Click here to read this article as a CME activity
Ann Thorac Surg 2009;88:131-136. doi:10.1016/j.athoracsur.2009.03.062
© 2009 The Society of Thoracic Surgeons
Original Articles: Pediatric Cardiac
Mid-Term Outcomes in Adults With Ebstein Anomaly and Cavopulmonary Shunts
Khalid S. Al-Najashi, MD,
Olga H. Balint, MD,
Erwin Oechslin, MD,
William G. Williams, MD,
Candice K. Silversides, MS, MD*
Toronto Congenital Cardiac Centre for Adults, University Health Network, University of Toronto, Toronto, Canada
Accepted for publication March 17, 2009.
* Address correspondence to Dr Silversides, University Health Network, Toronto General Hospital, 585 University Ave, 5N-521 North Wing, Toronto, Ontario, M5G 2N2, Canada (Email: candice.silversides{at}uhn.on.ca).
| PEDIATRIC CARDIAC SURGERY:
The Annals of Thoracic Surgery CME Program is located online at http://cme.ctsnetjournals.org. To take the CME activity related to this article, you must have either an STS member or an individual non-member subscription to the journal.
|
 |
Abstract
|
|---|
Background: In patients with Ebstein anomaly and poorly functioning right ventricles, a cavopulmonary shunt (CPS) can be created to reduce the preload on the right ventricle. The purpose of this study was to examine the early and mid-term outcomes in adults with Ebstein anomaly who have undergone tricuspid valve repair or replacement with or without a concomitant CPS.
Methods: We examined the outcomes of 40 consecutive patients seen at our center with Ebstein anomaly who had undergone tricuspid valve repair or replacement with (n = 23) or without (n = 17) concomitant CPS. Follow-up data were obtained by either chart review or contacting the referring cardiologist. Mid-term survival was examined using Kaplan-Meier curves.
Results: The mean age at surgery was similar in patients with and without CPS (42 ± 12 versus 39 ± 19 years; p = 0.63). There were 2 early postoperative deaths owing to refractory right-sided heart failure. Mid-term follow-up data were available in 95% of patients. The mean follow-up time was 6.7 ± 4.8 years. Patients who received a CPS more commonly had preoperative heart failure or cyanosis (p = 0.04) and had worse preoperative functional status (p = 0.09). In both groups, arrhythmias were the most common late complication. There were 5 late deaths, 3 of which occurred in patients with CPS. Five-year survival with or without CPS was comparable (83% ± 9% versus 86% ± 10%; p = 0.85).
Conclusions: Adolescent and adult patients with Ebstein anomaly undergoing tricuspid valve replacement or repair and concomitant CPS are at risk for early and mid-term complications. However, Ebstein surgery along with CPS appears to be a reasonable surgical strategy in patients not thought to be suitable for tricuspid valve surgery alone.
Ebstein anomaly of the tricuspid valve (TV) can lead to right heart failure, cyanosis, stroke, recurrent arrhythmias, and the subsequent need for surgical intervention [1, 2]. Surgery involves either repair or replacement of the TV, and a number of different surgical approaches have been described [1]. In select cases, a concomitant cavopulmonary shunt (CPS) can be added to reduce the preload to the hypokinetic or hypoplastic right ventricle (RV). This was first described by Marcelletti and colleagues in 1980 [3] and was first used at our center in this context in 1986. Although there have been reports describing the successful results of this surgical approach in children [4–6], late outcomes of this approach in adults have been less well studied and may differ in adults when compared with children. Furthermore, some centers have been reluctant to include the addition of CPS to standard Ebstein surgery because of the potential for higher complication rates and problems with vascular access [7].
The purpose of this study is to examine the early and mid-term outcomes in adult patients with Ebstein anomaly who have undergone TV repair or replacement with or without a concomitant CPS.
|
Material and Methods
|
|---|
The outcomes of all adult (>18 years) patients followed at our institution (Toronto Congenital Cardiac Centre for Adults, Toronto, Canada) who had undergone TV repair or replacement were examined. Patients who also had a CPS were compared with those who had not. All operations were performed between January 1986 and March 2005. An earlier publication (1996) from our surgical group addressed the use of CPS in all forms of congenital cardiac surgery and included a subset of the current study group [8]. Patients were excluded if they had congenitally corrected transposition of the great arteries or if the patient had single-ventricle type of repair. The institutional ethics board approved the study, and the need for patient consent was waived.
Baseline clinical, electrocardiographic, echocardiographic, and angiographic data were obtained from chart review. Demographic and clinical data included age, sex, associated lesions, indications for surgery, age at surgery, New York Heart Association (NYHA) functional classification, and surgical details. Cyanosis was defined as resting oxygen saturation of less than 90% by pulse oximetry or desaturations with exercise to less than 90%. Echocardiographically, TV regurgitation was classified qualitatively as mild, moderate, or severe either by transthoracic echocardiography, transesophageal echocardiography, or angiography. Right ventricular global systolic function was evaluated visually by echocardiography or angiography and reported to be mild, moderate, or severe systolic dysfunction. Hemodynamic data from cardiac catheterization were usually available in cases in which pulmonary artery pressure needed to be evaluated before surgery. The diagnosis of preoperative stroke was based on clinical or radiographic evidence of a cerebral vascular ischemic event.
Outcomes
Early postoperative adverse outcomes were defined as events occurring anytime before hospital discharge and included early death, repeat surgery before discharge, clinical heart failure, or arrhythmias requiring treatment. Follow-up data were obtained by either chart review or contacting the referring cardiologist. Mid-term outcomes of interest included death, clinical heart failure, arrhythmias requiring treatment, and NYHA functional status at the time of the last visit. Arrhythmias were identified on electrocardiograms or Holter monitors or at the time of an electrophysiology study. Heart failure was determined according to documentation of signs of heart failure by an attending physician.
Statistical Analysis
Data were analyzed using SPSS statistical software (version 11.5 for Windows, SPSS, Inc, Chicago, IL). All data are presented as mean ± standard deviation. Comparison between groups was performed using Fisher's exact test or Mann-Whitney U test as appropriate. Survival was examined using Kaplan-Meier method. Survival between patients with and without CPS was compared using a log-rank test. Significance was set at a probability value of less than 0.05 (two-sided).
|
Results
|
|---|
During the study period, 45 patients with Ebstein anomaly underwent surgical procedures in our institution. One patient underwent a single-ventricle type of repair and was excluded from the analysis. Four patients had their TV repair or replacement before 1986 and therefore were not included in this study. In total, 40 patients were included in this study; 17 patients underwent TV repair (n = 6) or replacement (n = 11) and 23 patients underwent TV repair (n = 12) or replacement (n = 11) along with a CPS. All associated shunt lesions were repaired at the time of surgery.
The use of CPS in this context was not initially performed at our institution until 1986. Figure 1
shows the number of cases and the year that they were performed. The mean age at surgery was 41 ± 15 years (range, 14 to 74 years). There were 4 patients who had undergone surgery before age 18 (age at surgery: 14 years in 1 patient, 16 years in 2 patients, and 17 years in 1 patient; 1 adolescent had a CPS). These patients were followed as adults in our adult cardiac clinic and were included in the study. In addition to the above TV surgery, associated intracardiac shunt lesions were repaired at the time of the initial operation. Characteristics of the cohort are shown in Table 1.
View larger version (34K):
[in this window]
[in a new window]
|
Fig 1. Surgical patterns: number of surgeries performed during 5-year intervals. (CPS = cavopulmonary shunt.)
|
|
The decision to perform a CPS at our institution was based on finding of significant RV dysfunction or hypoplasia before or at the time of surgery and normal pulmonary vascular resistance. Ultimately, this decision was at the discretion of the surgeon. The baseline characteristics of each group are shown in Table 1. Compared with patients who did not get a CPS, patients who received a CPS more commonly had associated cardiac lesions (83% versus 47%; p = 0.02) or preoperative heart failure or cyanosis (91% versus 65%; p = 0.04), and there was a trend for patients to have worse preoperative functional status (NYHA III, 56% versus 29%; p = 0.09). Furthermore, patients who received a CPS were deemed to be at higher risk by the treating surgeon. There were no differences in the age at surgery or the frequency of preoperative arrhythmias or strokes between patients with and without CPS.
Early Postoperative Outcomes
Early surgical outcomes were available in all patients (Table 2). There were 2 postoperative deaths (5%). The first patient had undergone a TV repair and a CPS and died 2 days after surgery with residual severe TV regurgitation, right-sided heart failure, and sustained atrial and ventricular arrhythmias. The second patient had a TV replacement and had severe RV failure, sustained atrial and ventricular arrhythmias, and subsequent multiorgan failure. Two patients (5%) required repeat surgery because of residual significant moderate to severe TV regurgitation; this was detected immediately in the first patient and at day 12 in the second patient.
Arrhythmias were the most common late complication occurring in 17 patients (total cohort 43%; with and without CPS 43% versus 41%; p = 0.88); 5 with paroxysmal supraventricular tachycardia, 6 with persistent atrial flutter, 3 with persistent atrial fibrillation, and 2 with both atrial and ventricular arrhythmias (both of whom died in the immediate postoperative period). One patient experienced third-degree heart block. Five patients (12%) required pacemaker insertion before discharge from hospital; 3 patients required pacemakers after intraoperative Maze procedures, 1 patient had second-degree heart block, and 1 patient had complete heart block.
Mid-Term Outcomes
Of the late survivors, 95% (36 of 38) of patients had cardiac follow-up available after discharge from hospital. Two patients with CPS were known to be currently alive, but had not had recent cardiology follow-up (within 5 years). The mean follow-up time was 6.7 ± 4.8 years (5.8 ± 0.9 years in patients with a CPS versus 7.9 ± 1.4 years in patients without CPS). There were 5 deaths among the 36 adults (14%) after discharge from the hospital (range, 9 months to 4 years; Table 3). Two patients had sudden deaths: 1 patient without a CPS had a cardiac arrest 9 months after surgery and 1 patient with a CPS and a pacemaker died suddenly 2 years after surgery. One patient had a gastrointestinal bleed 4 years after surgery secondary to a malignancy. Overall, 5-year survival was 84% ± 7%, comparable in patients with or without CPS (83% ± 9% versus 86% ± 10%; p = 0.85; Fig 2).
View larger version (9K):
[in this window]
[in a new window]
|
Fig 2. Comparison of 5-year survival between patients with (dashed line) and without (solid line) cavopulmonary shunts (CPS).
|
|
Arrhythmias were common in patients with or without a CPS (55% versus 41%; p = 0.60) at 5 years after surgery. Eleven patients had persistent atrial tachyarrhythmias; 5 with atrial fibrillation, 5 with atrial flutter, and 1 with a nonspecific atrial tachycardia. Six additional patients had nonspecified paroxysmal atrial arrhythmias. One death was thought to be secondary to a fatal arrhythmia according to the autopsy report. Four patients required pacemaker insertion after discharge from the hospital for bradyarrhythmias including sick sinus syndrome. Two of these patients had had CPS and had epicardial pacemakers inserted at 2 years and 7 months postoperatively. Ninety-two percent of patients (3 of 36 patients) were in NYHA functional class I or II in follow-up. There were no patients in NYHA functional class IV in follow-up. Although there was a trend for heart failure to occur more commonly in patients without CPS compared with those with CPS (15% versus 0%; p = 0.07), follow-up duration was relatively longer in patients without CPS. Of the 4 patients undergoing repeat TV surgery after the index surgery (1 patient with a CPS and 3 patients without a CPS), TV regurgitation was present in 2 patients and TV stenosis was present in 2 patients.
|
Comment
|
|---|
Our study examines the early and mid-term outcomes in adult patients with Ebstein anomaly who had undergone repair or replacement of the TV with or without a concomitant CPS procedure. Patients with CPS were at risk for early and mid-term complications. Despite the fact that the patients who received a CPS were a higher-risk group than those who did not receive a CPS, the mid-term survival was similar in both groups. Ebstein surgery along with CPS appears to be a reasonable surgical strategy in patients not believed to be suitable for TV surgery alone.
Patients with Ebstein anomaly and significant RV dysfunction or small RVs are a surgical challenge. One option is to perform a CPS. A CPS is believed to improve the RV function in patients with significant RV dysfunction by reducing the RV preload [3, 4, 8, 9]. Chauvaud and associates [10] have shown that there is significant reduction of RV end-diastolic volume after surgery when CPS was used. A number of institutions have reported on use of this surgical technique [4, 11, 12]. Quinones and coworkers [4] reported a similar approach in which CPS is either planned preoperatively or as an intraoperative salvage maneuver. The criteria to add a CPS to the TV surgery in patients with Ebstein anomaly described by Chauvaud and colleagues [6] was similar to the practice at our institution and includes "significant TV dysfunction, extended atrialized right atriums, poor RV contractility, or long standing atrial fibrillation."
In our series, early outcomes in patients with Ebstein anomaly undergoing TV surgery were similar to those of some other series. Attenhofer and associates [1], in a large series from the Mayo Clinic (median age, 20 years; range, 2 months to 79.1 years), reported an early mortality rate of 5.4%. The early mortality in patients who have had a CPS along with TV surgery has been reported in pediatric or primarily pediatric populations. Chauvaud and coworkers [5] reported an early mortality of 0% in patients with CPS (n = 15; age range, 5 to 65 years) and 24.4% in patients who had not undergone a CPS (n = 45; age range, 4 to 55 years). In a subsequent publication, Chavaud and associates [6] described a 9% (18 of 191 patients) hospital mortality in a larger population of 191 patients with Ebstein anomaly undergoing TV surgery (mean age, 24.4 ± 15 years; range, 1 to 65 years). Cavopulmonary shunts were performed in 60 patients. In this series the authors state that the hospital mortality decreased from 12% to 7% after the introduction of the CPS procedure. In a multicenter study by Sarris and colleagues [7] (median age of 6 years; range, 1 day to 48 years) the operative mortality rate in patients with Ebstein anomaly who had undergone a CPS was 16.6% (7 of 42 patients).
Early arrhythmias were common (43%) in our series, and 12% of the patients required pacemaker insertion in the early postoperative period. Chauvaud and colleagues [13] reported a similar finding with 11% of patients in their series requiring early pacemaker insertion. This highlights the concern by some centers pertaining to the possibility of immediate or future problems with intravenous access and pacemaker insertions in patients who have had a CPS [14]. Prophylactic implantation of epicardial pacemaker leads at the time of surgery can overcome this problem. In our series, 2 patients required pacemakers after CPS, and epicardial devices were inserted. Surveillance of tachyarrhythmias is equally important.
Consistent with other case series, patients tend to have good functional capacity after surgery [6, 14]. Reoperation is not uncommon. This is similar to the Mayo Clinic experience, in which a reoperation rate of 16.7% (138 of 540 patients) in late follow-up (mean, 9.4 years) was reported [15]. In our series, there were no statistically significant differences in reoperation rates between those patients who had had a CPS and those who had not. Chauvaud and coworkers [5] reported reoperation rates of 11% in patients with Ebstein anomaly who did not have a CPS and reoperation rates of 0% in those who had CPS.
Overall survival (82% at 5 years) in our series was similar to the series described by Dearani and associates [15]. In another series from the Mayo clinic the percentage of late deaths was 7.6% (23 of 323 patients) after a mean follow-up of 7.1 years [16]. Despite the obvious bias to select patients for CPS with more significant RV dysfunction, we found no difference in 5-year survival in patients undergoing TV surgery with and without CPS. Chauvaud and colleagues [5] reported similar 5-year findings in a mixed pediatric and adult population (range, 5 to 65 years) who had undergone CPS along with TV surgery compared with patients who had undergone TV surgery alone (80% ± 16% versus 66.1% ± 14%; not significant).
Limitations
This was a retrospective review with the limitations of a retrospective study. Because of the long time span involved (1986 to 2005), differences in surgical and anesthetic techniques may have varied and subsequently affected outcomes. However, all surgeries were performed in one center predominantly by one surgeon, and this may minimize at least some of the bias introduced by the long follow-up period. Furthermore, the control group of patients who had valve surgery but no CPS serves as a useful reference group. Unfortunately, because of the retrospective study design, the long time span of the study, and difficulties, at the best of times, assessing the function of the RV in patients with Ebstein anomaly, we were unable to consistently obtain adequate preoperative cardiac imaging data to determine the baseline function of the RV. We have therefore elected not to report on RV systolic function. However, this information is important, and hopefully future studies will be able to examine outcomes in relation to the status of the functional RV. The objective assessment of the functional status by exercise testing and maximum oxygen consumption could not be reported because it was missing in a number of patients, in part because it is not always performed routinely after the operation and in part because of the patients' inability and motivation to have serial exercise testing. Finally, because our center is a referral center, not all patients had mid-term follow-up in our clinic. When possible, we arranged for patients to return to the clinic for at least one late follow-up visit. Nonetheless, we were unable to locate 2 patients.
Conclusions
Adolescent and adult patients with Ebstein anomaly undergoing TV replacement or repair and concomitant CPS are at risk for early and mid-term complications. Patients who received a CPS are a higher-risk group than those who do not receive a CPS. Despite this difference, the mid-term survival is similar in both groups. Ebstein surgery along with CPS appears to be a reasonable surgical strategy in patients not believed to be suitable for TV surgery alone.
|
Acknowledgments
|
|---|
CKS is supported by an operating grant from the Heart and Stroke Foundation of Canada.
|
References
|
|---|
- Attenhofer Jost CH, Connolly HM, Dearani JA, Edwards WD, Danielson GK. Ebstein's anomaly Circulation 2007;115:277-285.[Free Full Text]
- Watson H. Natural history of Ebstein's anomaly of the tricuspid valve in childhood and adolescence. An international cooperative study of 505 cases. Br Heart J 1974;36:417-427.[Free Full Text]
- Marcelletti C, Duren DB, Schuilenburg RM, Becker AE. Fontan's operation for Ebstein anomaly J Thorac Cardiovasc Surg 1980;79:63-66.[Abstract]
- Quinonez LG, Dearani JA, Puga FJ, et al. Result of the 1.5-ventricle repair for Ebstein anomaly and the failing right ventricle J Thorac Cardiovasc Surg 2007;133:1303-1310.[Abstract/Free Full Text]
- Chauvaud S, Fruzellier JF, Berrebi A. Bi-directional cavopulmonary shunt associated with ventriculo and valvulopasty in Ebstein's anomaly: benefits in high risk patients Eur J Cardiothorac Surg 1998;13:514-519.[Medline]
- Chauvaud S, Berrebi A, Mousseaux E, Hernigou A, Carpentier A. Ebstein's anomaly: repair based on functional analysis Eur J Cardiothorac Surg 2003;23:525-531.[Abstract/Free Full Text]
- Sarris G, Giannopoulos N, Ziemer G, et al. Results of surgery for Ebstein's anomaly: a multicenter study from the European Congenital Heart Surgeons Association J Thorac Cardiovasc Surg 2006;132:50-57.[Abstract/Free Full Text]
- Van Arsdell GS, Williams WG, Maser CM, et al. Superior vena cava to pulmonary artery anastomosis: an adjunct to biventricular repair J Thorac Cardiovasc Surg 1996;112:1143-1148.[Abstract/Free Full Text]
- Stellin G, Vida VL, Milanesi O, et al. Surgical treatment of complex cardiac anomalies: the "one and one half ventricle repair" Eur J Cardiothorac Surg 2002;22:1043-1049.[Abstract/Free Full Text]
- Marianeschi SM, McElhinney DB, Reddy VM, Silverman NH, Hanley FL. Alternative approach to the repair of Ebstein's malformation: intracardiac repair with ventricular unloading Ann Thorac Surg 1998;66:1546-1550.[Abstract/Free Full Text]
- Kreutzer C, Mayorquim RC, Kreutzer GOA, et al. Experience with one and a half ventricle repair J Thorac Cardiovasc Surg 1999;117:662-668.[Abstract/Free Full Text]
- Chauvaud SM, Heringou AC, Mousseaux ER, Sidi D, Hebert JL. Ventricular volumes in Ebstein's anomaly: x-ray multislice computed tomography before and after repair Ann Thorac Surg 2006;81:1443-1449.[Abstract/Free Full Text]
- Chauvaud SM, Brancaccio G, Carpentire AF. Cardiac arrhythmia in patients undergoing surgical repair of Ebstein's anomaly Ann Thorac Surg 2001;71:1547-1552.[Abstract/Free Full Text]
- Boston U, Dearani J, O'Leary P, Driscoll D, Danielson G. Tricuspid valve repair for Ebstein's anomaly in young children: 30-year experience Ann Thorac Surg 2006;81:690-696.[Abstract/Free Full Text]
- Dearani JA, Danielson GK. Surgical management of Ebstein's anomaly in the adult Semin Thorac Cardiovasc Surg 2005;17:148-154.[Medline]
- Theodoro DA, Danielson GK, Kiziltan HT, et al. Surgical management of Ebstein's anomaly: a 25-year experience Circulation 1997;96(Suppl 1):I-507.
Top
Abstract
Introduction
