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J Thorac Cardiovasc Surg 2008;135:1120-1136
© 2008 The American Association for Thoracic Surgery

Surgery for Congenital Heart Disease

The outcomes of operations for 539 patients with Ebstein anomaly

^a Division of Cardiovascular Surgery, Mayo Clinic College of Medicine, Mayo Clinic and Foundation, Rochester, Minn
^b Division of Pediatric Cardiology, Mayo Clinic College of Medicine, Mayo Clinic and Foundation, Rochester, Minn
^c Division of Cardiovascular Medicine, Mayo Clinic College of Medicine, Mayo Clinic and Foundation, Rochester, Minn
^d Division of Biostatistics, Mayo Clinic College of Medicine, Mayo Clinic and Foundation, Rochester, Minn

Received for publication July 31, 2007; revisions received February 28, 2008; accepted for publication February 28, 2008.

^_* Address for reprints: Joseph A. Dearani, MD, Mayo Clinic, 200 1st St SW, Rochester MN 55905. (Email: jdearani{at}mayo.edu).

	Abstract

Top Abstract Introduction Materials and Methods Results Discussion Summary Appendix E1 Table E1 Table E2 Table E3 Table E4 Table E5 References

 
Objective: Our objective was to review the long-term outcomes of patients with Ebstein anomaly who underwent cardiac surgery at our institution.

Methods: Patient records were reviewed, and all patients were mailed a medical questionnaire or contacted by means of telephone. Patients who had pulmonary atresia with an intact ventricular septum, complex conotruncal abnormalities, and atrioventricular discordance with ventriculoarterial discordance were excluded.

Results: From April 1, 1972, to January 1, 2006, 539 patients with Ebstein anomaly had 604 cardiac operations. The mean age at the time of the initial operation at our institution was 24 years (range, 8 days–79 years). Three hundred seventeen of the patients were female. One hundred forty-three (26.5%) patients had a prior invasive cardiac procedure before coming to Mayo Clinic. At the time of the first operation at Mayo Clinic, 182 patients had tricuspid valve repair, and 337 had tricuspid valve replacement. The 30-day mortality was 5.9% for the entire cohort (2.7% after 2001). Late survival was 84.7% at 10 years and 71.2% at 20 years. In a multivariate analysis of overall mortality for the patients' first operation at Mayo Clinic, increased hematocrit values, pulmonary valve stenosis, tricuspid valve replacement, absence of ablation of an accessory pathway, miscellaneous arrhythmia procedure, branch pulmonary artery enlargement, need for mechanical support postoperatively, emergency chest opening in the intensive care unit, and absence of sinus rhythm at dismissal were all predictive of mortality. When only preoperative characteristics were included, increased hematocrit values, mitral valve regurgitation requiring surgical intervention, prior cardiac procedure, and moderate-to-severe to severe reduction in right ventricular systolic function were associated with mortality. Preoperative sinus rhythm and an accessory pathway were associated with survival. Patients rated their health as excellent or good (New York Heart Association class I or II) in 83% of surveys returned.

Conclusion: Ebstein anomaly can be surgically treated with low perioperative mortality. Both tricuspid valve repair and tricuspid valve replacement are associated with good long-term survival. Risk factors for poorer outcome included right, and/or left ventricular systolic dysfunction; increased hemoglobin/hematocrit values; male sex; right ventricular outflow tract obstruction; or hypoplastic pulmonary arteries.

	Introduction

Top Abstract Introduction Materials and Methods Results Discussion Summary Appendix E1 Table E1 Table E2 Table E3 Table E4 Table E5 References

 

Earn CME credits at http://cme.ctsnetjournals.org

 

Ebstein anomaly is a spectrum of tricuspid valve (TV) and right ventricular (RV) dysplasia.¹ The TV usually is insufficient but also might be stenotic. Atrial septal defect (ASD) or patent foramen ovale (PFO) occurs in 30% to 70% of cases, and ventricular pre-excitation is associated with approximately 15% of cases. Less commonly, left ventricular (LV) dysfunction and ventricular septal defect are observed. Morbidity and mortality are thought to be related to the degree of TV regurgitation or stenosis; the size, thickness, and function of the right ventricle; and the presence or absence of an ASD. Patients with mild forms of Ebstein anomaly can be symptom free throughout their entire lives, but patients with severe forms can die in utero.

The purpose of this report is to assess the long-term outcome of operations for Ebstein anomaly. Specifically, we intend to define operative and long-term mortality and the determinants of operative and long-term mortality, to define the determinants of reoperation, and to compare the outcomes of TV repair (TVrpr) and TV replacement (TVrpl).

	Materials and Methods

Top Abstract Introduction Materials and Methods Results Discussion Summary Appendix E1 Table E1 Table E2 Table E3 Table E4 Table E5 References

 
Patients
From April 1, 1972, to January 1, 2006, 539 patients with Ebstein anomaly had an operation at the Mayo Clinic performed by one of 2 surgeons (GKD, JAD). Excluded from this cohort are patients with pulmonary atresia with intact ventricular septum, complex conotruncal abnormalities, and atrioventricular discordance with ventriculoarterial discordance ("corrected transposition").

Indications for Operation
The indications for operation included 1 or more of the following: symptoms of dyspnea or right-sided heart failure (New York Heart Association class III or IV), progressive exercise intolerance, tachyarrhythmias not controllable with medication or amenable to catheter-based intervention, and significant associated lesions, including ASDs, ventricular septal defects, or pulmonary stenosis. In some patients, progressive cardiomegaly, a cardiothoracic ratio of greater than 0.65, severe cyanosis, and reduced LV function were an indication for operation.

Operative Management
Operative management of patients with Ebstein anomaly has been described previously and consisted of (1) electrophysiologic mapping for localization of accessory conduction pathways in patients with ventricular pre-excitation; (2) closure of any ASD or PFO; (3) elimination of previously placed shunts and correction of any associated anomalies, such as ventricular septal defect, pulmonary stenosis, or patent ductus arteriosus; (4) performance of any indicated antiarrhythmia procedures, such as surgical division of accessory conduction pathways, cryoablation of atrioventricular nodal re-entry tachycardia, or the right-sided maze procedure; (5) consideration of plication of the atrialized right ventricle; (6) reconstruction of the TV when feasible or valve replacement; and (7) excision of redundant right atrial wall (right reduction atrioplasty).^1,2 In a few patients a bidirectional cavopulmonary shunt was performed when the right ventricle was markedly dilated and functioning poorly.³

Our initial repair, reported in 1979, consisted of plication of the free wall of the atrialized portion of the right ventricle, posterior tricuspid annuloplasty, and right reduction atrioplasty.⁴ The repair was based on the construction of a monocusp valve by using the anterior leaflet; it brought the functional tricuspid annulus up to the true tricuspid annulus. More recently, we have incorporated various modifications of TVrpr based on the numerous variants of Ebstein anomaly subsequently encountered.¹ This includes TVrpr at the level of the functional tricuspid annulus by bringing the anterior papillary muscle or muscles toward the ventricular septum to facilitate coaptation of the leading edge of the anterior leaflet with the ventricular septum, adding an anterior annuloplasty when indicated, and selective plication or resection of the atrialized RV. In this experience the 2 most important features that enabled a successful durable repair were a free leading edge of the anterior leaflet and at least 50% delamination of the anterior leaflet. TVrpr is performed during aortic crossclamping, and therefore the valve anatomy could be accurately assessed and repair sutures could be placed with minimal motion trauma. After completion of a valve repair, the TV is tested by means of bulb syringe injection of saline into the right ventricle with temporary pulmonary artery occlusion. Before 1982, intraoperative assessment to detect residual tricuspid regurgitation (TR) after valve repair was performed by introducing an exploring finger into the right atrium through the appendage for direct palpation of the TV in the beating heart. Intraoperative assessment of the TV after repair or replacement was performed with epicardial echocardiography from 1982 through 1985, after which transesophageal echocardiography was used routinely.

When the TV could not be reconstructed, the valve tissue adjacent to the RV outflow tract was excised, and a prosthetic valve (bioprosthetic more often than mechanical) was inserted.⁵ The suture line was deviated cephalad to the atrioventricular node, bundle of His, and membranous septum to avoid injury to the conduction system. The suture line was deviated cephalad to the tricuspid annulus posterolaterally, where the tissues were frequently very thin, to avoid injury to the right coronary artery. The struts of the bioprosthetic valve were oriented so that they straddled the membranous septum and conduction tissue. The valve sutures were tied while the heart was perfused and beating to detect any rhythm abnormalities.

At the beginning of our experience, a portion of the atrialized right ventricle was routinely excised. However, postoperative ventricular arrhythmias were common, and we believed this could be related to ventricular suture lines, compromise of small branches of the right coronary artery, or both. In addition, we have a new appreciation that in some patients, the thin-walled atrialized right ventricle maintains contractility. Our current practice is to plicate or resect an atrialized ventricle selectively. This is most often performed when it is thin, transparent, and dyskinetic on echocardiography.

Data Collection
Two of the investigators (MLB and DJD) collected all pertinent data from the Mayo Clinic medical record. This included all follow-up information regarding reoperation and deaths that had been received from non–Mayo Clinic–referring physicians and health care providers.

In addition to review of medical records, vital status was assessed through an online database (www.Accurint.com). Detailed health status questionnaires were mailed to all participants not known to be dead. Patients who did not return or complete the questionnaire were sent a second questionnaire. If the second questionnaire was not returned or completed, attempts were made to contact the patient by means of telephone. Two hundred eighty-five patients (64% of 448 alive patients) completed questionnaires and Health Insurance Portability and Accountability Act forms. Forty-three patients completed a questionnaire but did not complete the Health Insurance Portability and Accountability Act form, and 28 patients refused to complete the survey.

Statistical Analysis
Statistical analysis included the Fisher exact test and the ² test of association for comparing proportions. Logistic regression was carried out to analyze early (30 days) mortality. Log-rank tests and Cox proportional hazards models were used to identify univariate and multivariate predictors of overall mortality, late (>30 days) mortality, and late (>30 days) reoperation. In addition, late survival free from reoperation was determined by using death or reoperation as an event. Kaplan–Meier survival curves were drawn for all late outcomes. Predictor variables were analyzed as continuous or categorical variables, as appropriate. Continuous variables were dichotomized when either statistically or clinically appropriate. Multivariate model building was based on a stepwise selection method. The SAS 9.1.3 statistical software system (SAS, Institute Inc, Cary, NC) was used throughout. Only 2-tailed probability values are reported.

Independent variables included, among others: age at operation; year of operation; surgeon; patient sex; age at diagnosis; cardiothoracic ratio on chest radiographic analysis; presence and type of preoperative arrhythmia; rhythm on electrocardiographic analysis; blood oxygen saturation (oximetry); hemoglobin value; hematocrit value; and previous cardiac procedures (see the complete list and definitions shown in Appendix E1). Associated cardiac defects and type of operation (including TVrpr, TVrpl, and miscellaneous concomitant procedures) also were included. Echocardiographic variables included preoperative and postoperative RV size and function; degree of tricuspid stenosis, regurgitation, or both; degree of pulmonary stenosis or regurgitation; degree of mitral valve (MV) stenosis or regurgitation; and LV ejection fraction. The degree of TR, RV or LV systolic dysfunction, and RV enlargement was graded as none, mild, moderate, moderate-to-severe, and severe on transthoracic echocardiographic analysis. Echocardiographic variables with more than 15% missing data (and up to 45%) were not included in the stepwise selection for the initial multivariate model. To assess their effects, a second multivariate model was fit by first forcing all the variables chosen for the initial multivariate model and then putting in the variables with missing data one by one to see whether they are significant after adjusting for the effects from the initial multivariate model.

The initial operation at Mayo Clinic was TVrpr for 182 patients, TVrpl for 337 patients, and a variety of nonvalve operations for 20 patients ( Figure 1). Of the patients who initially had TVrpr at the Mayo Clinic, 1 patient subsequently underwent rerepair, and 35 subsequently had TVrpl (Figure 1). In both the univariate and multivariate analyses, the 35 patients who had both TVrpr and TVrpl were included both in the repair group and in the replacement group. Also, 6 patients who had "other" operations and subsequent TVrpl were included in the replacement group. Thus the TVrpr group had 182 patients, and the TVrpl group had 378 patients.

View larger version (14K): [in this window] [in a new window]   Figure 1. Operations on the tricuspid valve (TV) at Mayo Clinic.

	Results

Top Abstract Introduction Materials and Methods Results Discussion Summary Appendix E1 Table E1 Table E2 Table E3 Table E4 Table E5 References

One hundred forty-three patients had prior cardiac procedures before coming to the Mayo Clinic ( Table 1). The 4 most common procedures were closure of an ASD or PFO in 41 (7.6%) patients, surgical ablation of an accessory pathway in 35 (6.5%) patients, placement of a systemic to pulmonary artery shunt in 33 (6.1%) patients, and TVrpr or TVrpl in 18 (3.3%) patients. Rereplacement of a tricuspid prosthesis was done for significant prosthesis regurgitation, stenosis, or both. None were replaced for patient–prosthesis mismatch.

The most common reason for early reoperation (30 days after the index operation) was for bleeding (16 patients) and delayed sternal closure (16 patients; Table E5). Eleven patients required emergency reopening of the chest in the intensive care unit (ICU). Permanent pacemakers were inserted in 21 patients: 12 for third-degree atrioventricular block and 9 for miscellaneous reasons.

Entire Cohort
Overall total mortality
Data from all 539 patients were used in this analysis. The 30-day and 1-, 5-, 10-, 15-, and 20-year survivals were 94%, 92%, 88%, 85%, 81%, and 71%, respectively ( Figure 2 and Table 3). In a multivariate model pulmonary valve stenosis, a higher hematocrit value, TVrpl, branch pulmonary artery enlargement, emergency opening of the chest in the ICU, a miscellaneous arrhythmia procedure (cryoablation, left-sided maze procedure, or ligation of the left atrial appendage), and postoperative need for mechanical cardiac support were associated with higher mortality. Ablation of an accessory pathway and sinus rhythm at discharge were associated with lower mortality.

View larger version (5K): [in this window] [in a new window]   Figure 2. Long-term survival for all 539 patients. Time 0 is the time of the first cardiac operation at the Mayo Clinic.

There were numerous variables associated with mortality in a univariate fashion (Table 3). Those of particular interest included increased preoperative RV size and decreased RV systolic function, preoperative decreased LV systolic function, cardiothoracic ratio of greater than 65% determined by means of chest radiography, and number of variables related to a right ventricular outflow tract (RVOT) obstruction or small pulmonary arteries.

Overall early mortality
There were 33 deaths 30 days or less after the operation. In a multivariate model emergency opening of the chest in the ICU, postoperative need for mechanical cardiac support, and early tamponade were associated with higher mortality. When only preoperative variables were entered into the model, moderate-to-severe and severe RV systolic dysfunction were the only variables associated with higher mortality (Table 3).

Overall late mortality
When the 33 early deaths were excluded, the 1-, 5-, 10-, 15-, and 20-year survivals were 98%, 94%, 90%, 86%, and 76%, respectively. In a multivariate model MV regurgitation requiring operative repair, RV outflow tract enlargement, enlargement of the pulmonary arteries, renal insufficiency, and increased QRS duration were associated with increased mortality. Late survival was associated with ablation of an accessory pathway and preoperative sinus rhythm (Table 3).

When only preoperative variables were entered into the model, MV regurgitation requiring operation, increased hematocrit values, and LV dysfunction were associated with greater mortality and an accessory conduction pathway and the presence of preoperative sinus rhythm were associated with lower mortality.

Overall freedom from late reoperation
The 1-, 5-, 10-, 15-, and 20-year freedoms from any (on the TV or other) reoperation were 97%, 91%, 82%, and 70%, respectively. In a multivariate model hypoplastic or stenotic pulmonary arteries, insertion of an intra-aortic balloon pump postoperatively, prior cavopulmonary shunt, younger age at the time of the operation, and earlier era of operation were associated with late reoperation. When only preoperative variables were entered into the model, pulmonary valve stenosis, hypoplastic or stenotic pulmonary arteries, prior cavopulmonary shunt, younger age at the time of the operation, and an earlier era of operation were associated with late reoperation.

Survival free from late operation
The 1-, 5-, 10-, 15-, and 20-year survivals and freedoms from any (on the TV or other) reoperation were 95%, 86%, 74%, 62%, and 46%, respectively ( Figure 3). In a multivariate model the following variables were associated with an increased risk of death or reoperation: deep sternal wound infection, miscellaneous arrhythmia procedure, wide complex tachycardia, moderate-to-severe or severe postoperative RV systolic dysfunction, moderate-to-severe preoperative LV systolic dysfunction, and age less than 12 years at the time of the operation. When only preoperative variables are considered, a prior cavopulmonary shunt, pulmonary valve stenosis, male sex, moderate-to-severe preoperative LV systolic dysfunction, and age less than 12 years were associated with higher risk of death or late reoperation (Table 3).

View larger version (6K): [in this window] [in a new window]   Figure 3. Survival free from late reoperation (on the tricuspid valve or other) for all patients after the first cardiac operation at the Mayo Clinic.

TVrpr Versus TVrpl
The patients whose operations at the Mayo Clinic were TVrprs differed from those whose operations were TVrpls in a number of ways. In these comparisons, patients who had TVrpr followed by TVrpl were only included in the TVrpr group. In general, patients who had valve repair were operated on earlier in the series (mean year of operation, 1989 vs 1996). On preoperative transthoracic echocardiographic analysis, the TV was judged to be not repairable in 32% of the repair group and in 85% in the replacement group. On echocardiographic analysis, anatomic severity of the valve malformation was accessed to be moderately severe and severe in 61% of the repair group and 93% of the replacement group. The replacement group had more prior cardiac procedures than the repair group. All of these differences were statistically significant ( Table 4). Postoperatively, 13% of the patients who had valve repair had at least moderate residual TR, whereas no patients who had replacement had moderate residual TR.

Late mortality
When the 9 early deaths were excluded, the 1-, 5-, 10-, 15-, and 20-year survivals were 98%, 98%, 93%, 92%, and 80%, respectively. In a multivariate model the previous cardiac procedure was associated with increased mortality, and preoperative sinus rhythm was associated with lower mortality (Table 5).

Freedom from late reoperation
The 1-, 5-, 10-, 15-, and 20-year freedoms from reoperation on the TV were 100%, 95%, 89%, 77%, and 64%, respectively. In a multivariate model prior surgical procedure and increased QRS duration were associated with lower freedom from reoperation. Plication of an atrialized right ventricle reduced the need for late reoperation. When only preoperative variables were entered into the model, patients with a prior cardiac surgical procedure were associated with a lower freedom from reoperation. The freedom from reoperation rate over a 20-year period was 51%, 83%, and 61% for patients 10 years of age or less, 11 to 20 years of age, and more than 20 years of age, respectively (P = .032).

Survival free from late reoperation
The 1-, 5-, 10-, 15-, and 20-year survivals free from reoperation on the TV was 98%, 93%, 84%, 73%, and 56%, respectively. In a multivariate model a greater risk of death or reoperation was associated with the following variables: right-sided maze procedure, pulmonary valve stenosis, a previous cardiovascular procedure, greater QRS duration, moderate-to-severe LV systolic dysfunction postoperatively, and male sex. When only preoperative variables were included, male sex, pulmonary valve stenosis, and previous cardiovascular procedures were associated with greater risk of death or late reoperation (Table 5).

Recurring variables
Several variables were statistically significant in a univariate model for 3 of the 4 following subsets: overall mortality, early mortality, late mortality, and survival free from late reoperation (Table 5). The 4 variables included male sex, reduced preoperative and postoperative LV systolic function, any prior cardiac procedure, prior cavopulmonary shunt, and prior stenting or coronary artery bypass grafting procedure.

TVrpl Subset
Overall mortality
Data from 378 patients who had TVrpl were used in this analysis. Of these 378 patients, 43 (11.4%) had a prior TVrpr. The 30-day and 1-, 5-, 10-, 15-, and 20-year survivals were 94%, 91%, 86%, 83%, 74%, and 68%, respectively. In a multivariate model repair of pulmonary valve stenosis, miscellaneous arrhythmia procedure, mechanical circulatory support postoperatively, and emergency opening of the chest in the ICU were associated with higher mortality. Surgical ablation of an accessory pathway and postoperative sinus rhythm were associated with lower mortality.

When only preoperative variables were included in the model, MV regurgitation requiring an operation, and reduced LV systolic function were associated with increased mortality. The presence of an accessory conduction pathway was associated with lower mortality. There were numerous variables associated with higher mortality in a univariate fashion ( Table 6). Those of particular interest included increased preoperative RV size and decreased RV systolic function, preoperative decreased LV systolic function, radiographic cardiothoracic ratio of greater than 0.65, lower blood oxygen saturation, and higher hemoglobin levels.

Late mortality
When the 23 early deaths were excluded, the 1-, 5-, 10-, 15-, and 20-year survivals were 97%, 92%, 88%, 79%, and 72%. In a multivariate model RVOT enlargement, wide complex tachycardia, early reoperation, and QRS duration were associated with higher mortality. An accessory pathway was associated with lower mortality (Table 6). Considering only preoperative variables, MV regurgitation requiring an operation and reduced RV systolic function preoperatively were associated with late mortality. An accessory pathway was associated with late survival.

Freedom from late reoperation on the TV
The 1-, 5-, 10-, 15-, and 20-year freedoms from reoperation were 99%, 95%, 83%, 70%, and 59%, respectively. In a multivariate model younger age, greater MV regurgitation requiring surgical intervention, postoperative need for an intra-aortic balloon pump, and a catheter ablation procedure were associated with lower freedom from reoperation. When only preoperative variables were entered into the model, younger age at the time of the operation, MV regurgitation requiring an operation, a catheter ablation procedure, and a prior cardiac procedure were associated with lower freedom from reoperation.

Survival free from reoperation on the TV
The 1-, 5-, 10-, 15-, and 20-year freedoms from reoperation were 96%, 87%, 74%, 56%, and 44%, respectively. In a multivariate model the following variables were associated with death or late reoperation on the TV: MV regurgitation requiring an operation, postoperative renal insufficiency, wide complex tachycardia, moderate-to-severe preoperative reduction of LV systolic function, and age less than 12 years at the time of operation. Preoperative variables associated with death or late reoperation on the TV were as follows: MV regurgitation requiring an operation, moderate-to-severe preoperative reduction of LV systolic function, and younger age at the time of the operation (Table 6).

Recurring variables
Several variables were statistically significant in a univariate model for 3 of the 4 following subsets and were associated with a lower rate of reoperation: overall mortality, early mortality, late mortality, and survival free from late reoperation (Table 6). They included increased preoperative cardiothoracic ratio, reduced preoperative RV and LV systolic function, reduced postoperative RV systolic function, MV regurgitation requiring surgical intervention, miscellaneous arrhythmia surgery, postoperative wide complex tachycardia, postoperative mechanical support or miscellaneous early operation.

A summary of all independent predictors of overall mortality, early and late mortality, and survival free from reoperation is shown in Table 7.

View larger version (13K): [in this window] [in a new window]   Figure 4. A and B, Survival free from late reoperation on the tricuspid valve (TV) for patients less than 12 or 12 or more years of age. The 35 patients who had TV replacement and had prior TV repair followed by TV replacement were removed from the TV replacement group.

Protective Effect of Pre-excitation
In several analyses the presence of ventricular pre-excitation or its likely surrogates (ie, preoperative arrhythmia, ablation of accessory pathways) was associated with improved survival. The only differences between patients with and without pre-excitation were surgeon (surrogate for surgical division of bypass tract), degree of MV regurgitation, prior closure of an ASD or PFO, history of an arrhythmia, year of operation, and age at the time of the operation. There did not appear to be a relationship between the severity of the Ebstein anomaly and the presence of pre-excitation.

	Discussion

Top Abstract Introduction Materials and Methods Results Discussion Summary Appendix E1 Table E1 Table E2 Table E3 Table E4 Table E5 References

 
The management of Ebstein anomaly requires a choice between nonoperative treatment and operative interventions. This choice may be difficult because of the very broad spectrum of severity of Ebstein anomaly. Patients with very severe forms of Ebstein anomaly can die in utero or shortly after birth, but patients with mild forms of the anomaly can be completely asymptomatic for their entire life and never need specific treatment. There are no randomized trials comparing the results of surgical treatment with those of nonsurgical treatment. Celermajer et al⁶ found that greater severity of Ebstein anomaly, cardiothoracic ratio of greater than 60%, RVOT obstruction, and presentation as a fetus were associated with poorer survival. Interestingly, ventricular pre-excitation and era of diagnosis were not associated with increased risk of death. Attie et al⁷ also reported that poorer outcome was associated with more severe forms of Ebstein anomaly, male sex, cyanosis, worse functional status, severe TR, and early age at diagnosis in 72 unoperated adult patients. Thus the indications for operation we used in the current study seem reasonable.

Our study is consistent with the findings of previous studies^6,7 because male sex, RVOT obstruction, cyanosis, and more severe forms of the anomaly were associated with poorer outcome. Cardiomegaly was associated with poorer outcome in our study in a univariate, but not in a multivariate, model.

Celermajer et al⁶ showed no effect of pre-excitation on outcome, and in our study ventricular pre-excitation was associated with better outcome. Many patients in this study were operated on before the era of transvenous catheter ablation. We speculated that the improved survival occurred because the primary indication for operation was for surgical division of the bypass tract, and these patients might have had less severe forms of Ebstein anomaly at the time they had their operations.^9,10 However, except for the fact that patients who had pre-excitation were younger than those without pre-excitation, our data did not support this speculation. It might also be that patients who have but are unknown to have a bypass tract might die later because of a ventricular arrhythmia. Thus patients who have a bypass tract and receive proper treatment might have a survival advantage when compared with patients who have not undergone adequate treatment.

Echocardiographic Assessment of Valve Reparability in Ebstein Anomaly
Favorable echocardiographic criteria for TVrpr include both the valve leaflet location and morphology and the papillary muscle location and attachments. Valves that have severe leaflet displacement into the RV apex or those anteriorly rotated into the RVOT are generally not suitable for the traditional monocusp repair. These forms of Ebstein anomaly leave very little effective right ventricle below the point of coaptation. If the echocardiographer does not view TV tissue in the apical 4-chamber view, the valve will typically need to be replaced. However, it is possible that the apical 4-chamber view can be angled anteriorly, so that some of the outflow tract is in view. In this projection one might falsely believe that the septal leaflet is present near the true TV inlet. The internal cardiac crux should be identified to avoid this pitfall. Views from the parasternal short-axis projection at the base of the heart can aid in identifying the proximity of TV tissue in the RVOT to the pulmonary valve. If the anterior leaflet is tethered at multiple sites (most importantly the tip), then the surgeon might have difficulty mobilizing the leaflet, and coaptation with the septum will be ineffective. The apical 4-chamber projection can be used to determine the tethering sites of the anterior leaflet. Positioning of the papillary muscles can be assessed from most imaging planes and is important for assessment of leaflet mobility. Dense papillary muscle attachments to the anterior leaflet can prohibit a traditional repair. Sacrificing these attachments might result in important residual TV regurgitation after repair.

Valves that have multiple leaflet fenestrations (hence multiple jets of TR) might be difficult to repair because of a deficiency of useable tissue. Color Doppler assessment of TR can be difficult because RV systolic pressure is usually normal in these patients, and regurgitant jets can appear as very laminar color flow. In addition, these jets can be oriented in directions that are not usually expected. The severity of TR is frequently underestimated by the inexperienced echocardiographer. Principles of color Doppler scanning are based on jet velocity and not on the quantity of regurgitation. This can have important implications because surgical intervention might be inappropriately deferred if the amount of TR is underestimated. The preoperative echocardiographic assessment of the patient with Ebstein anomaly requires meticulous use of all imaging planes and experience gathered from evaluating large numbers of these patients.

Caveats
In this study we hoped to identify the determinants of poor outcome and the determinants of good outcome. Intuitively, and based on prior small studies, one might suggest that RV size and function, as well as LV systolic function and other measures of severity of TV dysplasia, would correlate with outcome. Because our study is retrospective, we had to use echocardiographic data obtained over a 33-year period. These data were not reported in a uniform fashion. Also, over the study, measurement of RV size and function was (and remains) imperfect by means of echocardiographic analysis. In addition, because of leftward displacement of the ventricular septum, it is difficult to know the implications of measured LV ejection fraction. Despite these caveats, RV size and both RV and LV systolic function are important determinants of outcome. Improved measurements of ventricular size and function, such as could be obtained with magnetic resonance imaging, might increase the usefulness of ventricular size and function in predicting outcome.

Mortality
Previously, we reported early mortality of 6.3% for 189 patients who underwent TVrpr or TVrpl (these 189 patients are an early part of the current cohort). In the current era mortality ranges from 2.5% to 31%.^2,5,8,11-21 In a collaborative study of 150 patients, the early mortality was reported as 13%.⁸ In the current report the early mortality was 6% for all 539 patients, 5% for the 182 patients who had TVrpr, and 6% for the 378 patients who had TVrpl. Considering only the operations since 2001, perioperative mortality decreased to 2.7% (operations = 110).

Total mortality in the current study (20 year; both early and late) was 29% for all patients, 24% for patients who had TVrpr, and 32% for patients who had TVrpl. To our knowledge, there are no other studies of this magnitude and length of follow-up with which to compare our results.

Reoperation
We presented risk of late reoperation in 2 formats: freedom from late reoperation and survival free from late reoperation. In both analyses, 10- and 20-year freedom from reoperation was better in the valve repair than in the valve replacement group. The highest rate of reoperation was in patients less than 12 years of age who had valve replacement. These results, however, are not just a function of whether the valve was replaced or repaired but, more importantly, on the severity of the disease that led to the decision to repair or replace the valve. The decision to repair or replace the valve is guided by whether the valve is amenable to repair with minimal residual valve regurgitation, as well as comorbid conditions, which might sway the surgeon to do the quickest operation with the shortest ischemic time. For example, a patient with severely reduced RV systolic function, moderately reduced LV systolic dysfunction, hypoplastic pulmonary arteries, and secondary erythrocytosis might be better served by valve replacement than by an attempted valve repair associated with moderately-severe residual regurgitation.

Valve Repair Versus Valve Replacement
An important question is whether it is better to repair or replace the TV. Intuitively, one might suggest that the operation associated with lower mortality and lower reoperation rate would be the preferable operation. However, if the goal of the operation is to reduce or eliminate the volume overload of the right atrium and ventricle, then the operation that is associated with a lesser degree of residual TR might be preferable. In this study the 20-year survival for TVrpr was 76%, and that for TVrpl was 67%. Survival free from reoperation at 20 years for valve repair was 53% for patients operated on at less than 12 years of age and 57% for patients operated on at more than 12 years of age. Survival free from reoperation at 20 years for valve replacement was 16% for patients operated on at less then 12 years of age and 60% for patients operated on at more than 12 years of age.

One must resist the temptation to conclude that valve repair is inherently superior to valve replacement based on these data. It is clear that those patients who had valve replacement had more severe disease than those who had valve repair. In addition, significant residual TV regurgitation persisted in 33% of patients who had valve repair but in only 1% of patients who had valve replacement. This would favor valve replacement. It also is important not to conclude that one operation is superior to the other based on short-term outcome because medium- to late-term failure of valve repair clearly occurs.

For patients 12 years of age and older, we believe that the valve should be repaired if there is not more than moderate residual TR at the end of the operation. If there is greater than moderate residual TR, we believe the valve should be replaced because of the long-term effects of TR on RV function. For patients younger than 12 years of age, greater degrees of TV regurgitation might be acceptable because of the relatively greater need for reoperation for repeat valve replacement in this age group.

Numerous techniques have been described for repair of the TV since the first report of Hunter and Lillihei.^{1,2,13,17,20-32} This is not surprising because no 2 hearts with Ebstein anomaly have exactly the same anatomy. In addition, the enormous number of repair techniques is likely the result of incomplete satisfaction with current repair methods already described. Although many of the techniques available report good early results, very few document late outcomes. Undoubtedly, the techniques for valve repair will continue to evolve, and newer promising methods of repair need to be evaluated to determine whether a greater percentage of valves can be repaired while maintaining good long-term durability.^16,33

	Summary

Top Abstract Introduction Materials and Methods Results Discussion Summary Appendix E1 Table E1 Table E2 Table E3 Table E4 Table E5 References

 
We believe this is the largest surgical series of patients with Ebstein anomaly. Because we incorporated greater than 125 potential independent variables and assessed numerous dependent outcomes, there are numerous variables that were related to outcome both in a univariate fashion, multivariate fashion, or both. All of these can be important when trying to predict the early and late outcome of an operation. Table 7 provides a summary of the variables that were statistically significantly associated with 1 or more of the dependent outcome variables in a multivariate model.

In conclusion, it appears that male sex, RVOT obstruction (including pulmonary stenosis and hypoplastic or stenotic pulmonary arteries), MV regurgitation requiring surgical intervention, cyanosis, and of the RV and/or LV systolic dysfunction are among the more important preoperative variables that are predictive of higher mortality. These variables should be helpful in stratifying patients' potential operative risk.

	Appendix E1

Top Abstract Introduction Materials and Methods Results Discussion Summary Appendix E1 Table E1 Table E2 Table E3 Table E4 Table E5 References

 

Independent variables of interest

Demographics and laboratory data  Age at operation  Age at diagnosis by history  Patient sex  Surgeon (1 and 2)  Year of operation  Preoperative cardiothoracic ratio on chest radiography  Preoperative oxygen saturation  Preoperative hemoglobin  Preoperative hematocrit Echocardiography  Preoperative right ventricular size  Preoperative right ventricular systolic function  Preoperative tricuspid valve regurgitation severity  Preoperative left ventricular systolic function  Anatomic severity of Ebstein anomaly  TV repairable: yes, no, equivocal  Mitral valve systolic regurgitation  Pulmonary valve stenosis  Architecture of pulmonary arteries: hypoplastic, stenotic, or normal  Postoperative right ventricular size  Postoperative right ventricular systolic function  Postoperative tricuspid valve regurgitation severity  Postoperative left ventricular systolic function Associated heart disease  Atrial septal defect or patent foramen ovale  Accessory conduction pathway  Pulmonary valve stenosis  Ventricular septal defect  Atrioventricular node re-entry tachycardia  Bilateral superior venae cavae  Patent ductus arteriosus  Partial atrioventricular septal defect  Coronary artery disease on angiography  Anomalous pulmonary venous connection  Pericarditis  Hypertrophic obstructive cardiomyopathy  Absent coronary sinus  Mitral valve regurgitation requiring surgical intervention  Subvalvular pulmonary stenosis/right ventricular outflow tract obstruction  Pulmonary artery stenosis or hypoplasia  Pulmonary valve regurgitation  Miscellaneous associated cardiac defects not included in any above Previous cardiovascular procedure  Any prior cardiac procedure, including surgical procedures, pacemakers, and catheter-based interventions  Prior closure of atrial septal defect  Prior tricuspid valve repair  Prior tricuspid valve replacement  Prior systemic-to-pulmonary artery shunt  Prior cavopulmonary shunt  Prior permanent pacemaker  Prior ablation of accessory pathway: catheter based and surgical based  Prior closure of ventricular septal defect  Prior pulmonary valvotomy  Prior tricuspid valve replacement  Prior pulmonary artery banding  Prior coronary stenting or coronary artery bypass grafting  Prior repair of coarctation  Prior closure of patent ductus arteriosus  Prior enlargement of right ventricular outflow tract  Prior complete pericardiectomy  Prior left superior vena cava ligation Surgical procedure  Closure of atrial septal defect  Right reduction atrioplasty  Plication of atrialized right ventricle  Anterior right pericardiectomy  Tricuspid valve repair  Tricuspid valve replacement  Ablation of accessory pathway(s)  Right-sided maze procedure  Closure of any shunt  Systemic-to-pulmonary shunt  Cavopulmonary shunt  Repair of pulmonary valve stenosis  Closure of ventricular septal defect  Ablation of atrioventricular node re-entrant tachycardia  Fontan procedure  Coronary stenting or coronary artery bypass grafting  Repair of coarctation  Repair of partial atrioventricular septal defect  Systemic-to-pulmonary artery shunt  Coronary artery bypass grafting  Repair of partial anomalous pulmonary veins  Permanent pacing wires  Miscellaneous arrhythmia procedure: ablation of right or left isthmus or left-sided maze procedure  Mitral valve replacement or repair  Complete pericardiectomy  Pulmonary valve replacement  Myectomy  Enlargement of right ventricular outflow tract  Branch pulmonary artery enlargement  Electrophysiologic mapping  Closure of patent ductus arteriosus  Mechanical support and transplantation  Miscellaneous operation not listed above Early nonfatal complications (<30 d)  Third-degree heart block, transient (<3 d)  Third-degree heart block, permanent  Ventricular arrhythmia: premature ventricular contractions, ventricular tachycardia, or fibrillation  Wide complex tachycardia: ventricular tachycardia or fibrillation  Junctional tachycardia  Atrial arrhythmia: atrial fibrillation, atrial flutter, premature atrial contractions, supraventricular tachycardia  Miscellaneous arrhythmia not listed above  Myocardial infarction  Cerebrovascular accident  Low cardiac output state: inotropes >3 d  Intra-aortic balloon pump inserted in the intensive care unit  Respiratory insufficiency: ventilated >3 d  Renal insufficiency: >3 mg/dL  Dialysis  Gastrointestinal bleed  Deep sternal wound infection  Extracorporeal membrane oxygenation  Sepsis or endocarditis or pneumonia  Neurologic complication: seizures or transient ischemic attack  Thrombus on tricuspid valve or in right atrium requiring anticoagulation  Postpericardial syndrome  Hematologic complication  Miscellaneous complication not otherwise specified Early reoperation  Bleeding  Tamponade  Permanent pacemaker for third-degree heart block  Permanent pacemaker (miscellaneous reason)  Tracheostomy  Delayed sternal closure  Wound debridement  Tricuspid valve reoperation  LVAD, RVAD, or mechanical support  Creation of shunt  Pericardiectomy  Closure of shunt  Emergency opening of the chest in the intensive care unit  Miscellaneous early reoperation not listed above Arrhythmia data  Past history of any arrhythmia (including palpitations and other arrhythmias)  Past history of a specific arrhythmia (excluding palpitations)  Cardiac arrest before operation (before entering the operating theater)  Accessory pathway on electrophysiologic study  Atrioventricular nodal re-entry tachycardia on electrophysiologic study  Inducible ventricular tachycardia on electrophysiologic study  Inducible ventricular fibrillation on electrophysiologic study  Catheter ablation procedure on electrophysiologic study  Patients in sinus rhythm preoperatively on electrocardiography  Patients with pre-excitation on preoperative electrocardiography  Patients in sinus rhythm at dismissal on electrocardiography  QRS duration on dismissal or follow-up electrocardiography

	Table E1

Top Abstract Introduction Materials and Methods Results Discussion Summary Appendix E1 Table E1 Table E2 Table E3 Table E4 Table E5 References

 

Patient age at date of operation

All patients (first operation at Mayo Clinic; n = 539) Tricuspid valve repair group (n = 182) Tricuspid valve replacement group (n = 378) Age at operation (y) N % N % N % 10 156 29 39 21 112 30 11-20 115 21 51 28 68 18 >20 268 50 92 51 198 52

	Table E2

Top Abstract Introduction Materials and Methods Results Discussion Summary Appendix E1 Table E1 Table E2 Table E3 Table E4 Table E5 References

 

Associated cardiac defects

Patients Total (n = 539) % ASD or PFO 452 83.9 Ventricular septal defect 23 4.3 Pulmonary stenosis requiring surgical intervention 32 5.9 Subvalvular pulmonary stenosis/RVOT obstruction 9 1.7 Pulmonary artery stenosis or hypoplasia 8 1.5 Severe pulmonary regurgitation 4 0.7 Coronary artery disease by catheterization 9 1.7 Partial atrioventricular septal defect 8 1.5 Bilateral superior venae cavae 7 1.3 Patent ductus arteriosus 6 1.1 Absent coronary sinus 7 1.3 Mitral regurgitation (moderate to severe) 7 1.3 Pericarditis 5 0.9 Anomalous pulmonary venous connection 4 0.7 Hypertrophic obstructive cardiomyopathy 4 0.7 Miscellaneous cardiac defects 45 8.4

ASD, Atrial septal defect; PFO, patent foramen ovale; RVOT, right ventricular outflow tract.

	Table E3

Top Abstract Introduction Materials and Methods Results Discussion Summary Appendix E1 Table E1 Table E2 Table E3 Table E4 Table E5 References

 

Associated electrophysiologic abnormalities

Patients Total (n = 539) % Accessory conduction pathway 74 13.7 Atrioventricular nodal re-entry tachycardia 18 3.3 Inducible ventricular tachycardia or fibrillation 18 3.3 Cardiac arrest before surgical admission 15 2.8

	Table E4

Top Abstract Introduction Materials and Methods Results Discussion Summary Appendix E1 Table E1 Table E2 Table E3 Table E4 Table E5 References

 

Early complications (patients who died within 30 days are excluded)

Patients Total (n = 506) % Atrial arrhythmia: atrial fibrillation, atrial flutter, PACs, SVT 77 15.2 Ventricular arrhythmia: PVCs, Ventricular tachycardia, or ventricular fibrillation 72 14.2 Wide complex tachycardia: ventricular tachycardia or ventricular fibrillation only 42 8.3 Low cardiac output state 29 5.7 Respiratory insufficiency 28 5.5 Transient third-degree heart block 23 4.5 Junctional tachycardia 21 4.1 Sepsis or endocarditis or pneumonia 18 3.6 Renal insufficiency 12 2.4 Permanent third-degree heart block 11 2.2 Thrombus on tricuspid valve or in right atrium 9 1.8 Intra-aortic balloon pump 8 1.6 Dialysis 8 1.6 Deep sternal wound infection 6 1.2 Miscellaneous arrhythmias 5 1.0 Neurological complication: seizures, TIA 5 1.0 Hematologic complication 3 0.6 Myocardial infarction 2 0.4 Cerebrovascular accident 1 0.2 Gastrointestinal bleed 1 0.2

PACs, Premature atrial contractions; SVT, supraventricular tachycardia; TIA, transient ischemic attack.

	Table E5

Top Abstract Introduction Materials and Methods Results Discussion Summary Appendix E1 Table E1 Table E2 Table E3 Table E4 Table E5 References

 

Early cardiac reoperations (within 30 days)

Patients Total (n = 539) % Bleeding 16 3.0 Secondary sternal closure 16 3.0 Miscellaneous other early reoperations 13 2.4 Permanent pacemaker for third-degree HB 12 2.2 Emergency opening of the chest in the intensive care unit 11 2.0 Tamponade 10 1.9 Permanent pacemaker for miscellaneous reason 9 1.7 LVAD, RVAD, or mechanical support in perioperative period 6 1.1 Tricuspid valve reoperation 4 0.7 Wound debridement 4 0.7 Closure of shunt 3 0.6 Pericardiectomy 3 0.6 Creation of shunt 2 0.4

HB, Heart block; LVAD, left ventricular assist device; RVAD, right ventricular assist device.

	Footnotes

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

	References

Top Abstract Introduction Materials and Methods Results Discussion Summary Appendix E1 Table E1 Table E2 Table E3 Table E4 Table E5 References

 

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