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Ann Thorac Surg 2004;77:2167-2171
© 2004 The Society of Thoracic Surgeons

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Original article: cardiovascular

Posterior annular plication: tricuspid valve repair in Ebstein's anomaly

^a Division of Cardiac Surgery, Halifax, Nova Scotia, Canada
^b Division of Pediatric Cardiology, IWK Health Centre, Dalhousie University, Halifax, Nova Scotia, Canada
^c Division of Cardiology, QEII Health Sciences Centre, Halifax, Nova Scotia, Canada

Accepted for publication November 25, 2003.

^* Address reprint requests to Dr Hancock Friesen, Suite 2269, New Halifax Infirmary, 1796 Summer St, Halifax NS B3H 3A7, Canada
e-mail: camillehf{at}hotmail.com

	Abstract

Top Abstract Introduction Patients and methods Results Comment Acknowledgments References

 
BACKGROUND: Nonneonatal Ebstein's anomaly is a rare congenital anomaly for which numerous operative techniques have been described to repair the aberrant tricuspid valve, exclude the atrialized right ventricle, and address right heart dysfunction. We reviewed short-term outcomes in surgical patients treated by a simplified operative technique.

METHODS: Our operative approach to Ebstein's anomaly is approximation of the anteroposterior commissure to the remnant septal leaflet with closure of the cul-de-sac longitudinally. Plication of the atrialized right ventricle (1 patient) and a bidirectional cavopulmonary connection (2 patients) were performed only if necessary. All patients were followed postoperatively by their cardiac surgeon and cardiologist.

RESULTS: Seven patients with a mean age of 39 years (range, 3.6 to 63.8 years) underwent repair. Preoperatively all patients had 4+ tricuspid valve regurgitation and were New York Heart Association class III. Mean postoperative hospital stay was 7 ± 2 days (range, 4 to 11 days). Mean follow-up is 42 ± 18 months (range, 7 to 58 months). At last follow-up 5 patients are New York Heart Association class I and average tricuspid valve regurgitation is mild.

CONCLUSIONS: Plication of the posterior annulus without plication of the atrialized right ventricle, resiting the tricuspid valve, or performing prophylactic cavopulmonary connection appears to be a reasonable operative approach to nonneonatal Ebstein's anomaly. Long-term follow-up of this cohort is necessary to determine the durability of such a surgical approach.

	Introduction

Top Abstract Introduction Patients and methods Results Comment Acknowledgments References

 
Ebstein's anomaly (EA) is a heterogeneous disease process that may present neonatally with severe right ventricle (RV) and tricuspid valve dysfunction or may be latent, presenting after many years of mild tricuspid regurgitation (TR) and progressive annular dilation. Valve repair, valve replacement, one-and-one-half ventricle repair, single ventricle repair, and transplantation are surgical modalities that have been used for progressively more severe forms of the disease. Patients with more severe disease typically present at younger ages and require more aggressive surgical approaches to achieve good functional outcomes. Patients presenting later in life (childhood, adolescence, or adulthood) typically have milder disease, resulting in slower progression of tricuspid valve regurgitation and RV dysfunction. Repair of the tricuspid valve is a technique in the surgical armamentarium reserved for milder disease forms.

A number of repair techniques have been described in the literature. Most of the techniques require mobilization of the dominant leaflet both circumferentially, around the aperture of the valve to cover the areas with deficient leaflet, and vertically to the true tricuspid annulus. Many of the repairs also describe plication (either horizontal or vertical) of the atrialized RV (aRV). Hetzer and colleagues [1] described a more simple repair in which the posterior annulus is obliterated using horizontal mattress sutures with the remaining mobile leaflet transposed to the true tricuspid annulus without any plication of the aRV.

In this series an even more simplified Ebstein's valve-sparing operation was performed in which the posterior annular plication was performed without relocating the valve to the true tricuspid annulus and without plication of the aRV (Fig 1). Cavopulmonary connection (CPC) was used selectively when there was hemodynamic or echocardiographic evidence of increased right atrial pressure (or central venous pressure) as a result of either iatrogenic tricuspid stenosis or RV diastolic dysfunction.

View larger version (86K): [in this window] [in a new window]   Fig 1. Posterior annular plication operative technique: the anteroposterior commissure (A) and posteroseptal commissure (A1) are approximated at the level of the displaced annulus, closing the cul-de-sac leaving the atrialized right ventricle and the true tricuspid annulus on the atrial side of the repair. (Ant = anterior; Post = posterior; RV = right ventricle.)

	Patients and methods

Top Abstract Introduction Patients and methods Results Comment Acknowledgments References

Tricuspid valve regurgitation was assessed by transthoracic echocardiography as previously described [2]. The severity of TR was graded as 0 = none, 1 = trace or trivial, 2 = mild, 3 = moderate, and 4 = severe.

All patients were Carpentier class B by transthoracic echocardiographic assessment [3]. Operative intervention was scheduled when the patients became severely symptomatic with reduced exercise tolerance (New York Heart Association class III), profound cyanosis, or supraventricular tachycardia.

Intraoperative preparation included central venous access, arterial catheter, and transesophageal echocardiography. A conventional median sternotomy was performed, and patients were placed on cardiopulmonary bypass using routine aortic arterial cannulation and bicaval venous cannulation. The superior vena cava cannula was positioned extremely cephalad in the superior vena cava or in the innominate vein in the event that a CPC was required. Antegrade high-potassium (44 mmol/L) blood cardioplegia was used to maintain the heart in an arrested state after the aortic cross-clamp was applied and refreshed at 20-minute intervals throughout the cross-clamp period. The patient's core temperature was cooled to 32°C.

In all cases, the anatomy was as classically described with ventricular displacement of the septal and posterior leaflets and a large, parachutelike anterior leaflet (Table 1). All patients had a significant proportion of the RV "atrialized." By use of a 4-0 felt-pledgetted Prolene suture (Ethicon Inc, Somerville, NJ), the annulus at the anterior-posterior commissure was approximated to the annulus at the posterior-septal commissure (Figs 1, 2). The exact point at which the tricuspid annulus was approximated to itself was determined by sizing the results and orifice with a Hegar dilator on the basis of the patient's size. An appropriate orifice size was determined by indexing to the body surface area of the patient [4]. If the chosen point of annular approximation was not at a commissure, the anterior-posterior commissure was approximated to the annulus midleaflet (either midposterior leaflet or midseptal leaflet). No attempt was made to plicate the atrialized segment of the RV except for that portion which formed a cul-de-sac from the valve hinge line extending posterior to the free wall of the RV. This cul-de-sac was closed with the running Prolene suture that had been used to approximate the septal and posterior leaflets (Figs 2, 3), closing the potential communication between the aRV and the RV. The tricuspid orifice remained displaced inferiorly at the level of the "pseudoannulus" without any attempt to relocate the valve apparatus upward to the true annulus. The repair was tested passively with cold saline irrigation to ensure tricuspid valve competence with the RV loaded. When we were satisfied with the repair, associated atrial septal defects were closed, and the patient was warmed and weaned from cardiopulmonary bypass.

View larger version (116K): [in this window] [in a new window]   Fig 2. Ebstein's repair: silk traction sutures are placed at the edges of the atriotomy. The forceps point to the tricuspid orifice at the anteroseptal commissure, and the suction tip is in the cul-de-sac of the atrialized right ventricle after the initial pledgetted annuloplasty suture has been placed.

View larger version (118K): [in this window] [in a new window]   Fig 3. Ebstein's repair: the atrialized right ventricle is closed lateral to the annuloplasty with a running suture all the way to the free wall of the ventricle.

	Results

Top Abstract Introduction Patients and methods Results Comment Acknowledgments References

 
Six patients were female and 1 was male. The average age at operation was 39 years (range, 3.6 to 63.8 years; Table 1). Preoperatively, all patients had 4+ TR and all were New York Heart Association functional class III. All the patients in the series underwent a posterior annular plication. One patient (patient 5) required the placement of a Carpentier Edwards tricuspid annuloplasty ring; when following our standard repair, testing of the valve revealed improvement in the regurgitation but residual central insufficiency, which disappeared with placement of the ring annuloplasty.

Two patients (patients 1 and 6) had bidirectional CPC created at the time of the operation. Patient 1 was operated on early in the series, and the CPC was constructed because there was uncertainty about the adequacy of RV function. Subsequent to this patient there was selective use of CPC as described in the Methods. Patient 6 was a cyanotic 3-year-old child with a more severe morphologic variant of EA. This child became increasingly unstable in the intensive care unit several hours after the standard posterior annular plication. Echocardiography demonstrated persistent tricuspid valve insufficiency with severe distension of the aRV and compression of the left ventricle. The repair was revised by greatly downsizing the valve until it was stenotic (13 mm diameter), longitudinally plicating the RV, and performing a CPC. The child was weaned from bypass with a central venous pressure of 13 mm Hg and made an uneventful recovery. She is now asymptomatic.

The postoperative length of stay ranged from 4 to 11 days (mean, 7 ± 2 days) with no early mortality. Inotropic requirement was modest, with low-dose dopamine (5 µg · kg^–1 · min^–1) used for less than 48 hours in 3 of 7 patients. There were no instances of significant right heart dysfunction, and specifically no instances of prolonged pleural effusion or abdominal ascites. Patient 6 was readmitted for a pericardial effusion.

Mean follow-up is 42 ± 18 months (range, 7 to 58 months). There was one intermediate-term death. Patient 4 died 16 months after the operation. Her most recent clinic visit before death (9 months after surgery) indicated that she was symptomatically much improved. Her transthoracic echocardiogram done at the time revealed mild TR with an eccentric jet. Her RV was hypokinetic, and her left ventricular function was normal. There was no autopsy performed. All intermediate-term survivors but one are symptomatically improved. Patient 7 has persistent dyspnea and remains in New York Heart Association class III, but this is confounded by steroid-dependent rheumatoid arthritis and a previous lung resection for carcinoma. No patient has clinical evidence of right heart failure. Follow-up transthoracic echocardiography has been obtained in all patients with nil or trace TR in 2 patients, mild in 3, and moderate in 2. Despite aggressive annular narrowing there was no incidence of tricuspid valve stenosis as assessed by transthoracic echocardiography except in patient 6 as previously described.

Four patients were in normal sinus rhythm at follow-up (patients 1, 3, 5, and 6), 1 patient was in second-degree atrioventricular block after radiofrequency ablation of an accessory conduction pathway (Wolff-Parkinson-White, patient 2), and there was no electrocardiogram performed at most recent follow-up in the last patient (patient 7).

	Comment

Top Abstract Introduction Patients and methods Results Comment Acknowledgments References

 
We report a unique repair taking a number of surgical techniques previously described and assembling them into a simplified procedure. The salient features of this repair include (1) no attempt to restore the tricuspid valve apparatus to the true annulus so the aRV remains a part of the right atrium, similar to Vargas and colleagues [5] but without aRV plication; (2) no plication of the aRV like Hetzer and associates [1] and Augustin and colleagues [6]; and (3) selective and infrequent use of CPC, plication of aRV, and prosthetic annuloplasty ring similar to the series of Chauvaud and coworkers [7, 8].

Plication of the posterior annulus without addressing the aRV appears to be a reasonable operative strategy in moderate EA (Carpentier types B and C) as there is no evidence that surgical remodeling of the RV improves its performance [9]. Also, excluding the aRV to the atrial side of the repair (as Hetzer and coworkers describe [1]) has not been associated with progressive dilation of the aRV, RV dysfunction, or impairment of left ventricular function. Routine longitudinal plication of the ventricle may compromise macrocoronary or microcoronary circulation, predisposing the patient to ventricular dysrhythmia, which has been an important cause of death in some series [9, 10]. Even though we do not advocate routine aRV plication, there are cases of EA, such as patient 6, in which plication may be necessary. This child had a more severe form of EA requiring operation at 3 years of life for cyanosis and polycythemia and had very limited anterior leaflet tissue with which to repair the valve. Plication was performed only after she failed the original repair (see Results for description), for the purpose of reducing the diameter of the aRV, further narrowing the tricuspid orifice and decreasing the degree of TR.

Relocating the tricuspid valve to a more anatomic location (ie, to the true annulus) does not appear to be necessary for adequate valve function in the short term. Aggressive mobilization of the anterior tricuspid leaflet is unnecessary in all but the most severe forms of EA (Carpentier class D). Most of the repairs described transform the tricuspid valve into a functionally monocuspid structure. Mobilizing the leaflets clockwise and relocating the hinge line to the true tricuspid annulus is time-consuming with little benefit. The simple annuloplasty we describe achieves the same end result of the more complex valve repairs with the anterior tricuspid leaflet as the principle mobile component providing competence at the tricuspid valve orifice. More aggressive leaflet mobilization to create a trileaflet or monocuspid repair [11], or transecting and reimplanting the papillary muscles [12], is undesirable because of the increased suture lines in muscle that are prone to dehiscence or scarring.

Although CPC has been used routinely in some EA series, it serves no purpose if there is no evidence of restricted flow through the right atrioventricular valve or reduced systolic RV function. We propose bidirectional CPC be reserved for the patients who have impaired RV function and either native or iatrogenic tricuspid stenosis indicated by central venous pressures higher than mean pulmonary artery pressures in conjunction with transthoracic echocardiographic data consistent with significant tricuspid valve stenosis or RV dysfunction at the completion of the repair. This should be a small minority of patients inasmuch as no series of tricuspid valve repair in EA reports clinically significant tricuspid stenosis.

Candidates for this repair are clearly a small subset of the EA population, with milder pathologic involvement of the tricuspid valve apparatus, and sufficient anterior leaflet tissue to tolerate a unicuspid repair. This selection bias is reflected in the average age of our study patients (39 years). The only patient who experienced postrepair tricuspid stenosis was the youngest patient in the series (3.6 years old), who also had the most anatomically deformed tricuspid valve. Infants, who have proportionally less annular dilation than older patients (who have suffered from chronic TR), may in fact be at higher risk for tricuspid stenosis with this repair, and so the repair is advocated only for anatomically milder and likely older patients.

In some patients tricuspid valve replacement may be indicated. The role of tricuspid valve replacement versus a one-and-one-half ventricle repair is not clear from a review of the literature. Anecdotally, the 2 patients in our series who had CPC constructed at the time of repair are both asymptomatic with mild TR and good RV function at last follow-up. As always, in a child in whom growth potential is a consideration, it is preferable to pursue a repair strategy that leaves the patient with native valve tissue rather than a prosthetic valve with either thromboembolic or bleeding risks (mechanical) or the risk of structural deterioration (tissue bioprosthesis) and of outgrowth stenosis (any prosthetic valve) [13].

The majority of our patients have residual TR graded by transthoracic echocardiography as mild or less (n = 5). Of interest, the 2 patients who have moderate residual TR had intraoperative reports of good-quality anterior leaflets and would have been predicted to be the most suitable for this repair (Table 1). There are no other reported characteristics of these tricuspid valves that would explain the degree of residual TR.

The one death in our series is impossible to categorize given that no autopsy was performed and that the patient had not been reviewed in our Adult Congenital Heart Clinic for several months before her decease and, at the time of her most recent examination, was clinically well in normal sinus rhythm, with only mild TR. It is possible that this represents an arrhythmic death, or accelerated RV failure culminating in death.

The longevity of the repair remains unknown in this and other newer tricuspid valve-sparing innovations for nonneonatal EA of mild-to-moderate severity. Ebstein's anomaly is a complex constellation of pathologic processes involving both sides of the heart, and thus no single operative approach will ever prove satisfactory as a treatment for all patients. However, we believe adding the operative approach described here to the surgical armamentarium will improve the likelihood of achieving a successful tricuspid valve-sparing operation for EA.

	Acknowledgments

Top Abstract Introduction Patients and methods Results Comment Acknowledgments References

 
The authors acknowledge and thank Emily Flynn Macintosh for her assistance with the artwork for the manuscript.

	References

Top Abstract Introduction Patients and methods Results Comment Acknowledgments References

 

1. Hetzer R., Nagdyman M., Ewert P., et al. A modified repair technique for tricuspid incompetence in Ebstein's anomaly. J Thorac Cardiovasc Surg 1998;115:857-868.[Abstract/Free Full Text]
2. Geva T. Echocardiography, and Doppler ultrasound. In: Garson A.J., Bricker J.T., Fisher D.J., Neish S.R., eds. The science and practice of pediatric cardiology. Baltimore: Williams and Wilkins, 1997:789-843.
3. Carpentier A., Chauvaud S., Mace L., et al. A new reconstructive operation for Ebstein's anomaly of the tricuspid valve. J Thorac Cardiovasc Surg 1988;96:92-101.[Abstract]
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6. Augustin N., Schmidt-Habelmann P., Wottke M., Meisner H., Sebening F. Results after surgical repair of Ebstein's anomaly. Ann Thorac Surg 1997;63:1650-1656.[Abstract/Free Full Text]
7. Chauvaud S., Fuzellier J.F., Berrebi A., et al. Bi-directional cavopulmonary shunt associated with ventriculo and valvuloplasty in Ebstein's anomaly: benefits in high risk patients. Eur J Cardiothorac Surg 1998;13:514-519.
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9. Schreiber C., Cook A., Ho S.Y., Augustin N., Anderson R.H. Morphologic spectrum of Ebstein's malformation: revisitation relative to surgical repair. J Thorac Cardiovasc Surg 1999;117:148-155.[Abstract/Free Full Text]
10. Oh J.K., Holmes D.R., Hayes D.L., Co-Burn J.P., Danielson G.K. Cardiac arrhythmias in patients with surgical repair of Ebstein's anomaly. J Am Coll Cardiol 1985;6:1351-1357.[Abstract]
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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 Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Robert Chen David B. Ross Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Hancock Friesen, C. L. Articles by Ross, D. B. Search for Related Content PubMed PubMed Citation Articles by Hancock Friesen, C. L. Articles by Ross, D. B. Related Collections Congenital - acyanotic