatively, the hematocrit values ranged from 38% to 73%. Cardiothoracic ratios were calculated to be between 0.46 and 0.89. Concomitant cardiac lesions are shown in Table 1.

View larger version (43K): [in this window] [in a new window]   Fig 1. . Age distribution at time of operation of 60 patients with Ebstein's anomaly.

	Operative Technique

Top Footnotes Abstract Introduction Material and Methods Operative Technique Results Intraoperative Echocardiography Follow-up Comment References

View larger version (51K): [in this window] [in a new window]   Fig 2. . (A) Anatomic morphology of Ebstein's anomaly. (B) Single-stitch technique: mattress suture is placed at point A; (C) point A is brought to point B after suture is tightened; and (D) anteroinferior commissure and atrial septal defect (ASD) are closed. Valvuloplasty is completed. (ANT = anterior; AV = atrioventricular; CS = coronary sinus; IVC = inferior vena cava; post = posterior; RV = right ventricle; SVC = superior vena cava; TV = tricuspid valve.)

When all further procedures (eg, closure of the atrial septal defect) have been completed, the right atrium is closed. After discontinuation of cardiopulmonary bypass and venous decannulation, finger is introduced into the right atrium for direct palpation of the tricuspid valve while the heart is beating. Since April 1992, valvuloplasties have also been evaluated by intraoperative transesophageal echocardiography.

The concomitant atrial septal defect or patent foramen ovale was closed directly in 37 patients and with a patch in 7 patients.

	Results

Top Footnotes Abstract Introduction Material and Methods Operative Technique Results Intraoperative Echocardiography Follow-up Comment References

 
Valve repair was accomplished in 56 (93.3%) of the 60 consecutive patients (Table 2). In 38 patients, valvuloplasty was done by forming a monocusp valve and in 13 patients, by the Hardy operation [15]. Primary valve replacement was performed in 4 patients. Six reoperations were necessary (10.0%): four valve replacements and two repeat valvuloplasties (Table 3).

	Intraoperative Echocardiography

Top Footnotes Abstract Introduction Material and Methods Operative Technique Results Intraoperative Echocardiography Follow-up Comment References

 
Intraoperative transesophageal echocardiography was performed in the last 15 patients in the series. Before and after cardiopulmonary bypass, tricuspid regurgitation was assessed semiquantitatively by color flow Doppler mapping using the method described by Nanda [16]. After valvuloplasty, the regurgitation declined substantially in 12 patients. In 2 patients, no change was observed, and in 1 patient, increased regurgitation of mild to moderate degree became apparent. It was due mainly to rhythm disturbances, which did not persist postoperatively. The results of transesophageal echocardiography after cardiopulmonary bypass were in keeping with the postoperative transthoracic examinations in 13 of the 15 patients (Figs 3, 4).

View larger version (28K): [in this window] [in a new window]   Fig 3. . Assessment of tricuspid regurgitation by color Doppler echocardiography in 15 patients with Ebstein's anomaly. (ECC = extracorporal circulation; TEE = transesophageal echocardiography).

View larger version (48K): [in this window] [in a new window]   Fig 4. . (A) Intraoperative transesophageal echocardiogram of patient with Ebstein's anomaly. Transverse four-chamber view in diastole focusing on the right heart shows the large atrialized right ventricle (aRV) and the right atrium (RA). The three white arrows demonstrate the considerably elongated anterior tricuspid leaflet. (B) After valvuloplasty involving the formation of a monocusp valve, the systolic phase reveals good coaptation and position of the anterior leaflet (arrow).

	Follow-up

Top Footnotes Abstract Introduction Material and Methods Operative Technique Results Intraoperative Echocardiography Follow-up Comment References

View larger version (15K): [in this window] [in a new window]   Fig 5. . Actuarial survival rate (Kaplan-Meier) of 60 patients having operation for Ebstein's anomaly.

View larger version (20K): [in this window] [in a new window]   Fig 6. . Comparison of tricuspid insufficiency (TI) preoperatively and at follow-up (n = 35).

View larger version (23K): [in this window] [in a new window]   Fig 7. . Arrhythmias in 49 patients with Ebstein's anomaly. (AV = atrioventricular; WPW = Wolff-Parkinson-White; * = 2 patients required cryosurgical ablation, and 1 patient underwent catheter ablation.)

	Comment

Top Footnotes Abstract Introduction Material and Methods Operative Technique Results Intraoperative Echocardiography Follow-up Comment References

Ebstein's anomaly in the neonate entails a high mortality. Correspondingly, the results of tricuspid valve procedures as well as palliative procedures were poor [2, 9, 19]. Starnes and colleagues [20] advocated a palliative operation for critically ill infants, with a view toward a future Fontan procedure or heart transplantation. Despite this obviously successful palliative technique, surgical treatment of neonates with severe Ebstein's anomaly constitutes a crucial issue.

After the first report on surgical correction of Ebstein's anomaly with a prosthetic tricuspid valve by Barnard and Schrire [21] in 1963, valve replacement yielded a poor outcome [4–10]. Results were substantially improved only by the development of various modifications of valve repair [11–14]. In 1964, Hardy and colleagues [15] introduced a unique technique of tricuspid valve repair based on the concept of Hunter and Lillehei [22]. Hardy and co-workers emphasized the importance of "exclusion of the atrialized ventricle" by transposing the displaced tricuspid leaflets to their normal plane and reducing the dilated annulus.

Until the late 1970s, 13 patients were treated with the Hardy operation at our hospital. To improve our results, we have changed our surgical approach over a period of years inasmuch as the single-stitch technique (see Fig 2) creates a monocusp valve of the large anterior tricuspid leaflet followed by closure of the anteroinferior commissure. As we do not use a plication of the "atrialized" right ventricle, our repair differs considerably from previous techniques.

Danielson and colleagues [4, 12] prefer a transverse plication of the "atrialized" portion of the right ventricle, posterior tricuspid annuloplasty, and right atrial reduction. The anterior leaflet functions as a monocusp valve similar to our technique. This procedure was applied to 58.2% of 189 patients, with a hospital mortality rate of 6.3%, a late mortality rate of 5.3%, and a reoperation rate of 3.6%. In contrast, our valvuloplasty technique was applicable to 93.3% of patients and resulted in a lower hospital mortality rate (3.3%) but a higher late mortality rate (10.0%). Also, there is a higher incidence of reoperation (10.0%).

Chauvaud and coauthors [23] performed their "conservative" technique combining valvuloplasty and right ventricular remodeling in 98% of 59 patients. This operative procedure was first described in 1988 by that group [11], who emphasized the importance of restoring the shape and compliance of the right ventricle. They stated that this can be achieved only by a longitudinal plication, not by the transverse technique as described by Hardy [15], Danielson [12], and their associates. A similar technique without reinforcement by a prosthetic ring was performed by Quaegebeur and co-workers [13] in 10 patients.

The argument about the modifications to the plication procedure gives rise to the question whether plication of the "atrialized" ventricle is necessary at all. Despite various comments in the literature, there is no evidence that this procedure is beneficial to right ventricular performance, particularly in view of long-term results. In our experience, the most important component of repair in Ebstein's anomaly is the creation of a competent tricuspid valve. Whenever possible, a monocusp valve is created using the single-stitch technique. At several recent reoperations, we observed enormous dilatation of the atrioventricular annulus adjacent to the anterior leaflet with a tear in the monocusp valve causing severe tricuspid regurgitation. Therefore, repeat valvuloplasties were performed as described but with additional basal reinforcement of the anterior leaflet with a Teflon strip. Accordingly, primary reinforcement of the anterior atrioventricular annulus might be advisable in the presence of pronounced dilatation.

The analysis of the postoperative deaths revealed that all patients but 1 were near terminal (see Table 4). This underlines the importance of timely surgical intervention. It is undoubtedly true that patients who are in functional class III or IV benefit from surgical intervention. We agree with Danielson and co-workers [12] that most patients in class I or II can be managed medically. However, we believe that surgical repair is also indicated for those patients in functional class II who reveal clinical deterioration. To evaluate such "borderline" cases, closely coordinated medical examinations are indispensable.

In summary, valvuloplasty was applicable to more than 90% of our patients with Ebstein's anomaly. We advocate the creation of a monocusp valve, which yields long-term results showing substantial improvement in functional performance and clinical status. Surgical treatment is indicated for patients who are in functional class III or IV and for select patients with clinical deterioration.

	Footnotes

Top Footnotes Abstract Introduction Material and Methods Operative Technique Results Intraoperative Echocardiography Follow-up Comment References

 
Address reprint requests to Dr Augustin, Department of Cardiovascular Surgery, German Heart Center Munich, Munich, Germany (e-mail: augustin{at}dhm.mhn.de).

	References

Top Footnotes Abstract Introduction Material and Methods Operative Technique Results Intraoperative Echocardiography Follow-up Comment References

 

1. Roberson DA, Silverman NH. Ebstein's anomaly: echocardiographic and clinical features in the fetus and neonate. J Am Coll Cardiol 1989;14:1300–7.[Abstract]
2. Celermajer DS, Bull C, Till JA, et al. Ebstein's anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol 1994;23:170–6.[Abstract]
3. Mair DD. Ebstein's anomaly: natural history and management. J Am Coll Cardiol 1992;19:1047–8.[Medline]
4. Danielson GK, Maloney JD, Devloo RAE. Surgical repair of Ebstein's anomaly. Mayo Clin Proc 1979;54:185–92.[Medline]
5. McFaul RC, Davis Z, Giuliani ER, Ritter DG, Danielson G. Ebstein's malformation: surgical experience at the Mayo Clinic. J Thorac Cardiovasc Surg 1976;72:910–5.[Abstract]
6. Meisner H, Klinner W, Schmidt-Habelmann P, Sebening F. Zur Chirurgie des Morbus Ebstein. Langenbecks Arch Klin Chir 1968;320:307–21.
7. Ng R, Somerville J, Ross D. Ebstein's anomaly: late results of surgical correction. Eur J Cardiol 1979;9:39–52.[Medline]
8. Pasque M, Williams WG, Coles JG, Trusler GA, Freedom RM. Tricuspid valve replacement in children. Ann Thorac Surg 1987;44:164–8.[Abstract]
9. Watson H. Natural history of Ebstein's anomaly of tricuspid valve in childhood and adolescence: an international cooperative study of 505 cases. Br Heart J 1974;36:417–27.[Free Full Text]
10. Westaby S, Karp RB, Kirklin JW, Waldo AL, Blackstone EH. Surgical treatment in Ebstein's malformation. Ann Thorac Surg 1982;34:388–95.[Abstract]
11. Carpentier A, Chauvaud S, Macé L. A new reconstructive operation for Ebstein's anomaly of the tricuspid valve. J Thorac Cardiovasc Surg 1988;96:92–101.[Abstract]
12. Danielson GK, Driscoll DJ, Mair DD, Warnes CA, Oliver WC. Operative treatment of Ebstein's anomaly. J Thorac Cardiovasc Surg 1992;104:1195–1202.[Abstract]
13. Quaegebeur JM, Sreeram N, Fraser AG, et al. Surgery for Ebstein's anomaly: the clinical and echocardiographic evaluation of a new technique. J Am Coll Cardiol 1991;17:722–8.[Abstract]
14. Schmidt-Habelmann P, Meisner H, Struck E, Sebening F. Results of valvuloplasty for Ebstein's anomaly. Thorac Cardiovasc Surg 1981;29:155–7.[Medline]
15. Hardy KL, May IA, Webster CA, Kimball KG. Ebstein's anomaly: a functional concept and successful definitive repair. J Thorac Cardiovasc Surg 1964;48:927–40.
16. Nanda NC, ed. Doppler echocardiography. 2nd ed. Philadelphia: Lea & Febiger, 1993:148--9.
17. Ebstein W. Über einen sehr seltenen Fall von Insuffizienz der Valvula tricuspidalis, bedingt durch eine angeborene hochgradige Mißbildung derselben. Arch Anat Physiol Wiss Med 1866;33:238–54.
18. Anderson KR, Zuberbuhler JR, Anderson RH, Becker AE, Lie JT. Morphologic spectrum of Ebstein's anomaly of the heart. Mayo Clin Proc 1979;54:174–80.[Medline]
19. Gentles TL, Calder AL, Clarkson PM, Neutze JM. Predictors of long-term survival with Ebstein's anomaly of the tricuspid valve. Am J Cardiol 1992;69:377–81.[Medline]
20. Starnes VA, Pitlick PT, Bernstein D, Griffin ML, Choy M, Shumway NE. Ebstein's anomaly appearing in the neonate. A new surgical approach. J Thorac Cardiovasc Surg 1991;101:1082–7.[Abstract]
21. Barnard CN, Schrire V. Surgical correction of Ebstein's malformation with prosthetic tricuspid valve. Surgery 1963;54:302–8.
22. Hunter SW, Lillehei CW. Ebstein's malformation of the tricuspid valve: study of case, together with suggestion of a new form of surgical therapy. Dis Chest 1958;33:297–304.
23. Chauvaud S, Carpentier A, Mihaileanu S, Marino JP. Long-term results of conservative surgery for Ebstein's anomaly. Eur Heart J 1994;15(Abstract Suppl):127.

This article has been cited by other articles:

				S. Chauvaud and A. Carpentier Ebstein's anomaly: the Broussais approach MMCTS, June 26, 2008; 2008(0626): 3038. [Abstract] [Full Text] [PDF]

				M. L. Brown, J. A. Dearani, G. K. Danielson, F. Cetta, H. M. Connolly, C. A. Warnes, Z. Li, D. O. Hodge, D. J. Driscoll, and Mayo Clinic Congenital Heart Center The outcomes of operations for 539 patients with Ebstein anomaly. J. Thorac. Cardiovasc. Surg., May 1, 2008; 135(5): 1120 - 1136.e7. [Abstract] [Full Text] [PDF]

				C. J. Knott-Craig, S. P. Goldberg, E. D. Overholt, E. V. Colvin, and J. K. Kirklin Repair of Neonates and Young Infants With Ebstein's Anomaly and Related Disorders Ann. Thorac. Surg., August 1, 2007; 84(2): 587 - 593. [Abstract] [Full Text] [PDF]

				G. E. Sarris, N. M. Giannopoulos, A. J. Tsoutsinos, A. K. Chatzis, G. Kirvassilis, W. J. Brawn, J. V. Comas, A. F. Corno, D. Di Carlo, J. Fragata, et al. Results of surgery for Ebstein anomaly: A multicenter study from the European Congenital Heart Surgeons Association J. Thorac. Cardiovasc. Surg., July 1, 2006; 132(1): 50 - 57. [Abstract] [Full Text] [PDF]

				S. M. Chauvaud, A. C. Hernigou, E. R. Mousseaux, D. Sidi, and J.-L. Hebert Ventricular Volumes in Ebstein's Anomaly: X-Ray Multislice Computed Tomography Before and After Repair Ann. Thorac. Surg., April 1, 2006; 81(4): 1443 - 1449. [Abstract] [Full Text] [PDF]

				M. V. Ullmann, S. Born, C. Sebening, M. Gorenflo, H. E. Ulmer, and S. Hagl Ventricularization of the atrialized chamber: A concept of Ebstein's anomaly repair Ann. Thorac. Surg., September 1, 2004; 78(3): 918 - 924. [Abstract] [Full Text] [PDF]

				C. L. Hancock Friesen, R. Chen, J. G. Howlett, and D. B. Ross Posterior annular plication: tricuspid valve repair in Ebstein's anomaly Ann. Thorac. Surg., June 1, 2004; 77(6): 2167 - 2171. [Abstract] [Full Text] [PDF]

				J. P. A. Beerepoot and P. K. Woodard Case 71: Ebstein Anomaly Radiology, June 1, 2004; 231(3): 747 - 751. [Full Text] [PDF]

				J. M. Chen, R. S. Mosca, K. Altmann, B. F. Printz, K. Targoff, P. A. Mazzeo, and J. M. Quaegebeur Early and medium-term results for repair of Ebstein anomaly J. Thorac. Cardiovasc. Surg., April 1, 2004; 127(4): 990 - 999. [Abstract] [Full Text] [PDF]

				A. Pflaumer, A. Eicken, N. Augustin, and J. Hess Symptomatic neonates with Ebstein anomaly J. Thorac. Cardiovasc. Surg., April 1, 2004; 127(4): 1208 - 1209. [Full Text] [PDF]

				S. Chauvaud, A. Berrebi, N. d'Attellis, E. Mousseaux, A. Hernigou, and A. Carpentier Ebstein's anomaly: repair based on functional analysis Eur. J. Cardiothorac. Surg., April 1, 2003; 23(4): 525 - 531. [Abstract] [Full Text] [PDF]

				C. J. Knott-Craig, E. D. Overholt, K. E. Ward, J. M. Ringewald, S. S. Baker, and J. D. Razook Repair of Ebstein's anomaly in the symptomatic neonate: an evolution of technique with 7-year follow-up Ann. Thorac. Surg., June 1, 2002; 73(6): 1786 - 1793. [Abstract] [Full Text] [PDF]

				C. J. Knott-Craig, E. D. Overholt, K. E. Ward, and J. D. Razook Neonatal repair of Ebstein's anomaly: indications, surgical technique, and medium-term follow-up Ann. Thorac. Surg., May 1, 2000; 69(5): 1505 - 1510. [Abstract] [Full Text] [PDF]

				M. E. Brickner, L. D. Hillis, and R. A. Lange Congenital Heart Disease in Adults- Second of Two Parts N. Engl. J. Med., February 3, 2000; 342(5): 334 - 342. [Full Text] [PDF]

				C. Schreiber, A. Cook, S. Y. Ho, N. Augustin, and R. H. Anderson MORPHOLOGIC SPECTRUM OF EBSTEIN'S MALFORMATION: REVISITATION RELATIVE TO SURGICAL REPAIR J. Thorac. Cardiovasc. Surg., January 1, 1999; 117(1): 148 - 155. [Abstract] [Full Text] [PDF]

				S. M. Marianeschi, D. B. McElhinney, V. M. Reddy, N. H. Silverman, and F. L. Hanley Alternative approach to the repair of Ebstein's malformation: intracardiac repair with ventricular unloading Ann. Thorac. Surg., November 1, 1998; 66(5): 1546 - 1550. [Abstract] [Full Text] [PDF]

This Article Abstract 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 Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Norbert Augustin Hans Meisner Fritz Sebening Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Augustin, N. Articles by Sebening, F. Search for Related Content PubMed PubMed Citation Articles by Augustin, N. Articles by Sebening, F.