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J Thorac Cardiovasc Surg 2007;133:1318-1328
© 2007 The American Association for Thoracic Surgery
Surgery for Congenital Heart Disease |
Department of Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women’s Medical University, Tokyo, Japan.
Read at the Eighty-sixth Annual Meeting of The American Association for Thoracic Surgery, Philadelphia, Pa, April 29–May 3, 2006
Received for publication July 25, 2006; revisions received October 25, 2006; accepted for publication November 3, 2006. * Address for reprints: Dr Toshiharu Shin’oka, Department of Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women’s Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 162-8666, Japan. (Email: ssinoka{at}hij.twmu.ac.jp).
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Abstract |
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 Top Abstract IntroductionMaterials and MethodsResultsDiscussionAppendixAppendix E1Appendix E2Appendix E3References |
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Methods: Between January 1972 and September 2005, a total of 189 patients (median age 8.3 years, range 2 months to 47 years old) with congenitally corrected transposition of the great arteries or double outlet right ventricle with discordant atrioventricular connections underwent definitive repairs. The definitive repairs comprised a conventional repair (atrial septal defect, or ventricular septal defect closure with or without pulmonary stenosis release, or isolated tricuspid valve surgery) in 36 patients (group I), conventional Rastelli in 31 patients (group II), double-switch operation (atrial switch plus arterial switch) in 15 patients (group III), atrial switch plus intraventricular rerouting (with or without extracardiac conduits) in 69 patients (group IV), and a Fontan-type repair in 38 patients (group V). The mean follow-up period was 10.1 years. Hospitalization and late mortality and reoperation were indicated as events. Risk factors for these events were analyzed by logistic regression for hospital death and a Cox proportional hazards model for late events.
Results: The Kaplan–Meier survival including hospital and late mortality was 62.4% at 32 years in group I, 78.5% at 27 years in group II, 74.5% at 15 years in group III, 80% at 16 years in group IV, and 79.3% at 22 years in group V. The reoperation-free ratio was 64.2% in group I, 76.6% in group II, 84.4% in group III, 89.6% in group IV, and 91.3% in group V. Risk analyses showed that the risk for hospital death was preoperative in patients with more than moderate tricuspid regurgitation and a cardiopulmonary bypass time of more than 240 minutes. A risk for late mortality was the presence of tricuspid regurgitation. Risks for reoperation were preoperative cardiomegaly, preoperative tricuspid regurgitation of more than grade II, ventricular septal defect enlargement, and body weight less than 10 kg. Risks for pacemaker implantation, as indicated by multivariate analysis, were ventricular septal defect enlargement during operation and age less than 3 years.
Conclusions: There were no statistical differences between long-term survival rates of patients who underwent conventional surgical repair versus those of patients who underwent anatomic surgical repair. Results of conventional repair were satisfactory except in patients with significant tricuspid regurgitation. Results of anatomic repair were also satisfactory even for patients with significant tricuspid regurgitation, and therefore, anatomic repair should be the procedure of choice for those patients.
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Introduction |
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TopAbstractIntroductionMaterials and MethodsResultsDiscussionAppendixAppendix E1Appendix E2Appendix E3References |
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Congenitally corrected transposition of the great arteries (cc-TGA) or atrioventricular (AV) discordance with a double-outlet right ventricle (l-DORV) are characterized by AV and ventriculoarterial discordance.1
These two entities were thought to differ only in the grade of overriding of the posterior pulmonary artery. When both septums in the heart are intact, the circulation is physiologically normal, with no shunt, no pressure load, and no cyanosis. However, the anatomic right ventricle and tricuspid valve must sustain a systemic pressure. Before 1983, in our institute, conventional repair was the procedure of choice. However, we found that, after conventional repair, some of our patients developed a systemic right ventricular (RV) dysfunction or tricuspid regurgitation (TR) over the long term. Because of the disappointing results with conventional repairs, the first patient with cc-TGA underwent a Mustard–Rastelli procedure that resulted in early mortality in 1983. In 1989, a second patient successfully survived a double-switch operation (DSO). Since then, anatomic repair has become the procedure of choice in our institute (Figure E1). The purpose of this study was to compare long-term outcomes after conventional versus anatomic repair for cc-TGA/ l-DORV. We also reviewed long-term results after a Fontan-type procedure for cc-TGA/ l-DORV, although this comparison is only for reference because the biventricular repair was not feasible in this group. In addition, we had 6 patients with anatomically corrected malposition who underwent definitive correction using the anatomic left ventricle as a systemic ventricle without any mortality. These patients were excluded in this study because systemic ventricles are the anatomic left ventricles after corrective surgery without any switch procedures.
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Materials and Methods |
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Briefly, the terms "right ventricle" and "left ventricle" describe ventricular morphology rather than spatial orientation. "Tricuspid valve" refers to the AV valve associated with the anatomic right ventricle regardless of its location. Regarding the surgical procedure, "conventional repair" includes a conventional repair and conventional Rastelli procedure using the anatomic right ventricle as a systemic ventricle, and "anatomical repair" indicates a DSO or Senning/Mustard plus Rastelli procedure in which the anatomic left ventricle functions as a systemic ventricle.
Patient Population and Data Acquisition
One hundred eighty-nine patients with segmental anatomy [SLL] (n = 147) or [IDD] (n = 42) who underwent definitive surgical repair at Tokyo Women’s Medical University between January 1972 and September 2005 were identified from the database of the Departments of Cardiovascular Surgery and Pediatric Cardiology. Medical records, preoperative and postoperative echocardiograph data, and cardiac catheterization data and operative notes were reviewed. Long-term follow-up was obtained by review of patient records and outpatient clinic notes. Institutional Review Board approval was obtained prior to the initiation of these retrospective analyses.
Diagnosis
Patient diagnoses were categorized in the following 6 groups according to the associated cardiac lesions (Table 1).
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Surgical Procedures
The surgical procedures comprised a conventional repair (ASD or VSD closure ± relief of PS, or isolated tricuspid valve surgery) in 36 patients (group I: conventional), conventional Rastelli in 31 patients (group II: conventional Rastelli), DSO (atrial switch plus arterial switch) in 15 patients (group III: DSO), atrial switch plus intraventricular rerouting (±extracardiac conduits) in 69 patients (group IV: M/S-Rastelli), and a Fontan-type repair in 38 patients (group V: Fontan).
Group I: Conventional Repair (1972–2003; n = 36)
Two patients had a complete form of AV septal defect; both defects were repaired by a conventional method, with 1 late death. Twelve of 36 patients required surgery for TR: valve replacement (TVR) in 8 patients and valve repairs in 4 patients. A mechanical valve was used in 6 patients, and a bioprosthetic valve was used in 2 patients. One patient with TVR had concomitant left ventricular (LV) assist device implantation with later heart transplantation. Three of 8 patients with TVR underwent PA banding for left ventricle training and failed.
The VSD was closed through the right atrium (RA) in 13 patients, through the right ventricle in 8 patients, through the aorta in 2 patients, and through the pulmonary artery (PA) in 1 patient. The PS was released in 15 patients without extracardiac conduits (patch LV outflow reconstruction in 2 patients, pulmonary valvotomy in 7 patients, and infundibular muscle resection in 6 patients). Two patients underwent debanding and pulmonary artery reconstruction. Two patients required surgical intervention to the anatomic mitral valve (annuloplasty in 1 patient and valve replacement in 1 patient).
Group II: Conventional Rastelli Procedure (1977–2004; n = 31)
In this group, the VSD was closed using DeLeval’s technique in 27 patients. Transaortic approach was used in 4 patients. LV-PA conduits were placed between the LV apex and the central portion of the pulmonary artery. Dacron conduits with a bioprosthetic valve were used as extracardiac conduits in 25 patients, Dacron conduits with an autologous pericardial valve in 2 patients, nonvalved Dacron conduit in 1 patient, xenopericardial valved conduit in 1 patient, and a valveless polytetrafluoroethylene conduit in 2 patients. TR was found in 3 patients and was repaired by annuloplasty. Mitral regurgitation was also found in 3 patients and was repaired by annuloplasty.
Group III: DSO (1980–2000; n = 15)
Of the 15 patients who underwent DSO, 14 had situs solitus and 13 had levocardia. As an atrial switch procedure, 12 patients underwent the Senning procedure and 3 had the Mustard procedure. All arterial switch procedures were performed using Lecompte’s modification. The VSD was closed in 12 patients through RA. Seven of 15 patients had previous PA banding for LV training. Significant TR was found in 10 patients, and 5 of 10 patients underwent tricuspid annuloplasty. There were 2 patients who had significant mitral regurgitation. One underwent mitral annuloplasty, and the other underwent a concomitant mitral valve replacement. Three patients had significant supraventricular arrhythmia, and 2 patients underwent surgical cryoablation.
Group IV: Senning or Mustard Plus Rastelli Procedure (1983–2003; n = 69)
In this group, 46 of 69 patients had PA. Forty-eight patients had situs solitus, and 21 had situs inversus. Twenty-one Senning and 48 Mustard procedures were performed as atrial switch procedures, depending on the size of the RA. Concomitant extensive PA angioplasty for branch PA stenosis was needed in 22 of 69 patients. RV-PA reconstruction was done by RV-PA direct anastomosis plus valved patch in 19 patients, autologous pericardial conduit in 22 patients, and xenopericardial conduit in 28 patients. When the diameter of the VSD (primary interventricular foramen) was less than half of the aortic annulus, the VSD was enlarged (n = 33). Preoperative electrophysiologic studies indicated the position of the VSD enlargement. If the patients had an anterior AV node, the VSD was enlarged posteriorly. Significant TR was found in 5 patients, and mitral regurgitation was found in 5 patients. Mitral valve or annuloplasty was performed in 5 patients. One patient needed a concomitant aortic valve replacement. Eighteen patients experienced paroxysmal supraventricular tachyarrhythmia, 16 patients underwent surgical ablation, and 5 patients had catheter ablation before or after the operation.
Group V: Fontan Procedure (1980–2004; n = 38)
Between 1980 and 2004, 38 patients with a diagnosis of cc-TGA or l-DORV underwent a Fontan procedure. Thirty-four patients underwent right atrial appendage–pulmonary artery anastomosis, and 4 patients underwent extracardiac total cavo-pulmonary connection with 4 hospital and 3 late deaths. Nine patients required tricuspid annuloplasty during the Fontan operation. No heart blocks occurred in this group.
Postoperative Outcome
Hospital mortality (HD) and late mortality (LD) and reoperation or reintervention were indicated as events. Pacemaker (PM) implantation was analyzed as a separate event from the reoperation. Risk factors for these events were analyzed by logistic regression for HD, and a Cox proportional hazards model was used for LD. Postoperative various hemodynamic data were compared among groups.
Statistical Analysis
Data were analyzed with statistical software (SPSS Windows 14.0, SPSS Inc, Chicago, Ill). The primary outcome variable was survival after the operation. Hospital death was defined as death in hospital after the operation, and late death was defined as death after discharge from the hospital. All death consisted of hospital death and late death. Reoperation events and pacemaker implantation events were also examined as outcome variables. Multiple clinical variables were analyzed for their possible effect on overall survival and event freedom by using the Cox proportional hazards model for categorical and continuous variables, in which cutoff points were also considered. Variables that were significant at the .1 levels in univariate analysis were included in a multivariate Cox proportional hazards model. A significant level of .05 was required for retention in the multivariate model. A logistic regression model was used for factorial analysis of hospital death. The Kaplan–Meier method was used to estimate probabilities of survival from all deaths and late deaths, as well as the probabilities of freedom from reoperation and PM implantation. Subgroups were compared with the use of the log–rank test. Patients lost to follow-up were excluded from analyses of survival and other outcomes.
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Results |
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TopAbstractIntroductionMaterials and MethodsResultsDiscussionAppendixAppendix E1Appendix E2Appendix E3References |
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Follow-up, Survival, and Events
Patients were followed for a median of 9.8 years (range, 0.1–33 years). The mean follow-up period was 10.1 years. Considering both in-hospital and late events, the Kaplan–Meier survival rate including HD and LD was 62.4% at 32 years in group I, 78.5% at 27 years in group II, 74.5% at 15 years in group III, 80% at 16 years in group IV, and 79.3% at 22 years in group V (Figure 1, A). No statistical difference between the anatomic and conventional repair groups was observed (Figure 1, B). However, patients with a preoperative significant TR showed a statistically worse survival rate than did those of the other groups (Figure 2, A). In addition, patients with significant TR after conventional repair showed a statistically worse result than that of the other groups (Figure 2, B). The reoperation-free ratio was 64.2% in group I, 76.6% in group II, 84.4% in group III, 89.6% in group IV, and 91.3% in group V (Figure 3, A). There was no statistical difference between the anatomic and conventional repair groups in the reoperation incidence (Figure 3, B). The probability of PM implantation was higher in the anatomic repair group than that of the conventional repair group (Figure 3, D).
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Analysis of Variance Among Groups in Postoperative Hemodynamic Data
Postoperative hemodynamic data were compared among groups. The results are shown in Table 2. The postoperative systemic ventricular volume and ejection fraction were comparable between the conventional and anatomic repair groups. The Fontan group showed the lowest CTR and the least cardiac output index with the highest RA pressure.
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Discussion |
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TopAbstractIntroductionMaterials and MethodsResultsDiscussionAppendixAppendix E1Appendix E2Appendix E3References |
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These patients are at risk of RV and TV dysfunction with increasing age, especially if Ebstein’s malformation of TV is present.9,10 The optimal surgical management of patients with cc-TGA/l-DORV is still to be determined. Although several high-volume studies have reported long-term results after conventional surgical repair using the anatomic right ventricle as a systemic ventricle,11-13 no study has compared the long-term results between conventional and anatomic repair for cc-TGA/l-DORV.
The longest surgical follow-up series in cc-TGA was reported in 1999 by Yeh and colleagues,14 who described the outcome of 127 patients over a 40-year time period at the Hospital for Sick Children in Toronto, Canada. The rate of 20-year survival in their series was 48%. In 2005, Hraska and colleagues12 analyzed 123 patients with cc-TGA. One hundred thirteen patients underwent intracardiac procedure, using either a traditional 2-ventricle repair (n = 96) or Fontan procedure (n = 17). They concluded that the poorest outcome was seen in patients who required tricuspid valve replacement either at the initial operation or later during follow-up; patients subjected to alternative approaches, such as Fontan, double-switch, or Senning–Rastelli procedure, may have better long-term results. The authors demonstrated a much better outcome in the Fontan patient group than the outcome of patients who underwent conventional surgical repair. However, the Fontan patients may have had lower cardiac output, and the follow-up periods described were relatively shorter than those of the other groups. In our experience, the survival rate after a Fontan procedure (RA appendage to PA anastomosis) seems to be satisfactory until 20 years after the operation. After 20 years, the survival curve dropped rapidly due to increased arrhythmia or heart failure. Therefore, we should be careful in using the Fontan procedure, even though the early results seemed to be good.
The concept of anatomic repair was introduced by Ilbawi and colleagues24 in 1990 and by Di Donato and associates in 199215 as a combined atrial switch and Rastelli procedure. Combined arterial switch and atrial repair as an anatomic repair for cc-TGA was reported by Imai and coworkers in 1994.16 The first occasion of a DSO consisting of Senning and arterial switch operation was performed on a 2-year-old boy with cc-TGA associated with grade 3 TR on June 12, 1989, and a combined Mustard–Rastelli was successfully performed on May 25, 1990.17 Mee18 stated that a Senning and arterial switch operation for cc-TGA was successfully performed in 1989. Imai and colleagues16 reported that early postoperative ventricular function after an anatomic repair was comparable to that of conventional repair and was even better in midterm results. The fact that the medium-term results indicated improved LV function and a favorable increase in cardiac output and LV ejection fraction with exercise suggests that temporary depression of the LV function in the immediate postoperative period may improve.
The outcome of patients with cc-TGA is variably affected by associated intracardiac defects, especially in the presence of tricuspid valve competence and systemic RV function. The importance of preoperative tricuspid valve regurgitation in conventional groups was reconfirmed in this study as in a previous study,12 although the mechanism of the TR was unknown and the incidence remains unpredictable. When patients do not have TR before their operation, the survival rate at 30 years after operation was about 72%, even in the conventional repair group. This value is quite satisfactory, and the results of an anatomic repair were not superior to those after conventional repair in our series. We speculate that the reasons for this phenomenon are as follows:
Recently, several groups have reported excellent early results after the double-switch procedure, bringing this procedure into the mainstream.21-23 However, additional long-term data are needed, and early enthusiasm must be tempered with objective evidence. Our study failed to demonstrate the superiority of anatomic repair compared with conventional repair when the patient had no TR before operation.
In our series, 35 Senning and 49 Mustard procedures were selected as atrial switch procedures, depending on the size of the RA. Recently, we suggested that the Senning procedure is feasible in all atrial switch procedures, applying a large pericardial patch to augment a new functional left atrium, as others have described.18,24,25 No difference was observed between the Senning and Mustard groups in long-term results (data not shown).
Conduction abnormalities are common in discordant AV connections, predisposing the patients to the development of complete heart block.26,27 The incidence of heart block was higher in the anatomic group, which was related to the fact that VSD enlargement was aggressively performed to relieve a potential subaortic stenosis in our series. The VSD enlargement was a risk factor for complete heart block/PM implantation, although it was not a risk factor for hospital and late death.
There is general agreement among authors concerning the anterior conduction system in the situs solitus group,28 whereas the posterior conduction system was reported in cc-TGA in situs inversus.29 However, we had several patients with an anterior conduction system in IDD hearts (16 of 21 patients examined) and also a dominant posterior conduction system even in an SLL heart (5 of 48 patients examined). Furthermore, a sling of conduction bundle was reported in cc-TGA in situs solitus with a straddling mitral valve.30 Therefore, the direction of the VSD enlargement should be carefully determined by means of preoperative electrophysiologic examination, which usually diagnoses the site and dominance of the AV node, in our experience.
In conclusion, no differences were observed between long-term survival rates of patients who underwent conventional versus anatomic surgical repair. Results of conventional repair were satisfactory except in patients with significant TR. Results of anatomic repairs were also satisfactory even in patients with significant TR, and therefore, it should be the procedure of choice for those patients.
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Appendix |
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TopAbstractIntroductionMaterials and MethodsResultsDiscussionAppendixAppendix E1Appendix E2Appendix E3References |
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Appendix E1 |
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TopAbstractIntroductionMaterials and MethodsResultsDiscussionAppendixAppendix E1Appendix E2Appendix E3References |
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Appendix E2 |
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TopAbstractIntroductionMaterials and MethodsResultsDiscussionAppendixAppendix E1Appendix E2Appendix E3References |
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Appendix E3 |
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TopAbstractIntroductionMaterials and MethodsResultsDiscussionAppendixAppendix E1Appendix E2Appendix E3References |
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References |
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TopAbstractIntroductionMaterials and MethodsResultsDiscussionAppendixAppendix E1Appendix E2Appendix E3References |
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