- Email: [email protected]
Rastelli Operation for Transposition of the Great Arteries With Ventricular Septal Defect and Pulmonary Stenosis
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Rastelli Operation for Transposition of the Great Arteries With Ventricular Septal Defect and Pulmonary Stenosis John W. Brown, MD, Mark Ruzmetov, MD, PhD, Daniel Huynh, BS, Mark D. Rodefeld, MD, Mark W. Turrentine, MD, and Andrew C. Fiore, MD Section of Cardiothoracic Surgery, Indiana University School of Medicine, Indianapolis, Indiana; and St. Louis University School of Medicine, St. Louis, Missouri
Background. The optimal surgical treatment of patients with transposition of the great arteries, ventricular septal defect, and pulmonary stenosis is controversial. Although the Rastelli operation has been standard surgical management of this lesion, aortic root translocation with right ventricular outflow tract (RVOT) reconstruction (Nikaidoh) and the pulmonary artery translocation (Lecompte) or REV (réparation a l’étage ventriculaire) are surgical alternatives more recently introduced to treat this complex lesion. This report reviews our 20-year experience with the Rastelli procedure and attempts to compare our outcomes with those recently published using the Nikaidoh and REV procedures. Methods. Between 1988 and 2008, 40 patients (median age, 4 years; range, 9 months to 17 years) underwent Rastelli operation at our institutions. The RVOT was obstructed in 32 and atretic in 8. Follow-up was available for all but one patient (mean follow-up, 8.6 ⴞ 5.6 years). The RVOT was reconstructed with homograft (n ⴝ 25), bovine jugular vein (n ⴝ 8), nonvalved Dacron tube (n ⴝ 5), or a porcine valved conduit (n ⴝ 2). Two patients required a pacemaker. Results. There were no early, but three late deaths and one heart transplantation 12 years postoperative the Rastelli operation. Kaplan-Meier survival was 93% at 5, 10, and 20 years. Univariate risk factors for death or trans-
plantation included surgery before 1998 (p ⴝ 0.03) and concomitant noncardiac anomalies (p ⴝ 0.001). Sixteen patients (40%) had reoperation for right ventricularpulmonary artery conduit stenosis (mean, 7.8 ⴞ 3.8 years) without mortality. Freedom from conduit replacement was 86%, 74%, 63%, and 59% at 5, 10, 15, and 20 years, respectively. Multivariate analysis revealed that the risk factors of conduit replacement were younger age at operation (p ⴝ 0.001) and surgery before 1998 (p < 0.001). Two patients (5%) required reoperation for left ventricular outflow tract obstruction. At follow-up, there were no sudden unexplained deaths, and New York Heart Association functional class is I or II. Conclusions. The Rastelli procedure is a low-risk operation with regard to early and late mortality and reoperation for left ventricular outflow tract obstruction. Conduit change operations will be required in most patients regardless of the technique of repair, but currently can be performed with low morbidity and mortality. These midterm outcomes after the Rastelli operation should serve as a basis for comparison with surgical alternatives more recently introduced for transposition of the great arteries and ventricular septal defect with RVOT obstruction. (Ann Thorac Surg 2011;91:188 –94) © 2011 by The Society of Thoracic Surgeons
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technique (réparation a l’étage ventriculaire) to reconstruct the pulmonary outflow tract without using a prosthetic conduit as an alternative to the Rastelli repair. Two years later, Nikaidoh [4] proposed aortic translocation and biventricular outflow tract reconstruction for the management of TGA with VSD and PS. Each of these techniques has advantages and disadvantages. Controversy surrounds the optimal surgical procedure for TGA, VSD, and PS with respect to mortality, morbidity, and long-term results. The fate of the reconstructed left and right ventricular outflow tract (LVOT; RVOT) plays an essential role in defining longterm prognosis in these patients. The RO requires late right ventricle (RV) to pulmonary artery (PA) extracardiac conduit replacements and may be associated with recurrent LVOT obstruction (LVOTO) and reduce long-
urgical management of patients with transposition of the great arteries (TGA), ventricular septal defect (VSD), and pulmonary stenosis (PS) continues to be a challenge, because anatomic correction requires reconstruction of both ventricular outflow tracts. Three major surgical techniques have been developed during the past four decades. The Rastelli operation (RO) was first introduced in 1969 and soon became the standard surgical treatment for patients with TGA, VSD, and PS [1, 2]. In 1980, Lecompte and associates [3] introduced the REV
Accepted for publication July 19, 2010. Presented at the Fifty-sixth Annual Meeting of the Southern Thoracic Surgical Association, Marco Island, FL, Nov 4 –7, 2009. Address correspondence to Dr Brown, Section of Cardiothoracic Surgery, Indiana University School of Medicine, 545 Barnhill Dr, EH 215, Indianapolis, IN 46202-5123; e-mail: [email protected].
© 2011 by The Society of Thoracic Surgeons Published by Elsevier Inc
0003-4975/$36.00 doi:10.1016/j.athoracsur.2010.07.057
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Table 1. Associated Lesions
term survival. The REV procedure radically removes the infundibular septum, theoretically preventing LVOTO, but employs direct PA to RV connection with or without a monocusp valve. This procedure has a higher earlier mortality and also requires revision of the RVOT. Since 1988, the RO has been practiced at our respective institutions. We reviewed our entire 21-year experience involving the surgical treatment of TGA, VSD, and PS. The aim of the present study is to review our surgical experience with the RO in this patient population to analyze early and late mortality, morbidity, and need for reoperation.
Material and Methods Our institutional review boards approved this study, and individual consent was not required. All patients who underwent a Rastelli operation for TGA and PS between 1988 and 2009 at James Whitcomb Riley Children’s Hospital in Indiana University, Indianapolis, Indiana (n ⫽ 34) and Cardinal Glennon Children’s Hospital at St. Louis University, St. Louis, Missouri (n ⫽ 6), were included in this retrospective study. Patients with double-outlet right ventricle were excluded. Median age at the time of operation was 4 years (range, 9 months to 17 years) and median weight was 23 kg (range, 5.2 to 102 kg). There were 24 male and 16 female patients. Palliative shunt operations (modified BlalockTaussig shunts, central shunts) were required in 36; 7 of our patients had balloon or surgical atrial septostomy without a shunt in 4. All patients had normal ventricular morphology. Five patients (12.5%) presented with pulmonary atresia. Associated lesions are shown in Table 1.
Surgical Technique Median sternotomy using conventional cardiopulmonary bypass with bicaval cannulation and cold potassium blood cardioplegic solution was employed in all patients. The median cardiopulmonary bypass time was 178 minutes (range, 120 to 340) while the median ischemic time was 98 minutes (range, 60 to 173). All operations were
Patients Lesion Cardiac Atrial septal defect Coronary artery anomaly Left superior vena cava Subaortic stenosis Juxtaposed atrial appendages on the left side TAPVR Unroofed coronary sinus Azygous continuation of inferior vena cava MAPCA (right) Noncardiac Spina bifida Budd-Chiari malformation Klippel-Feil syndrome Goldenhar syndrome MAPCA ⫽ multiple aortopulmonary collaterals; anomalous pulmonary vein return.
No
%
14 7 1 2 2 1 1 1 1
35 18 2.5 5 5 2.5 2.5 2.5 2.5
2 1 1 1
5 2.5 2.5 2.5
TAPVR ⫽ total
performed with hypothermia, mean rectal temperature of 27°C, and left ventricular venting. All systemic to pulmonary shunts were ligated and divided. In 3 patients (8%), the VSD was enlarged at the anterosuperior margin of the defect. The VSD was baffled to the aorta using a gluteraldehyde preserved autologous pericardial patch or a polytetrafluoroethylene patch (Gore-Tex; W. L. Gore & Assoc, Flagstaff, AZ). The patch was sutured in place with continuous suture in 12 patients (30%), by interrupted pledgeted sutures in 5 patients (13%), and by a combination of both in the remaining patients. In two cases, a papillary muscle from the tricuspid valve straddled the LVOT and was resected with its attached chordae tendineae. After the Gore-Tex patch was inserted, the papillary muscle was reimplanted into an appropriate spot on the RV side of the tunnel. In patients with late subaortic obstruction, the conduit is removed from the RV after cardioplegic arrest. The VSD patch is opened longitudinally and resected, leaving a 1 to 2 mm rim of patch near the conduction system. The subaortic stenosis is removed, in addition to any scar tissue along the pathway between the VSD and the aortic valve. If necessary, the VSD is enlarged so that its diameter is equal to or 1 to 2 mm greater than the diameter of the aortic annulus. An appropriately shaped patch is then inserted and sewn with continuous monofilament suture. Inferiorly, the rim of the old patch is used to avoid injury to the conduction system. Subsequently, the aortic cross-clamp is removed (if no atrial septal defect is present) and the RV anastomosis is completed with the heart beating during rewarming. Alternatively, a new conduit can be inserted. The RV to PA connection was reconstructed with a homograft in 25 patients (62%), bovine jugular vein conduit (Contegra; Medtronic Inc, Minneapolis, MN) in 8
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Abbreviations and Acronyms LVOTO ⫽ left ventricular outflow tract obstruction MAPCA ⫽ multiple aortopulmonary collaterals PA ⫽ pulmonary artery PS ⫽ pulmonary stenosis REV ⫽ réparation a l’étage ventriculaire RO ⫽ Rastelli operation RV ⫽ right ventricular RVOTO ⫽ right ventricular outflow tract obstruction TAPVR ⫽ total anomalous pulmonary vein return TGA ⫽ transposition of the great arteries VSD ⫽ ventricular septal defect
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(20%), polyethylene terephthalate fiber (Dacron; DuPont, Wilmington, DE) nonvalved graft conduit in 5 patients (13%), and with a Freestyle porcine root (Medtronic Inc) in 2 patients (5%). The mean size of the RV-PA conduit was 17.3 ⫾ 2.3 mm (range, 13 to 22 mm). In order to avoid valve distortion after sternal closure, we inserted the conduit to the left of midline into the left pulmonary artery and shortened the conduit outflow end so that the pulmonary valve rests near the pulmonary artery bifurcation. Gore-Tex membrane is used in all patients to cover the conduit, innominate vein, ascending aorta, and right ventricle so as to avoid injury during reoperation. Concomitant procedures are shown in Table 2.
Follow-Up One patient was lost to follow-up at 2 years postoperatively. The functional status was determined according to the New York Heart Association class. On follow-up examinations, patients underwent echocardiography and electrocardiography. Ventricular function was graded as normal, mild, moderate, or severely impaired. Insufficiency of the atrioventricular valve, the aortic valve, and the conduit valve in the RVOT was graded as none, mild, moderate, or severe. The LVOTO and RVOTO at latest follow-up were graded as mild stenosis when the maximal instantaneous pressure gradient was less than 30 mm Hg, moderate stenosis when the gradient was 30 to 60 mm Hg, and severe stenosis when the gradient was greater than 60 mm Hg.
Statistical Analysis Measured and calculated data are expressed as mean ⫾ SD. The Kaplan-Meier product limit method and Cox proportional hazards regression methods were used for analysis of survival and freedom from reoperation for RVOTO, LVOTO, residual VSD, or pacemaker implantation. Multiple regression analysis was performed as conditional backward stepwise proportional hazards regression. In the analysis of risk factors for mortality or heart transplantation, freedom from any type of reoperation, variables with significance levels of 0.1 in univariate analysis were submitted to a multivariate logistic regression model. Early mortality was defined as death during initial hospitalization or within 30 days of operation. Any Table 2. Concomitant Procedures During Rastelli Operation Patients Procedure
No
%
Atrial septal defect closure Ventricular septal defect enlargement Left superior vena cava ligation Unroofed coronary sinus repair Subaortic membrane resection TAPVR repair Right MAPCA ligation
14 3 1 1 2 1 1
35 8 2.5 2.5 5 2.5 2.5
MAPCA ⫽ multiple aortopulmonary collaterals; anomalous pulmonary vein return.
TAPVR ⫽ total
deaths later than that were defined as late mortality. A p value of less than 0.05 was considered significant. Specific statistical software SPSS for Windows version 10 (SPSS Inc, Chicago, IL) was used for data analysis.
Results Mortality and Morbidity There were no hospital deaths. Median hospital stay was 12 days (range, 6 to 52 days). There was one early reoperation for bleeding. Two patients (5%) developed permanent atrioventricular block postoperatively and required a permanent pacemaker. The incidence of postoperative permanent atrioventricular block was not significantly higher in patients who had VSD enlargement. Within a mean follow-up of 8.6 ⫹ 5.6 years (range, 3 months to 21 years), there were 3 late deaths and one cardiac transplantation. Kaplan-Meier survival was 93% at 5, 10, and 20 years. Freedom from death or cardiac transplantation was 92% ⫾ 4% and 90% ⫾ 5% at 10 and 20 years, respectively (Fig 1). The first death occurred in a patient with coronary artery anomaly (RV branches arising from the left coronary artery) who died suddenly 1 year postoperatively. This patient had documented sinus rhythm and no LVOTO or RVOTO. The second death presented with multiple stenoses of the peripheral and central pulmonary arteries and Klippel-Feil syndrome, with multiple skeletal and rib anomalies. She died of low cardiac output 3 months after the RO. The third death was in a patient with a myelomeningocele who expired at another hospital 6 months after RO secondary to pneumonia and sepsis. A fourth patient underwent two reinterventions for pacemaker exchange due to complete atrioventricular block. Before cardiac transplantation (12 years after RO), he presented with severely impaired right and left ventricular function with end-stage heart failure and ejection fraction of 15%. Univariate risk factors for death or transplantation included surgery before 1998 (p ⫽ 0.03) and concomitant noncardiac anomalies (p ⫽ 0.001).
Reoperations During follow-up, 18 patients (45%) underwent reoperations: sixteen patients required conduit change for RVOTO and two patients required reoperation for LVOTO. Two patients with LVOTO had scar formation within the baffle pathway from the VSD to the aortic valve. Freedom from reoperation for LVOTO or conduit change at 20 years was 95% ⫾ 2 % and 60 % ⫾ 6.4%, respectively (Fig 2). The average time to conduit failure was 7.8 ⫾ 3.8 years (range, 2 to 16 years). The mean age at reintervention was 12 ⫾ 6.0 years (range, 4 to 27 years). The peak systolic gradient across the RVOT before conduit replacement was 59 ⫾ 8 mm Hg (range, 30 to 80 mm Hg). The most common indication for a conduit replacement was stenosis, which was present in 14 (88%) of the 16 conduit replacement procedures. A less frequent primary indication for conduit replacement was conduit
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Information concerning the functional status at the time of final follow-up could be obtained from 36 patients (excluding lost and late death patients). Accordingly, 97% of the patients were in New York Heart Association class I or II, and 3% in class III. The majority of the patients were leading a normal life with full-time work or school (ability index class I or II), and only one patient experienced noticeable limitations of activities (class III).
Comment
Fig 1. Survival curve in patients with Rastelli operation.
valve insufficiency noted in two patients (12%). All conduits that required replacement had varying degrees of insufficiency, graded as mild in 6, moderate in 8, and severe in 2. Thirteen patients underwent replacement with a second biologic valve, whereas 3 children had their conduit revised with the insertion of a polytetrafluoroethylene monocusp valve. Multivariate analysis revealed risk factors for conduit replacement were younger age at operation (p ⫽ 0.001) and surgery before 1998 (p ⬍ 0.001). Additional operations at time of conduit change were required: closure of residual VSD in 2; closure of atrial septal defect in 1; and muscle resection for infundibular obstruction in 1 patient.
Follow-Up The mean follow-up period for children after RO is 8.6 ⫾ 5.6 years (range, 3 months to 21 years). Mean age of the patients at final follow-up was 13.6 ⫾ 7.7 years. Echocardiographic and electrocardiographic data were obtained at last follow-up in 18 patients without reoperation (all patients had their original conduit). Left ventricular function was normal in 95% of the patients, while freedom from LVOTO or aortic insufficiency was 89% and 95%, respectively. One patient had a 22 mm Hg LVOTO gradient, and another had mild aortic insufficiency. Right ventricular function was normal in 89% of the patients; 89% presented with no RVOTO and 100% with no or mild pulmonary insufficiency. Two patients presented with moderate stenosis of the conduit (41 and 47 mm Hg) and are being followed with serial echocardiography. At last follow-up, sinus rhythm with complete right bundle branch block was present in 31 patients. Two patients who received pacemaker implantation post RO had pacemaker rhythm at final follow-up. In 3 patients, permanent pacemaker implantation was performed after hospital discharge for complete atrioventricular block (n ⫽ 2) and sick sinus syndrome (n ⫽ 1). Freedom from pacemaker implantation was 92% and 86%, at 5 and 20 years, respectively. Enlargement of the VSD at the time of the RO had no significant influence on freedom from pacemaker implantation (p ⫽ 0.36).
The optimal anatomic surgical repair of patients with TGA, VSD, and PS is controversial. The Rastelli operation has become the “gold standard” in the surgical management in patients with this pathology. The RO in its original description directed the LV blood to the aorta by suturing a Teflon patch (Boston Scientific Corp, Meditec, NJ) between the VSD and the aortic valve orifice and establishing continuity from the RV to the PA with a valved conduit [1, 2]. In 1984, Nikaidoh [4] published a new surgical repair for TGA, VSD, and PS. This operation achieves an anatomic repair that utilizes aortic root mobilization and transfer to the LV combined with reconstruction of the RVOT and LVOT tracts. The potential advantage of this technique is that mobilization of the aortic root and direct anastomosis to the LV is accomplished with the coronary arteries intact and obviates the need for coronary artery transfer and the potential hazard of early or late ostial stenosis. However, the RVOT is devoid of a functioning valve and after requires valved conduit at a subsequent surgery. An important variation on the theme of RO was published by Lecompte and his associates in 1982 [3]. This operation was called “reparation a l’etage ventriculaire” or the REV procedure. This technique departs from the classic RO in that the infundibular septum is totally resected providing a wide, nonobstructed tunnel from the LV to the aorta, avoiding the potential for subaortic stenosis. The pulmonary artery is directly connected to the RV and a monocusp valve is inserted [3, 5].
Fig 2. Freedom from reoperation curve in patients with Rastelli operation.
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Metras and associates [6] have published a modification of the Rastelli-REV procedures. Rather than a direct connection between the RV and the PA, they use an aortic autograft taken from the patients’ ascending aorta to facilitate this connection. The authors favor this approach because the classic REV procedure with a direct RV to PA connection places tension on the pulmonary artery resulting in frequent reinterventions.
Survival In the earlier published series with RO, mortality was high [7, 8]. Dearani and associates [8] reviewed 160 hospital survivors operated on between 1968 and 1990, who were followed for at least 10 years. They found an actuarial survival of 74% at 10 years and 59% at 20 years. In their series, mortality decreased from 24% for the years 1968 through 1977 to 4% from 1988 to 1997. Kreutzer and associates [7] reviewed the results of the RO in 101 patients at the Boston Children’s Hospital operated on from 1973 to 1998 and operative mortality was 7%, with no operative deaths in the last 7 years of the study. During the last 3 to 5 years, there have been several new reports of patients who underwent the RO with mortality less than 5% [9 –11]. In this series, there are no early mortality but the late mortality decreased from 15% for the years 1990 through 1998 to 4% from 1998 to 2008; 2 of the 4 adverse outcomes were in patients with severe noncardiac anomalies. Similar low mortality rates were reported by Yeh and Morell and associates (5%) in patients with the Nikaidoh procedure [12–15] and by Prêtre (8%) [5] and Lecompte (6%) [3] and associates in patients with the REV procedure.
Reoperations on RVOTO and LVOTO Freedom from the RVOT reoperation was reported to be significantly higher after the REV procedure, compared with the RO [5, 9, 15–17]. One disadvantage of the REV procedure is that it cannot be applied to patients with pulmonary atresia. In addition, the pulmonary artery bifurcation is placed anterior to the aorta (Lecompte maneuver) which may lead to tension and sternal compression of the branch pulmonary arteries as they pass over the enlarged ascending aorta [17]. Potentially, this may result in chronic elevation of RV pressure. The fate of the RVOT is an important consideration when evaluating the effectiveness of the REV procedure. Lee and associates [18] experienced RVOTO in 25% of patients after the REV procedure. Vouhé and associates [16] reported that RVOTO occurred in 26% of their patients, with a mean follow-up of 55 months. Late LVOTO may be an important late complication after the RO [19, 20]. The obstruction is usually located at the level of the VSD or beneath the aortic valve. The LVOTO may persist because the VSD was restrictive at initial operation and not sufficiently enlarged or it may develop as a result of muscular hypertrophy within the outflow tract. Rychik and associates [21] have postulated that in TGA, as in double-inlet LV, the VSD or bulboventricular foramen becomes restrictive after biventricular repair or Fontan operation because LV volume is often
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increased before correction and decreases after the RO. Kreutzer and associates [7] reported freedom from LVOTO reintervention of 84% at 15 years in contrast to 97% at 20 years in our cohort. Hörer and associates [19] reported a freedom from LVOTO to be 93% at 15 years and explained that LVOTO may be related to inadequate enlargement of the VSD (59% of their patients). Hörer and associates noted that resecting the conal septum or the anterosuperior margin can enlarge even a small restrictive VSD to the size of the aortic annulus and provide a straight nonobstructed pathway along the LVOT. Hörer and associates’ higher freedom from LVOTO may also be explained by the higher median age at the time of RO when compared with the cohort reported by Kreutzer and associates [7] (4.5 vs 3.1 years). Hörer and associates reported that age younger than 1 year at the initial operation is a risk factor for a shorter time to LVOTO reintervention. Lecompte and associates [22] reported that reintervention for LVOTO was required in 2 of 111 patients; this low frequency of reintervention confirms the efficacy of aggressive VSD enlargement combined with resection of the infundibular septum in reducing the risk of recurrent LVOTO in patients with REV procedure. Yeh and associates [13] reported midterm outcomes in 19 patients after the Nikaidoh operation. No LVOTO was evident and no patient had aortic insufficiency that was more than mild. Conversely, Morell and Wearden [12] reported midterm outcomes in 21 patients after a modified Nikaidoh procedure; LVOTO was eliminated but 3 patients developed moderate aortic insufficiency (15%), with 1 requiring reoperation for severe aortic insufficiency. The higher incidence of reoperation in the later study may be related to technical differences: Yeh and associates [13] and Nikaidoh [4] selectively reimplant the right coronary, but do not reimplant the left coronary artery, perform the Lecompte maneuver, or divide the ascending aorta. However, they operate on a slightly older patient population compared with Morell and associates [12]. In comparison, the risk of aortic insufficiency is very small in patients undergoing the RO, and only one patient in our series has developed mild aortic insufficiency on follow-up echo.
Coronary Artery Anomalies Morell and associates [12, 14] concluded that the only contraindication to the Nikaidoh procedure has been the presence of anomalous coronary anatomy. The epicardial course of a major coronary artery could preclude the safe mobilization of the aortic root from the right ventricle (ie, right coronary artery originating from the left main). Also, the coronary anatomy could affect the ability to safely move the aorta posteriorly, into the pulmonary annulus (ie, posterior intramural coronary artery course); this anatomy may preclude coronary reimplantation in conjunction with aortic translocation. For patients with coronary anomalies, the Rastelli procedure is a better option. In our study, only one patient with a coronary artery anomaly died during follow-up (1 of 7; 14%).
Conclusion The RO remains the procedure of choice at many institutions throughout the world for children with TGA, VSD, and PS. The RO protects the RV from chronic regurgitation where as the REV and Nikaidoh procedures do not. The early mortality of the RO has been reduced to less than 5% in the majority of institutions [7, 10, 11]. Most patients will require reoperation for conduit change, but these procedures are currently performed with a low morbidity and mortality [7, 10, 11, 20]. Aggressive resection of the anterosuperior portion of the ventricular septum to enlarge the VSD has nearly eliminated the need for LVOTO reoperation in most series [7, 8, 10]. However, the incidence of late sudden death from arrhythmia remains to be explained and is a potentially problematic long-term complication. The results of this retrospective, observational study demonstrate, at intermediate term follow-up, excellent early and late results with the Rastelli operation for transposition of the great arteries, ventricular septal defect, and right ventricular outflow tract obstruction. However, comparative studies with longer follow-up in a large patient population will be required to determine the superiority of the RO when compared with alternative procedures (REV or Nikaidoh) for patients with this morphology.
References 1. Rastelli GC. A new approach to “anatomic” repair of transposition of the great arteries. Mayo Clin Proc 1969;44:1–12. 2. Rastelli GC, McGoon DC, Wallace RB. Anatomic correction of transposition of the great arteries with ventricular septal defect and subpulmonary stenosis. J Thorac Cardiovasc Surg 1969;58:545–52. 3. Lecompte Y, Neveux JY, Leca F, et al. Reconstruction of the pulmonary outflow tract without a prosthetic conduit. J Thorac Cardiovasc Surg 1982;84:727–33. 4. Nikaidoh H. Aortic translocation and biventricular outflow tract reconstruction. A new surgical repair for transposition of the great arteries associated with ventricular septal defect and pulmonary stenosis. J Thorac Cardiovasc Surg 1984;88: 365–72. 5. Prêtre R, Gendron G, Tamisier D, Vernant F, Sidi D, Vouhé P. Results of the Lecompte procedure in malposition of the great arteries and pulmonary obstruction. Eur J Cardiothorac Surg 2001;19:283–9. 6. Metras D, Kreitmann B, Riberi A, et al. Extending the concept of the autograft for complete repair of transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction: a report of ten cases of a modified procedure. J Thorac Cardiovasc Surg 1997;114: 746 –53. 7. Kreutzer C, de Vive J, Oppido G, et al. Twenty-five-year experience with Rastelli repair for transposition of the great arteries. J Thorac Cardiovasc Surg 2000;120:211–23.
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8. Dearani JA, Danielson GK, Puga FJ, Mair DD, Schleck CD. Late results of the Rastelli operation for transposition of the great arteries. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2001;4:3–15. 9. Hu SS, Liu ZG, Li SJ, et al. Strategy for biventricular outflow tract reconstruction: Rastelli, REV, or Nikaidoh procedure? J Thorac Cardiovasc Surg 2008;135:331– 8. 10. Alsoufi B, Awan A, Al-Omrani A, et al. The Rastelli procedure for transposition of the great arteries: resection of the infundibular septum diminishes recurrent left ventricular outflow tract obstruction risk. Ann Thorac Surg 2009;88: 137– 43. 11. Ali Navabi M, Shabanian R, Kiani A, Rahimzadeh M. The effect of ventricular septal defect enlargement on the outcome of Rastelli or Rastelli-type repair. J Thorac Cardiovasc Surg 2009;138:390 – 6. 12. Morell VO, Wearden PD. Nikaidoh operation for transposition of the great arteries with a ventricular septal defect and pulmonary stenosis. Multimedia Manual of Cardiothoracic Surgery doi:10.1510/mmcts.2006.002337. 13. Yeh T Jr, Ramaciotti C, Leonard SR, Roy L, Nikaidoh H. The aortic translocation (Nikaidoh) procedure: midterm results superior to the Rastelli procedure. J Thorac Cardiovasc Surg 2007;133:461–9. 14. Morell VO, Wearden PD. Aortic translocation for the management of transposition of the great arteries with a ventricular septal defect, pulmonary stenosis, and hypoplasia of the right ventricle. Eur J Cardiothorac Surg 2007;31:552– 4. 15. Morell VO, Jacobs JP, Quintessenza JA. Aortic translocation in the management of transposition of the great arteries with ventricular septal defect and pulmonary stenosis: results and follow-up. Ann Thorac Surg 2005;79:2089 –93. 16. Vouhé PR, Tamisier D, Leca F, Ouaknine R, Vernant F, Neveux JY. Transposition of the great arteries, ventricular septal defect, and pulmonary outflow tract obstruction: Rastelli or Lecompte procedure? J Thorac Cardiovasc Surg 1992;103:428 –36. 17. Kim YJ, Park JJ, Lee JR, et al. Modified Lecompte procedure for the anomalies of ventriculoarterial connection. Ann Thorac Surg 2001;72:176 – 81. 18. Lee JR, Lim HG, Kim YJ, et al. Repair of transposition of the great arteries, ventricular septal defect and left ventricular outflow tract obstruction. Eur J Cardiothorac Surg 2004;25: 735– 41. 19. Hörer J, Schreiber C, Dworak E, et al. Long-term results after the Rastelli repair for transposition of the great arteries. Ann Thorac Surg 2007;83:2169 –75. 20. Niinami H, Imai Y, Sawatari K, Hoshino S, Ishihara K, Aoki M. Surgical management of tricuspid malinsertion in the Rastelli operation: conal flap method. Ann Thorac Surg 1995;59:1476 – 80. 21. Rychik J, Jacobs ML, Norwood WI. Early changes in ventricular geometry and ventricular septal defect size following Rastelli operation or intra-ventricular baffle repair for conotruncal anomaly: A cause for development of sub-aortic stenosis. Circulation 1994;90(5 pt 2):II13–9. 22. Lecompte Y, Vouhe P. Reparation a l’Etage Ventriculare (REV procedure): not a Rastelli procedure without conduit. Oper Tech Thorac Cardiovasc Surg 2003;8:150 –9.
DISCUSSION DR VICTOR MORELL (Pittsburgh, PA): John, those are wonderful results, and I want to congratulate you and Andy for having such a wonderful series. You compare the Toronto, the Boston, and the Mayo Clinic results at 20-plus years of followup. We are talking about a 50% survival if you include operative mortality on it. Why is your series so different than theirs when
we look at results? Are we talking about the same patients, are we talking about different patients, because it is really hard to imagine that we could have 93% survival in one series and 50% survival in another. We know that not all TGA [transposition of the great arteries], VSD [ventricular septal defect], PSs [pulmonary stenosis] are exactly the same, like the tets. Every patient is
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a little different; like Fontans, they are all not the same. I am fascinated by the fact that you show outstanding results with the Rastelli, which, part of me, I am not surprised. I think the Rastelli is a great operation and it certainly works well in a lot of patients out there who have had practically normal lives with their Rastelli. I think that there are a subset of patients that may not do as well with the Rastelli, and I am wondering, for example, how many patients in your series were patients that had inlet ventricular septal defects? Do you know specifically? DR BROWN: We didn’t separate those out. DR MORELL: Because I wonder, there are some anatomical substrates that might prevent us from doing Rastellis, inlet VSDs, if you have some type of straddling across the ventricular septal defect, and do you think in your series you might have had patients that maybe ended up having a single ventricle as opposed to having a two-ventricle repair if we were, for example, to try a Nikaidoh with an inlet VSD as opposed to just going for the Rastelli? DR BROWN: I am sure that is likely to be the major difference. I think it is patient selection and we have used a strict selection criteria for the Rastelli. Our patients had transposition VSD with pulmonary stenosis with the typical malalignment perimembranous ventricular septal defect. In patients with straddling AV [atrioventricular] valves or an inlet septal VSD, most of those patients, at least in my hands, and I think I can speak for Andy, most would probably be treated with a single-ventricle repair. We have a fairly pure group, at least, and I didn’t include any double outlet right ventricles in this report. They had to meet the criteria of transposition VSD PS. If you are extending the Rastelli to patients with remote VSDs and doubly committed or noncommitted VSDs, the results are not going to look as good. I think patient selection if probably why our results have remained good. DR MORELL: How many of these patients had shunts? Were they all essentially shunted initially? DR BROWN: They were all shunted initially. DR MORELL: What is your preferred age for repair on a patient you are going to do a Rastelli? Do you still wait until four now? DR BROWN: No, no. Most of them now are operated under the age of two years. Whenever they outlive their first BT [BlalockTaussig] shunt is when we intervene. The second procedure is almost always a Rastelli.
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DR MORELL: I do agree with you; I think the Rastelli is still a great operation. I do think that instead of competing procedures, they are complementary. There are a subset of patients that may not be candidates for a Rastelli who probably are good candidates for a Nikaidoh. Probably the REV [réparation a l’étage ventriculaire] and the Rastelli have similar contraindications, but for a Nikaidoh that could undergo a two-ventricle repair, with maybe a more complex operation as opposed to going single ventricle, I honestly don’t think the Nikaidoh is the standard procedure for a transposition VSD PS. I think it is an interesting procedure. It probably plays a role in the management of patients, but I am not sure it is the number one procedure for a TGA, VSD, PS. I think today the Rastelli still is. DR BROWN: I agree with you. I just haven’t learned how to do the Nikaidoh. I think the REV operation is not that dissimilar from the Rastelli. But the Nikaidoh is a more formidable procedure, and I just haven’t had the right patient at the right time or I haven’t been out to watch you do one to figure out how often I would apply this procedure to the patient population that we see. DR MORELL: Again, congratulations. Excellent results. DR CONSTANTINE MAVROUDIS (Cleveland, OH): As usual, John, that was a delightful presentation. What do you mean by enlargement of the VSD? Is it done in the lower portion of the defect or are you in fact resecting the infundibular septum? And if you don’t resect the infundibular septum, what do you think about the REV operation that requires resection of the infundibular septum in every case, a Lecompte maneuver, and shortening the ascending aorta? DR BROWN: Your point is well taken. If I had a patient with a fairly restrictive VSD, and I think maybe that is what Victor is talking about, I would probably want to [do] a Nikaidoh. What we have done when we have enlarged the VSD, we have resected the conal septum up high as possible. If you resect anything inferiorly you are going to give the patient heart block, and you could see that we created heart block in three of our patients as it was. It turns out that the three who developed AV block weren’t the three that had their VSDs enlarged and I can’t quite tell you for sure why they developed AV block. I think the Nikaidoh operation will have its place. I am looking for the right patient to do it on. But I think if you have a patient with a restrictive VSD, the Nikaidoh operation is probably a better way to go if you can’t enlarge the conal septum by as much as you really need to. So I think you probably need to be prepared to do one or the other at the time that you approach the patient.
Rastelli Operation for Transposition of the Great Arteries With Ventricular Septal Defect and Pulmonary Stenosis John W. Brown, MD, Mark Ruzmetov, MD, PhD, Daniel Huynh, BS, Mark D. Rodefeld, MD, Mark W. Turrentine, MD, and Andrew C. Fiore, MD Section of Cardiothoracic Surgery, Indiana University School of Medicine, Indianapolis, Indiana; and St. Louis University School of Medicine, St. Louis, Missouri
Background. The optimal surgical treatment of patients with transposition of the great arteries, ventricular septal defect, and pulmonary stenosis is controversial. Although the Rastelli operation has been standard surgical management of this lesion, aortic root translocation with right ventricular outflow tract (RVOT) reconstruction (Nikaidoh) and the pulmonary artery translocation (Lecompte) or REV (réparation a l’étage ventriculaire) are surgical alternatives more recently introduced to treat this complex lesion. This report reviews our 20-year experience with the Rastelli procedure and attempts to compare our outcomes with those recently published using the Nikaidoh and REV procedures. Methods. Between 1988 and 2008, 40 patients (median age, 4 years; range, 9 months to 17 years) underwent Rastelli operation at our institutions. The RVOT was obstructed in 32 and atretic in 8. Follow-up was available for all but one patient (mean follow-up, 8.6 ⴞ 5.6 years). The RVOT was reconstructed with homograft (n ⴝ 25), bovine jugular vein (n ⴝ 8), nonvalved Dacron tube (n ⴝ 5), or a porcine valved conduit (n ⴝ 2). Two patients required a pacemaker. Results. There were no early, but three late deaths and one heart transplantation 12 years postoperative the Rastelli operation. Kaplan-Meier survival was 93% at 5, 10, and 20 years. Univariate risk factors for death or trans-
plantation included surgery before 1998 (p ⴝ 0.03) and concomitant noncardiac anomalies (p ⴝ 0.001). Sixteen patients (40%) had reoperation for right ventricularpulmonary artery conduit stenosis (mean, 7.8 ⴞ 3.8 years) without mortality. Freedom from conduit replacement was 86%, 74%, 63%, and 59% at 5, 10, 15, and 20 years, respectively. Multivariate analysis revealed that the risk factors of conduit replacement were younger age at operation (p ⴝ 0.001) and surgery before 1998 (p < 0.001). Two patients (5%) required reoperation for left ventricular outflow tract obstruction. At follow-up, there were no sudden unexplained deaths, and New York Heart Association functional class is I or II. Conclusions. The Rastelli procedure is a low-risk operation with regard to early and late mortality and reoperation for left ventricular outflow tract obstruction. Conduit change operations will be required in most patients regardless of the technique of repair, but currently can be performed with low morbidity and mortality. These midterm outcomes after the Rastelli operation should serve as a basis for comparison with surgical alternatives more recently introduced for transposition of the great arteries and ventricular septal defect with RVOT obstruction. (Ann Thorac Surg 2011;91:188 –94) © 2011 by The Society of Thoracic Surgeons
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technique (réparation a l’étage ventriculaire) to reconstruct the pulmonary outflow tract without using a prosthetic conduit as an alternative to the Rastelli repair. Two years later, Nikaidoh [4] proposed aortic translocation and biventricular outflow tract reconstruction for the management of TGA with VSD and PS. Each of these techniques has advantages and disadvantages. Controversy surrounds the optimal surgical procedure for TGA, VSD, and PS with respect to mortality, morbidity, and long-term results. The fate of the reconstructed left and right ventricular outflow tract (LVOT; RVOT) plays an essential role in defining longterm prognosis in these patients. The RO requires late right ventricle (RV) to pulmonary artery (PA) extracardiac conduit replacements and may be associated with recurrent LVOT obstruction (LVOTO) and reduce long-
urgical management of patients with transposition of the great arteries (TGA), ventricular septal defect (VSD), and pulmonary stenosis (PS) continues to be a challenge, because anatomic correction requires reconstruction of both ventricular outflow tracts. Three major surgical techniques have been developed during the past four decades. The Rastelli operation (RO) was first introduced in 1969 and soon became the standard surgical treatment for patients with TGA, VSD, and PS [1, 2]. In 1980, Lecompte and associates [3] introduced the REV
Accepted for publication July 19, 2010. Presented at the Fifty-sixth Annual Meeting of the Southern Thoracic Surgical Association, Marco Island, FL, Nov 4 –7, 2009. Address correspondence to Dr Brown, Section of Cardiothoracic Surgery, Indiana University School of Medicine, 545 Barnhill Dr, EH 215, Indianapolis, IN 46202-5123; e-mail: [email protected].
© 2011 by The Society of Thoracic Surgeons Published by Elsevier Inc
0003-4975/$36.00 doi:10.1016/j.athoracsur.2010.07.057
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Table 1. Associated Lesions
term survival. The REV procedure radically removes the infundibular septum, theoretically preventing LVOTO, but employs direct PA to RV connection with or without a monocusp valve. This procedure has a higher earlier mortality and also requires revision of the RVOT. Since 1988, the RO has been practiced at our respective institutions. We reviewed our entire 21-year experience involving the surgical treatment of TGA, VSD, and PS. The aim of the present study is to review our surgical experience with the RO in this patient population to analyze early and late mortality, morbidity, and need for reoperation.
Material and Methods Our institutional review boards approved this study, and individual consent was not required. All patients who underwent a Rastelli operation for TGA and PS between 1988 and 2009 at James Whitcomb Riley Children’s Hospital in Indiana University, Indianapolis, Indiana (n ⫽ 34) and Cardinal Glennon Children’s Hospital at St. Louis University, St. Louis, Missouri (n ⫽ 6), were included in this retrospective study. Patients with double-outlet right ventricle were excluded. Median age at the time of operation was 4 years (range, 9 months to 17 years) and median weight was 23 kg (range, 5.2 to 102 kg). There were 24 male and 16 female patients. Palliative shunt operations (modified BlalockTaussig shunts, central shunts) were required in 36; 7 of our patients had balloon or surgical atrial septostomy without a shunt in 4. All patients had normal ventricular morphology. Five patients (12.5%) presented with pulmonary atresia. Associated lesions are shown in Table 1.
Surgical Technique Median sternotomy using conventional cardiopulmonary bypass with bicaval cannulation and cold potassium blood cardioplegic solution was employed in all patients. The median cardiopulmonary bypass time was 178 minutes (range, 120 to 340) while the median ischemic time was 98 minutes (range, 60 to 173). All operations were
Patients Lesion Cardiac Atrial septal defect Coronary artery anomaly Left superior vena cava Subaortic stenosis Juxtaposed atrial appendages on the left side TAPVR Unroofed coronary sinus Azygous continuation of inferior vena cava MAPCA (right) Noncardiac Spina bifida Budd-Chiari malformation Klippel-Feil syndrome Goldenhar syndrome MAPCA ⫽ multiple aortopulmonary collaterals; anomalous pulmonary vein return.
No
%
14 7 1 2 2 1 1 1 1
35 18 2.5 5 5 2.5 2.5 2.5 2.5
2 1 1 1
5 2.5 2.5 2.5
TAPVR ⫽ total
performed with hypothermia, mean rectal temperature of 27°C, and left ventricular venting. All systemic to pulmonary shunts were ligated and divided. In 3 patients (8%), the VSD was enlarged at the anterosuperior margin of the defect. The VSD was baffled to the aorta using a gluteraldehyde preserved autologous pericardial patch or a polytetrafluoroethylene patch (Gore-Tex; W. L. Gore & Assoc, Flagstaff, AZ). The patch was sutured in place with continuous suture in 12 patients (30%), by interrupted pledgeted sutures in 5 patients (13%), and by a combination of both in the remaining patients. In two cases, a papillary muscle from the tricuspid valve straddled the LVOT and was resected with its attached chordae tendineae. After the Gore-Tex patch was inserted, the papillary muscle was reimplanted into an appropriate spot on the RV side of the tunnel. In patients with late subaortic obstruction, the conduit is removed from the RV after cardioplegic arrest. The VSD patch is opened longitudinally and resected, leaving a 1 to 2 mm rim of patch near the conduction system. The subaortic stenosis is removed, in addition to any scar tissue along the pathway between the VSD and the aortic valve. If necessary, the VSD is enlarged so that its diameter is equal to or 1 to 2 mm greater than the diameter of the aortic annulus. An appropriately shaped patch is then inserted and sewn with continuous monofilament suture. Inferiorly, the rim of the old patch is used to avoid injury to the conduction system. Subsequently, the aortic cross-clamp is removed (if no atrial septal defect is present) and the RV anastomosis is completed with the heart beating during rewarming. Alternatively, a new conduit can be inserted. The RV to PA connection was reconstructed with a homograft in 25 patients (62%), bovine jugular vein conduit (Contegra; Medtronic Inc, Minneapolis, MN) in 8
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Abbreviations and Acronyms LVOTO ⫽ left ventricular outflow tract obstruction MAPCA ⫽ multiple aortopulmonary collaterals PA ⫽ pulmonary artery PS ⫽ pulmonary stenosis REV ⫽ réparation a l’étage ventriculaire RO ⫽ Rastelli operation RV ⫽ right ventricular RVOTO ⫽ right ventricular outflow tract obstruction TAPVR ⫽ total anomalous pulmonary vein return TGA ⫽ transposition of the great arteries VSD ⫽ ventricular septal defect
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(20%), polyethylene terephthalate fiber (Dacron; DuPont, Wilmington, DE) nonvalved graft conduit in 5 patients (13%), and with a Freestyle porcine root (Medtronic Inc) in 2 patients (5%). The mean size of the RV-PA conduit was 17.3 ⫾ 2.3 mm (range, 13 to 22 mm). In order to avoid valve distortion after sternal closure, we inserted the conduit to the left of midline into the left pulmonary artery and shortened the conduit outflow end so that the pulmonary valve rests near the pulmonary artery bifurcation. Gore-Tex membrane is used in all patients to cover the conduit, innominate vein, ascending aorta, and right ventricle so as to avoid injury during reoperation. Concomitant procedures are shown in Table 2.
Follow-Up One patient was lost to follow-up at 2 years postoperatively. The functional status was determined according to the New York Heart Association class. On follow-up examinations, patients underwent echocardiography and electrocardiography. Ventricular function was graded as normal, mild, moderate, or severely impaired. Insufficiency of the atrioventricular valve, the aortic valve, and the conduit valve in the RVOT was graded as none, mild, moderate, or severe. The LVOTO and RVOTO at latest follow-up were graded as mild stenosis when the maximal instantaneous pressure gradient was less than 30 mm Hg, moderate stenosis when the gradient was 30 to 60 mm Hg, and severe stenosis when the gradient was greater than 60 mm Hg.
Statistical Analysis Measured and calculated data are expressed as mean ⫾ SD. The Kaplan-Meier product limit method and Cox proportional hazards regression methods were used for analysis of survival and freedom from reoperation for RVOTO, LVOTO, residual VSD, or pacemaker implantation. Multiple regression analysis was performed as conditional backward stepwise proportional hazards regression. In the analysis of risk factors for mortality or heart transplantation, freedom from any type of reoperation, variables with significance levels of 0.1 in univariate analysis were submitted to a multivariate logistic regression model. Early mortality was defined as death during initial hospitalization or within 30 days of operation. Any Table 2. Concomitant Procedures During Rastelli Operation Patients Procedure
No
%
Atrial septal defect closure Ventricular septal defect enlargement Left superior vena cava ligation Unroofed coronary sinus repair Subaortic membrane resection TAPVR repair Right MAPCA ligation
14 3 1 1 2 1 1
35 8 2.5 2.5 5 2.5 2.5
MAPCA ⫽ multiple aortopulmonary collaterals; anomalous pulmonary vein return.
TAPVR ⫽ total
deaths later than that were defined as late mortality. A p value of less than 0.05 was considered significant. Specific statistical software SPSS for Windows version 10 (SPSS Inc, Chicago, IL) was used for data analysis.
Results Mortality and Morbidity There were no hospital deaths. Median hospital stay was 12 days (range, 6 to 52 days). There was one early reoperation for bleeding. Two patients (5%) developed permanent atrioventricular block postoperatively and required a permanent pacemaker. The incidence of postoperative permanent atrioventricular block was not significantly higher in patients who had VSD enlargement. Within a mean follow-up of 8.6 ⫹ 5.6 years (range, 3 months to 21 years), there were 3 late deaths and one cardiac transplantation. Kaplan-Meier survival was 93% at 5, 10, and 20 years. Freedom from death or cardiac transplantation was 92% ⫾ 4% and 90% ⫾ 5% at 10 and 20 years, respectively (Fig 1). The first death occurred in a patient with coronary artery anomaly (RV branches arising from the left coronary artery) who died suddenly 1 year postoperatively. This patient had documented sinus rhythm and no LVOTO or RVOTO. The second death presented with multiple stenoses of the peripheral and central pulmonary arteries and Klippel-Feil syndrome, with multiple skeletal and rib anomalies. She died of low cardiac output 3 months after the RO. The third death was in a patient with a myelomeningocele who expired at another hospital 6 months after RO secondary to pneumonia and sepsis. A fourth patient underwent two reinterventions for pacemaker exchange due to complete atrioventricular block. Before cardiac transplantation (12 years after RO), he presented with severely impaired right and left ventricular function with end-stage heart failure and ejection fraction of 15%. Univariate risk factors for death or transplantation included surgery before 1998 (p ⫽ 0.03) and concomitant noncardiac anomalies (p ⫽ 0.001).
Reoperations During follow-up, 18 patients (45%) underwent reoperations: sixteen patients required conduit change for RVOTO and two patients required reoperation for LVOTO. Two patients with LVOTO had scar formation within the baffle pathway from the VSD to the aortic valve. Freedom from reoperation for LVOTO or conduit change at 20 years was 95% ⫾ 2 % and 60 % ⫾ 6.4%, respectively (Fig 2). The average time to conduit failure was 7.8 ⫾ 3.8 years (range, 2 to 16 years). The mean age at reintervention was 12 ⫾ 6.0 years (range, 4 to 27 years). The peak systolic gradient across the RVOT before conduit replacement was 59 ⫾ 8 mm Hg (range, 30 to 80 mm Hg). The most common indication for a conduit replacement was stenosis, which was present in 14 (88%) of the 16 conduit replacement procedures. A less frequent primary indication for conduit replacement was conduit
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Information concerning the functional status at the time of final follow-up could be obtained from 36 patients (excluding lost and late death patients). Accordingly, 97% of the patients were in New York Heart Association class I or II, and 3% in class III. The majority of the patients were leading a normal life with full-time work or school (ability index class I or II), and only one patient experienced noticeable limitations of activities (class III).
Comment
Fig 1. Survival curve in patients with Rastelli operation.
valve insufficiency noted in two patients (12%). All conduits that required replacement had varying degrees of insufficiency, graded as mild in 6, moderate in 8, and severe in 2. Thirteen patients underwent replacement with a second biologic valve, whereas 3 children had their conduit revised with the insertion of a polytetrafluoroethylene monocusp valve. Multivariate analysis revealed risk factors for conduit replacement were younger age at operation (p ⫽ 0.001) and surgery before 1998 (p ⬍ 0.001). Additional operations at time of conduit change were required: closure of residual VSD in 2; closure of atrial septal defect in 1; and muscle resection for infundibular obstruction in 1 patient.
Follow-Up The mean follow-up period for children after RO is 8.6 ⫾ 5.6 years (range, 3 months to 21 years). Mean age of the patients at final follow-up was 13.6 ⫾ 7.7 years. Echocardiographic and electrocardiographic data were obtained at last follow-up in 18 patients without reoperation (all patients had their original conduit). Left ventricular function was normal in 95% of the patients, while freedom from LVOTO or aortic insufficiency was 89% and 95%, respectively. One patient had a 22 mm Hg LVOTO gradient, and another had mild aortic insufficiency. Right ventricular function was normal in 89% of the patients; 89% presented with no RVOTO and 100% with no or mild pulmonary insufficiency. Two patients presented with moderate stenosis of the conduit (41 and 47 mm Hg) and are being followed with serial echocardiography. At last follow-up, sinus rhythm with complete right bundle branch block was present in 31 patients. Two patients who received pacemaker implantation post RO had pacemaker rhythm at final follow-up. In 3 patients, permanent pacemaker implantation was performed after hospital discharge for complete atrioventricular block (n ⫽ 2) and sick sinus syndrome (n ⫽ 1). Freedom from pacemaker implantation was 92% and 86%, at 5 and 20 years, respectively. Enlargement of the VSD at the time of the RO had no significant influence on freedom from pacemaker implantation (p ⫽ 0.36).
The optimal anatomic surgical repair of patients with TGA, VSD, and PS is controversial. The Rastelli operation has become the “gold standard” in the surgical management in patients with this pathology. The RO in its original description directed the LV blood to the aorta by suturing a Teflon patch (Boston Scientific Corp, Meditec, NJ) between the VSD and the aortic valve orifice and establishing continuity from the RV to the PA with a valved conduit [1, 2]. In 1984, Nikaidoh [4] published a new surgical repair for TGA, VSD, and PS. This operation achieves an anatomic repair that utilizes aortic root mobilization and transfer to the LV combined with reconstruction of the RVOT and LVOT tracts. The potential advantage of this technique is that mobilization of the aortic root and direct anastomosis to the LV is accomplished with the coronary arteries intact and obviates the need for coronary artery transfer and the potential hazard of early or late ostial stenosis. However, the RVOT is devoid of a functioning valve and after requires valved conduit at a subsequent surgery. An important variation on the theme of RO was published by Lecompte and his associates in 1982 [3]. This operation was called “reparation a l’etage ventriculaire” or the REV procedure. This technique departs from the classic RO in that the infundibular septum is totally resected providing a wide, nonobstructed tunnel from the LV to the aorta, avoiding the potential for subaortic stenosis. The pulmonary artery is directly connected to the RV and a monocusp valve is inserted [3, 5].
Fig 2. Freedom from reoperation curve in patients with Rastelli operation.
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Metras and associates [6] have published a modification of the Rastelli-REV procedures. Rather than a direct connection between the RV and the PA, they use an aortic autograft taken from the patients’ ascending aorta to facilitate this connection. The authors favor this approach because the classic REV procedure with a direct RV to PA connection places tension on the pulmonary artery resulting in frequent reinterventions.
Survival In the earlier published series with RO, mortality was high [7, 8]. Dearani and associates [8] reviewed 160 hospital survivors operated on between 1968 and 1990, who were followed for at least 10 years. They found an actuarial survival of 74% at 10 years and 59% at 20 years. In their series, mortality decreased from 24% for the years 1968 through 1977 to 4% from 1988 to 1997. Kreutzer and associates [7] reviewed the results of the RO in 101 patients at the Boston Children’s Hospital operated on from 1973 to 1998 and operative mortality was 7%, with no operative deaths in the last 7 years of the study. During the last 3 to 5 years, there have been several new reports of patients who underwent the RO with mortality less than 5% [9 –11]. In this series, there are no early mortality but the late mortality decreased from 15% for the years 1990 through 1998 to 4% from 1998 to 2008; 2 of the 4 adverse outcomes were in patients with severe noncardiac anomalies. Similar low mortality rates were reported by Yeh and Morell and associates (5%) in patients with the Nikaidoh procedure [12–15] and by Prêtre (8%) [5] and Lecompte (6%) [3] and associates in patients with the REV procedure.
Reoperations on RVOTO and LVOTO Freedom from the RVOT reoperation was reported to be significantly higher after the REV procedure, compared with the RO [5, 9, 15–17]. One disadvantage of the REV procedure is that it cannot be applied to patients with pulmonary atresia. In addition, the pulmonary artery bifurcation is placed anterior to the aorta (Lecompte maneuver) which may lead to tension and sternal compression of the branch pulmonary arteries as they pass over the enlarged ascending aorta [17]. Potentially, this may result in chronic elevation of RV pressure. The fate of the RVOT is an important consideration when evaluating the effectiveness of the REV procedure. Lee and associates [18] experienced RVOTO in 25% of patients after the REV procedure. Vouhé and associates [16] reported that RVOTO occurred in 26% of their patients, with a mean follow-up of 55 months. Late LVOTO may be an important late complication after the RO [19, 20]. The obstruction is usually located at the level of the VSD or beneath the aortic valve. The LVOTO may persist because the VSD was restrictive at initial operation and not sufficiently enlarged or it may develop as a result of muscular hypertrophy within the outflow tract. Rychik and associates [21] have postulated that in TGA, as in double-inlet LV, the VSD or bulboventricular foramen becomes restrictive after biventricular repair or Fontan operation because LV volume is often
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increased before correction and decreases after the RO. Kreutzer and associates [7] reported freedom from LVOTO reintervention of 84% at 15 years in contrast to 97% at 20 years in our cohort. Hörer and associates [19] reported a freedom from LVOTO to be 93% at 15 years and explained that LVOTO may be related to inadequate enlargement of the VSD (59% of their patients). Hörer and associates noted that resecting the conal septum or the anterosuperior margin can enlarge even a small restrictive VSD to the size of the aortic annulus and provide a straight nonobstructed pathway along the LVOT. Hörer and associates’ higher freedom from LVOTO may also be explained by the higher median age at the time of RO when compared with the cohort reported by Kreutzer and associates [7] (4.5 vs 3.1 years). Hörer and associates reported that age younger than 1 year at the initial operation is a risk factor for a shorter time to LVOTO reintervention. Lecompte and associates [22] reported that reintervention for LVOTO was required in 2 of 111 patients; this low frequency of reintervention confirms the efficacy of aggressive VSD enlargement combined with resection of the infundibular septum in reducing the risk of recurrent LVOTO in patients with REV procedure. Yeh and associates [13] reported midterm outcomes in 19 patients after the Nikaidoh operation. No LVOTO was evident and no patient had aortic insufficiency that was more than mild. Conversely, Morell and Wearden [12] reported midterm outcomes in 21 patients after a modified Nikaidoh procedure; LVOTO was eliminated but 3 patients developed moderate aortic insufficiency (15%), with 1 requiring reoperation for severe aortic insufficiency. The higher incidence of reoperation in the later study may be related to technical differences: Yeh and associates [13] and Nikaidoh [4] selectively reimplant the right coronary, but do not reimplant the left coronary artery, perform the Lecompte maneuver, or divide the ascending aorta. However, they operate on a slightly older patient population compared with Morell and associates [12]. In comparison, the risk of aortic insufficiency is very small in patients undergoing the RO, and only one patient in our series has developed mild aortic insufficiency on follow-up echo.
Coronary Artery Anomalies Morell and associates [12, 14] concluded that the only contraindication to the Nikaidoh procedure has been the presence of anomalous coronary anatomy. The epicardial course of a major coronary artery could preclude the safe mobilization of the aortic root from the right ventricle (ie, right coronary artery originating from the left main). Also, the coronary anatomy could affect the ability to safely move the aorta posteriorly, into the pulmonary annulus (ie, posterior intramural coronary artery course); this anatomy may preclude coronary reimplantation in conjunction with aortic translocation. For patients with coronary anomalies, the Rastelli procedure is a better option. In our study, only one patient with a coronary artery anomaly died during follow-up (1 of 7; 14%).
Conclusion The RO remains the procedure of choice at many institutions throughout the world for children with TGA, VSD, and PS. The RO protects the RV from chronic regurgitation where as the REV and Nikaidoh procedures do not. The early mortality of the RO has been reduced to less than 5% in the majority of institutions [7, 10, 11]. Most patients will require reoperation for conduit change, but these procedures are currently performed with a low morbidity and mortality [7, 10, 11, 20]. Aggressive resection of the anterosuperior portion of the ventricular septum to enlarge the VSD has nearly eliminated the need for LVOTO reoperation in most series [7, 8, 10]. However, the incidence of late sudden death from arrhythmia remains to be explained and is a potentially problematic long-term complication. The results of this retrospective, observational study demonstrate, at intermediate term follow-up, excellent early and late results with the Rastelli operation for transposition of the great arteries, ventricular septal defect, and right ventricular outflow tract obstruction. However, comparative studies with longer follow-up in a large patient population will be required to determine the superiority of the RO when compared with alternative procedures (REV or Nikaidoh) for patients with this morphology.
References 1. Rastelli GC. A new approach to “anatomic” repair of transposition of the great arteries. Mayo Clin Proc 1969;44:1–12. 2. Rastelli GC, McGoon DC, Wallace RB. Anatomic correction of transposition of the great arteries with ventricular septal defect and subpulmonary stenosis. J Thorac Cardiovasc Surg 1969;58:545–52. 3. Lecompte Y, Neveux JY, Leca F, et al. Reconstruction of the pulmonary outflow tract without a prosthetic conduit. J Thorac Cardiovasc Surg 1982;84:727–33. 4. Nikaidoh H. Aortic translocation and biventricular outflow tract reconstruction. A new surgical repair for transposition of the great arteries associated with ventricular septal defect and pulmonary stenosis. J Thorac Cardiovasc Surg 1984;88: 365–72. 5. Prêtre R, Gendron G, Tamisier D, Vernant F, Sidi D, Vouhé P. Results of the Lecompte procedure in malposition of the great arteries and pulmonary obstruction. Eur J Cardiothorac Surg 2001;19:283–9. 6. Metras D, Kreitmann B, Riberi A, et al. Extending the concept of the autograft for complete repair of transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction: a report of ten cases of a modified procedure. J Thorac Cardiovasc Surg 1997;114: 746 –53. 7. Kreutzer C, de Vive J, Oppido G, et al. Twenty-five-year experience with Rastelli repair for transposition of the great arteries. J Thorac Cardiovasc Surg 2000;120:211–23.
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8. Dearani JA, Danielson GK, Puga FJ, Mair DD, Schleck CD. Late results of the Rastelli operation for transposition of the great arteries. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2001;4:3–15. 9. Hu SS, Liu ZG, Li SJ, et al. Strategy for biventricular outflow tract reconstruction: Rastelli, REV, or Nikaidoh procedure? J Thorac Cardiovasc Surg 2008;135:331– 8. 10. Alsoufi B, Awan A, Al-Omrani A, et al. The Rastelli procedure for transposition of the great arteries: resection of the infundibular septum diminishes recurrent left ventricular outflow tract obstruction risk. Ann Thorac Surg 2009;88: 137– 43. 11. Ali Navabi M, Shabanian R, Kiani A, Rahimzadeh M. The effect of ventricular septal defect enlargement on the outcome of Rastelli or Rastelli-type repair. J Thorac Cardiovasc Surg 2009;138:390 – 6. 12. Morell VO, Wearden PD. Nikaidoh operation for transposition of the great arteries with a ventricular septal defect and pulmonary stenosis. Multimedia Manual of Cardiothoracic Surgery doi:10.1510/mmcts.2006.002337. 13. Yeh T Jr, Ramaciotti C, Leonard SR, Roy L, Nikaidoh H. The aortic translocation (Nikaidoh) procedure: midterm results superior to the Rastelli procedure. J Thorac Cardiovasc Surg 2007;133:461–9. 14. Morell VO, Wearden PD. Aortic translocation for the management of transposition of the great arteries with a ventricular septal defect, pulmonary stenosis, and hypoplasia of the right ventricle. Eur J Cardiothorac Surg 2007;31:552– 4. 15. Morell VO, Jacobs JP, Quintessenza JA. Aortic translocation in the management of transposition of the great arteries with ventricular septal defect and pulmonary stenosis: results and follow-up. Ann Thorac Surg 2005;79:2089 –93. 16. Vouhé PR, Tamisier D, Leca F, Ouaknine R, Vernant F, Neveux JY. Transposition of the great arteries, ventricular septal defect, and pulmonary outflow tract obstruction: Rastelli or Lecompte procedure? J Thorac Cardiovasc Surg 1992;103:428 –36. 17. Kim YJ, Park JJ, Lee JR, et al. Modified Lecompte procedure for the anomalies of ventriculoarterial connection. Ann Thorac Surg 2001;72:176 – 81. 18. Lee JR, Lim HG, Kim YJ, et al. Repair of transposition of the great arteries, ventricular septal defect and left ventricular outflow tract obstruction. Eur J Cardiothorac Surg 2004;25: 735– 41. 19. Hörer J, Schreiber C, Dworak E, et al. Long-term results after the Rastelli repair for transposition of the great arteries. Ann Thorac Surg 2007;83:2169 –75. 20. Niinami H, Imai Y, Sawatari K, Hoshino S, Ishihara K, Aoki M. Surgical management of tricuspid malinsertion in the Rastelli operation: conal flap method. Ann Thorac Surg 1995;59:1476 – 80. 21. Rychik J, Jacobs ML, Norwood WI. Early changes in ventricular geometry and ventricular septal defect size following Rastelli operation or intra-ventricular baffle repair for conotruncal anomaly: A cause for development of sub-aortic stenosis. Circulation 1994;90(5 pt 2):II13–9. 22. Lecompte Y, Vouhe P. Reparation a l’Etage Ventriculare (REV procedure): not a Rastelli procedure without conduit. Oper Tech Thorac Cardiovasc Surg 2003;8:150 –9.
DISCUSSION DR VICTOR MORELL (Pittsburgh, PA): John, those are wonderful results, and I want to congratulate you and Andy for having such a wonderful series. You compare the Toronto, the Boston, and the Mayo Clinic results at 20-plus years of followup. We are talking about a 50% survival if you include operative mortality on it. Why is your series so different than theirs when
we look at results? Are we talking about the same patients, are we talking about different patients, because it is really hard to imagine that we could have 93% survival in one series and 50% survival in another. We know that not all TGA [transposition of the great arteries], VSD [ventricular septal defect], PSs [pulmonary stenosis] are exactly the same, like the tets. Every patient is
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a little different; like Fontans, they are all not the same. I am fascinated by the fact that you show outstanding results with the Rastelli, which, part of me, I am not surprised. I think the Rastelli is a great operation and it certainly works well in a lot of patients out there who have had practically normal lives with their Rastelli. I think that there are a subset of patients that may not do as well with the Rastelli, and I am wondering, for example, how many patients in your series were patients that had inlet ventricular septal defects? Do you know specifically? DR BROWN: We didn’t separate those out. DR MORELL: Because I wonder, there are some anatomical substrates that might prevent us from doing Rastellis, inlet VSDs, if you have some type of straddling across the ventricular septal defect, and do you think in your series you might have had patients that maybe ended up having a single ventricle as opposed to having a two-ventricle repair if we were, for example, to try a Nikaidoh with an inlet VSD as opposed to just going for the Rastelli? DR BROWN: I am sure that is likely to be the major difference. I think it is patient selection and we have used a strict selection criteria for the Rastelli. Our patients had transposition VSD with pulmonary stenosis with the typical malalignment perimembranous ventricular septal defect. In patients with straddling AV [atrioventricular] valves or an inlet septal VSD, most of those patients, at least in my hands, and I think I can speak for Andy, most would probably be treated with a single-ventricle repair. We have a fairly pure group, at least, and I didn’t include any double outlet right ventricles in this report. They had to meet the criteria of transposition VSD PS. If you are extending the Rastelli to patients with remote VSDs and doubly committed or noncommitted VSDs, the results are not going to look as good. I think patient selection if probably why our results have remained good. DR MORELL: How many of these patients had shunts? Were they all essentially shunted initially? DR BROWN: They were all shunted initially. DR MORELL: What is your preferred age for repair on a patient you are going to do a Rastelli? Do you still wait until four now? DR BROWN: No, no. Most of them now are operated under the age of two years. Whenever they outlive their first BT [BlalockTaussig] shunt is when we intervene. The second procedure is almost always a Rastelli.
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DR MORELL: I do agree with you; I think the Rastelli is still a great operation. I do think that instead of competing procedures, they are complementary. There are a subset of patients that may not be candidates for a Rastelli who probably are good candidates for a Nikaidoh. Probably the REV [réparation a l’étage ventriculaire] and the Rastelli have similar contraindications, but for a Nikaidoh that could undergo a two-ventricle repair, with maybe a more complex operation as opposed to going single ventricle, I honestly don’t think the Nikaidoh is the standard procedure for a transposition VSD PS. I think it is an interesting procedure. It probably plays a role in the management of patients, but I am not sure it is the number one procedure for a TGA, VSD, PS. I think today the Rastelli still is. DR BROWN: I agree with you. I just haven’t learned how to do the Nikaidoh. I think the REV operation is not that dissimilar from the Rastelli. But the Nikaidoh is a more formidable procedure, and I just haven’t had the right patient at the right time or I haven’t been out to watch you do one to figure out how often I would apply this procedure to the patient population that we see. DR MORELL: Again, congratulations. Excellent results. DR CONSTANTINE MAVROUDIS (Cleveland, OH): As usual, John, that was a delightful presentation. What do you mean by enlargement of the VSD? Is it done in the lower portion of the defect or are you in fact resecting the infundibular septum? And if you don’t resect the infundibular septum, what do you think about the REV operation that requires resection of the infundibular septum in every case, a Lecompte maneuver, and shortening the ascending aorta? DR BROWN: Your point is well taken. If I had a patient with a fairly restrictive VSD, and I think maybe that is what Victor is talking about, I would probably want to [do] a Nikaidoh. What we have done when we have enlarged the VSD, we have resected the conal septum up high as possible. If you resect anything inferiorly you are going to give the patient heart block, and you could see that we created heart block in three of our patients as it was. It turns out that the three who developed AV block weren’t the three that had their VSDs enlarged and I can’t quite tell you for sure why they developed AV block. I think the Nikaidoh operation will have its place. I am looking for the right patient to do it on. But I think if you have a patient with a restrictive VSD, the Nikaidoh operation is probably a better way to go if you can’t enlarge the conal septum by as much as you really need to. So I think you probably need to be prepared to do one or the other at the time that you approach the patient.