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J Thorac Cardiovasc Surg 2005;129:182-191
© 2005 The American Association for Thoracic Surgery

Surgery for Congenital Heart Disease

Long-term outcome of surgically treated patients with corrected transposition of the great arteries

^a Department of Pediatric Cardiac Surgery, University Hospital Hamburg–Eppendorf, Germany
^b Department of Pediatric Cardiac Surgery, Children's Hospital at the Cleveland Clinic, Cleveland, Ohio
^c Department of Cardiovascular Surgery, Children's Hospital, Boston, Mass
^d Department of Cardiology, Children's Hospital, Boston, Mass
^e Children's National Medical Center, Washington, DC

Received for publication December 2, 2003; accepted for publication February 26, 2004.

^* Address for reprints: Richard A. Jonas, MD, Cardiac Surgery, Children's National Medical Center, 111 Michigan Ave NW, Washington, DC 20010 (E-mail: rjonas{at}cnmc.org).

	Abstract

Top Abstract Methods Results Discussion Conclusions Appendix 1 Appendix 2 Appendix 3 References

 
OBJECTIVES: The purpose of the study was to examine long-term outcome after traditional surgical treatment of corrected transposition of the great arteries to provide a basis for comparison with new procedures, such as the double-switch or Senning-Rastelli procedures.

METHODS: Patient- and procedure-related variables in 123 patients with corrected transposition and 2 functional ventricles operated on between 1963 and 1996 were analyzed. Patients with intracardiac procedures underwent either a traditional 2-ventricle repair or a Fontan procedure.

RESULTS: The 1-, 5-, 10-, and 15-year survivals after the operation were 84%, 75%, 68%, and 61%, respectively. Patients requiring tricuspid valve replacement (27 patients) at any time during follow-up had a significantly worse outcome (P < .001; hazard ratio, 4.4), whereas the best outcome was seen in patients undergoing the Fontan procedure (17 patients, 0 deaths). Right ventricular end-diastolic pressure of greater than 17 mm Hg before the operation (P < .0001), complete heart block after the operation (P = .001), subvalvular pulmonary stenosis (P = .013), Ebstein malformation of the tricuspid valve (P = .025), and preoperative systemic (right) ventricular dysfunction (P = .041) were identified as risk factors for death at any time by means of univariate analysis. Ebstein malformation of the tricuspid valve (P = .036; hazard ratio, 1.5) was identified as a risk factor for death by multivariate analysis.

CONCLUSIONS: The long-term outcome of patients with corrected transposition after a classic surgical approach is unsatisfactory. The poorest outcome was seen in patients who required tricuspid valve replacement either at their initial operation or later during follow-up. Alternative surgical approaches, such as the double-switch, Senning-Rastelli, or Fontan procedures, are likely to have better long-term results, especially in the highest risk groups.

	Methods

Top Abstract Methods Results Discussion Conclusions Appendix 1 Appendix 2 Appendix 3 References

 
Patient population and data acquisition
One hundred twenty-three patients with 2 functional ventricles with segmental anatomy {S,L,L} or {I,D,D} operated on at Children's Hospital, Boston, between 1963 and 1996 were identified from the database of the Departments of Cardiac Surgery and Cardiology. Medical records, preoperative and postoperative echocardiographic data, and cardiac catheterization data and operative notes were reviewed. Long-term follow-up was obtained by review of patient records and clinic notes.

Patient-related variables
The morphology of associated lesions was classified on the basis of preoperative cardiac catheterization and echocardiography, as well as operative findings. Atrioventricular valve regurgitation was judged as absent, trivial, mild, moderate, or severe. Right (systemic) and left (pulmonary) ventricular function were judged as normal, mildly decreased, moderately decreased, or severely decreased. The onset of complete heart block (CHB) was assumed to occur at the time it was first documented by means of electrocardiography (Appendix 1).

Patients were placed into 6 diagnostic groups according to their underlying anatomy and pathophysiology: hemodynamically significant ventricular septal defect (VSD) with pulmonary stenosis (VSD+PS); hemodynamically significant VSD with pulmonary atresia (VSD+PA); hemodynamically significant VSD alone (VSD); hemodynamically significant VSD with tricuspid regurgitation (more than mild; VSD+TR); TR alone; or other (Figure 1).

View larger version (29K): [in this window] [in a new window]   Figure 1. Block analysis by diagnostic group. Numbers of total mortality are in brackets. L-TGA, Corrected transposition of the great arteries; VSD, ventricular septal defect; TR, tricuspid regurgitation; PS, pulmonary stenosis; PA, pulmonary atresia.

Postoperative outcome variables
TV dysfunction after the operation was defined as the need for TVR or TV function that deteriorated after the operation to moderate or severe incompetence. Right ventricular (RV) dysfunction after the operation was defined as being present when it was worse after the operation, with moderately or severely decreased function (Appendix 2).

Statistical analysis
Data were analyzed with a statistical program (JMP Statistical Analysis, Cary, NC). The primary outcome variable was nontransplanted survival after the operation. Early failure was defined as death or heart transplantation within 30 days of the operation, and late failure was defined as death or transplantation beyond 30 days after the operation. Multiple clinical variables were analyzed for their possible effect on overall survival by using the log-rank test for categorical variables and the Cox proportional hazards model for continuous variables. For continuous variables, cutoff points were also considered. Variables that were significant at the .1 level in univariate analysis were included in a multivariate Cox proportional hazards regression model. A significance level of .05 was required for retention in the multivariate model. The Kaplan-Maier method was used to estimate probabilities of freedom from reoperation, RV failure, and TV failure. Survival probabilities and 70% confidence intervals were calculated at 5-year intervals.

Subgroups were compared with the use of the log-rank test. The effect of TVR on survival was examined by using TVR as a time-varying covariate in a Cox proportional hazards model. Patients lost to follow-up were censored in analyses of survival and other outcomes.

	Results

Top Abstract Methods Results Discussion Conclusions Appendix 1 Appendix 2 Appendix 3 References

 
General
Between 1963 and 1996, 123 patients (49 female and 74 male patients) with ccTGA and associated lesions potentially suitable for biventricular repair underwent a surgical procedure. Associated lesions are listed in Appendix 1. One hundred three (84%) patients had {S,L,L} segmental anatomy, and 20 (16%) patients had {I,D,D} segmental anatomy.

Follow-up and survival
Patients were followed for a median of 5.2 years (range, 0.1-34 years). Considering both early and late events, the probability of survival from the time of the operation was 91% (70% confidence limits, 88%-94%) at 1 year, 81% (70% confidence limits, 77%-85%) at 5 years, 72% (70% confidence limits, 67%-77%) at 10 years, 67% (70% confidence limits, 61%-73%) at 15 years, and 59% (70% confidence limits, 52%-66%) at 20 years (Figure 2).

View larger version (13K): [in this window] [in a new window]   Figure 2. The probability of survival, in years, for the whole group (n = 123). Numbers of patients at risk are in parentheses. Error bars indicate 70% confidence limits.

Analysis by surgical approach: Palliative procedures versus intracardiac repair
Mortality by surgical approach is summarized in Figure 3. Causes of early and late death are summarized in Appendix 3.

View larger version (28K): [in this window] [in a new window]   Figure 3. Block analysis by surgical approach. Numbers of total mortality are in brackets. L-TGA, Corrected transposition of the great arteries; VSD, ventricular septal defect; TV, tricuspid valve.

Intracardiac procedure
An intracardiac procedure was performed in 113 patients, with 99 (88%) early survivors. The median age at the time of the operation was 4.1 years. The patients were followed for a median of 3.9 years, with 16 patients lost to follow-up. The 1-, 5-, 10-, and 15-year survivals were 84% (70% confidence limits, 80%-88%), 75% (70% confidence limits, 70%-80%), 68% (70% confidence limits, 62%-73%), and 61% (70% confidence limits, 53%-69%), respectively.

Significant differences (P = .006) were apparent among the surgical groups. Patients requiring TVR at their initial operation demonstrated the shortest survival (6/14 [43%] survivors). The best outcome was seen in patients undergoing a Fontan procedure (17 patients, 0 deaths, no heart block; Figure 4).

View larger version (14K): [in this window] [in a new window]   Figure 4. Survivals for the following operative groups: Fontan pathway (dotted line; n = 17), VSD surgery (solid line; n = 76), and TV surgery (dashed line; n = 14). Numbers of patients at risk are in parentheses. Error bars indicate 70% confidence limits. VSD, Ventricular septal defect; TV, tricuspid valve.

View larger version (13K): [in this window] [in a new window]   Figure 5. Survivals for patients with CHB (dotted line; n = 35) and without CHB (solid line; n = 78). Numbers of patients at risk are in parentheses. Error bars indicate 70% confidence limits. CHB, Complete heart block.

View larger version (10K): [in this window] [in a new window]   Figure 6. Freedom from right ventricular dysfunction after 2-ventricle intracardiac procedure (75 patients analyzed). Numbers of patients at risk are in parentheses. Error bars indicate 70% confidence limits. RV, Right ventricular.

View larger version (9K): [in this window] [in a new window]   Figure 7. Freedom from tricuspid valve dysfunction after intracardiac procedure (83 patients analyzed). Numbers of patients at risk are in parentheses. Error bars indicate 70% confidence limits. TV, Tricuspid valve.

	Discussion

Top Abstract Methods Results Discussion Conclusions Appendix 1 Appendix 2 Appendix 3 References

 
Optimal surgical management of patients with ccTGA and associated lesions remains controversial.^3-8 The ability of the right ventricle to support the systemic circulation over a normal lifespan remains a serious concern.^6,9,10 This report analyzes the long-term survival of a large group of patients with ccTGA who underwent repair of associated lesions at a single institution. The overall 1-, 5-, 10-, and 15-year survivals after intracardiac procedures were 84%, 75%, 68%, and 61%, respectively. Significant differences were apparent among the surgical groups. Patients requiring TVR at the initial operation had the poorest outlook, whereas the best outcome was seen in patients undergoing the Fontan procedure.

Risk factors for TR and influence on outcome
Significant TV regurgitation after surgical intervention occurred in 42% of patients. Late development of TV dysfunction was associated with Ebstein malformation of TV and RV dysfunction before the operation. It has been reported that significant TR develops postoperatively in many patients with corrected transposition, especially in patients with coexisting TV abnormalities.^3,7,11 TV regurgitation results from a combination of annular dilatation and the high incidence of morphologically TV abnormalities present in these patients. RV dysfunction after surgical intervention associated with changes in RV geometry is likely to further contribute to deterioration of TV function. Increasing valvular regurgitation leading to progressive volume overload of the RV, followed by annular dilatation and further regurgitation, constitutes a reinforcing cycle of TV dysfunction. Splinting of the interventricular septum by increased morphologically left ventricular pressure either naturally by means of PS or by banding could help to maintain proper geometry of the right ventricle and could prevent progression of TV regurgitation. The favorable natural history of corrected transposition with PS in comparison with the other clinical subsets supports this argument. Other evidence is banding of the PA as an interim step in preparation of the LV for systemic function, which usually immediately improves systemic TV function because of geometric changes of the interventricular septum. Whether natural banding (PS) or banding itself in selected group of patients with ccTGA could be considered as a definitive palliation remains to be seen. In the present study, Ebstein malformation of the TV was not only a risk factor for late development of TR but also was an independent preoperative predictor of mortality. RV intolerance of the increased afterload resulting from isolated VSD closure also might contribute to the deterioration of TV function. In addition, the method for VSD closure, placing sutures on the morphologically RV side of the septum, requires suture placement either extremely close to the TV or even incorporating TV tissue. Thus, the subsequent deterioration in TV function after the surgical intervention might be not just a consequence of RV shape and size or Ebstein-like malformation but also a consequence of surgical distortion and scarring.¹² On the basis of our data, deterioration of systemic RV function was not delayed by TVR. In fact, patients who required TVR at the initial operation or during the follow-up had significantly poorer outcomes. Furthermore, the need for TVR was a significant predictor of RV dysfunction. Other reports advocate replacement of the TV in patients with ccTGA at the earliest sign of increasing symptoms or progressive systemic ventricular deterioration to preserve RV function.⁷ Therefore, the surgical management should include consideration of earlier timing of TVR or a cardiac repair, which relieves the RV and TV, of a systemic workload.¹³

Risk factors for RV dysfunction and influence on outcome
The ability of the right ventricle to function as a systemic ventricle throughout a normal lifetime remains unproved. Although previous reports have demonstrated that in most cases function remains satisfactory during an intermediate period of follow-up,^9,10,11,13 nevertheless, the 1-, 5-, 10-, 15-, and 20-year freedom from RV dysfunction after intracardiac procedure was 85%, 73%, 57%, 43%, and 39%, respectively. Our results demonstrate that postoperative RV dysfunction occurred in 44% of patients. Significant risk factors for RV dysfunction were Ebstein malformation of the TV, the need for TVR, and CHB. This finding is consistent with the report of Sano and colleagues,¹¹ in which 55% of patients had RV dysfunction after an intracardiac repair. In that study, 2 factors were responsible for late development of RV dysfunction; the first, postoperative TR, is consistent with our data (need for TVR). They further demonstrated that the pulmonary to systemic flow ratio was significantly higher in patients who had RV dysfunction than in those who did not. We did not find a correlation between higher Qp/Qs and postoperative RV dysfunction, although the diagnostic group VSD+PS with limited pulmonary blood flow had the best outcome.^11,14,15

Risk factors for CHB and influence on outcome
The incidence of perioperative CHB in the present study (28%) is comparable with that seen in other series.^3,4,8,11 Patients without CHB demonstrated a significantly better outcome (84% survival at 15 years of follow-up) compared with patients with CHB (56% survival at 15 years of follow-up). The incidence of CHB significantly decreased after 1981 because of implementation of a new approach for VSD closure.¹⁶

Current strategies for operative approach
Alternative surgical approaches are a single-ventricle type of repair (Fontan procedure) or an anatomic correction that maintains the left ventricle as the systemic ventricle. The present results for patients with ccTGA undergoing the Fontan procedure confirm the promising long-term outlook suggested by previous reports for this anatomic subgroup of patients.¹⁷ This approach eliminates the surgical risks of CHB and acceleration of progressive TR after VSD closure. Another potential advantage results from retaining both ventricles in the systemic circulation. In the case of complex VSD and TV anatomy or abnormal systemic or pulmonary venous return, this approach is fully justifiable, with excellent intermediate-term results. In the present study, 17 patients who underwent a Fontan procedure had the best outcome, with no deaths and no postoperative heart block with preservation of good TV function.

The main theoretical advantage of anatomic correction (double switch or Senning-Rastelli) arises from its use of the LV as the systemic pumping chamber and the mitral valve as the systemic atrioventricular valve. Performing an anatomic correction should be strongly considered for patients with TR before surgical intervention, Ebstein malformation of the TV, and RV dysfunction.^13,18-25 Heart transplantation remains another option for this difficult patient population but requires immune suppression and provides an uncertain long-term outlook.

	Conclusions

Top Abstract Methods Results Discussion Conclusions Appendix 1 Appendix 2 Appendix 3 References

 
The long-term outcome of patients with ccTGA who have undergone a classic surgical approach is unsatisfactory. Systemic RV dysfunction and TV dysfunction predict poor outcomes for many of these patients. The poorest outcome was seen in patients who required TVR either at their initial operation or later during follow-up. Alternative surgical approaches, such as anatomic correction or the Fontan procedure, might lead to improved results, especially in the highest-risk groups. The Fontan procedure, leaving the VSD untouched, might be the preferable option if there is an unfavorable anatomic location of the VSD or straddling of the atrioventricular valves. Otherwise, the performance of an anatomic correction with the LV as the systemic ventricle should be considered. The advantages of an anatomic correction should be balanced against the potential long-term problems resulting from the atrial switch portion of the procedure.

	Appendix 1

Top Abstract Methods Results Discussion Conclusions Appendix 1 Appendix 2 Appendix 3 References

 

	Appendix 2

Top Abstract Methods Results Discussion Conclusions Appendix 1 Appendix 2 Appendix 3 References

	Appendix 3

Top Abstract Methods Results Discussion Conclusions Appendix 1 Appendix 2 Appendix 3 References

	Acknowledgments

 
We thank Kimberlee Geavreau, ScD, for her invaluable assistance in the statistical analysis of the data for this report.

	References

Top Abstract Methods Results Discussion Conclusions Appendix 1 Appendix 2 Appendix 3 References

 

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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): Viktor Hraska Brian W. Duncan John E. Mayer, Jr Pedro J. del Nido Richard A. Jonas Permission Requests Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Hraska, V. Articles by Jonas, R. A. Search for Related Content PubMed PubMed Citation Articles by Hraska, V. Articles by Jonas, R. A. Related Collections Congenital - acyanotic Congenital - cyanotic