MMCTS
(January 23, 2009). doi:10.1510/mmcts.2007.003046
Copyright © 2009 European Association for Cardio-thoracic Surgery
Procedure
Surgery for malposition of the great arteries: the REV procedure
Duccio Di Carloa,*,
Yves Lecompteb,
Biagio Tomascoc,
Laurence Cohenb and
Pascal Vouhéd
a Ospedale Pediatrico Bambino Gesù, Piazza Sant'Onofrio 4, 00165 Rome, Italy
b Institut Hospitalier Jacques Cartier, Massy, France
c Ospedale San Carlo, Potenza, Italy
d Hôpital Necker, Paris, France
* Corresponding author: Tel.: +39-06-6859 2465; fax: +39-06-6859 2670. ducciodicarlo{at}mac.com
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Summary
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The REV procedure was introduced in 1980 to treat transposition of the great arteries with ventricular septal defect (VSD) and pulmonary stenosis and malpositions similar to transposition of the great arteries (TGA). It aims at overcoming the drawbacks and limitations of the classic Rastelli operation, such as subaortic stenosis, late ventricular deterioration, arrhythmias and sudden death. In particular, the resection of the infundibular septum allows for the placement of a straighter, smaller ventricular patch, bulging much less in the right ventricular cavity. The extensive mobilization of the main pulmonary branches permits a direct connection with the right ventricular incision, thus avoiding the need for an extracardiac conduit. The procedure was performed in 205 patients as of December 2003 with 12% hospital mortality. Patients for whom the Rastelli operation would have been contraindicated, were accepted for REV repair. Late results show a clear improvement over those reported with the Rastelli operation in terms of overall survival (85% at 25-year follow-up interval) and prevalence of reoperation for right ventricular obstruction. Obstruction of the left ventricle-to-aorta tunnel is exceedingly rare. This operation should be considered the gold standard when new surgical options are considered for this complex form of transpositions/malpositions of the great arteries.
Key Words: Anomalies of ventriculo-arterial connection • Intraventricular repair • Malposition • Rastelli operation • Transposition of the great arteries
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Introduction
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Extreme anatomic variability has ignited long controversies on definition and classification of the anomalies of the ventriculo-arterial connection [1]. In turn, embryology, anatomy and hemodynamics have been used to define subgroups of malformation, in a way helpful to surgical management. This fact has generated further confusion.
Our group has chosen to group all these malformations under the term of malposition of the great arteries and to pursue their classification on the basis of available surgical options. The final goal is to establish the best match between the individual patient and surgical repair.
The REV procedure [2,3,4,5,6,7,8] is a surgical technique used to treat transposition of the great arteries with ventricular septal defect (VSD) and pulmonary stenosis and malpositions similar to transposition of the great arteries (TGA), as an alternative to the classic Rastelli repair [9].
In such patients, pulmonary stenosis is usually caused to malalignment of the upper portion of the muscular septum (conal septum) under the pulmonary valve; its resection represents a fundamental step of the procedure (Video 1 – Animation). The resulting wide pathway between the left ventricle and the aorta allows for the placement of a straight re-routing ventricular patch. This is crucial in order to avoid the complication of subaortic stenosis. An extracardiac conduit needs not to be used [2].
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Video 1 Animation. Principles of REV operation: a) Resection of a posteriorly deviated conal septum effectively enlarges the VSD and facilitates the construction of a wide and straight LV to aorta tunnel, bulging very little in the right ventricular cavity. The native pulmonary artery is sutured. b) Transection of the great arteries and French manoeuvre permit direct implantation of the pulmonary artery on the right ventriculotomy.
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A typical 2-D echocardiographic pattern of TGA, VSD, left ventricular outflow tract obstruction (LVOTO) is shown (Video 2).
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Video 2 Bidimensional echocardiography. Typical pattern of TGA, VSD and LVOTO, caused by posteriorly deviated conal septum.
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Surgical technique
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The procedure is carried out through a median sternotomy. The great arteries are dissected (Video 3) and normothermic cardiopulmonary bypass is instituted. The aorta is cross-clamped and antegrade warm cardioplegia administered in the aortic root. Through a right atriotomy, a patent foramen ovale (PFO) or atrial septal defect is closed.
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Video 3 The aorta (anterior and to the left), main pulmonary artery (posterior and right) are dissected free; umbilical tapes are passed around the vessels.
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Through a subaortic incision of the right ventricle (Video 4) anatomy is assessed to confirm the preoperative data regarding feasibility of the repair. The aorta (Video 5) and the pulmonary artery are divided (Video 6). The dissection of the pulmonary confluence and branches is completed. In most cases, the ascending aorta is translocated posteriorly to the pulmonary bifurcation (Video 7 – French manoeuvre).
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Video 4 A subaortic right ventricular incision is performed.
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Video 5 Transection of the aorta, sharp and blunt dissection of the pulmonary trunk.
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Video 6 Division of the pulmonary trunk at the level of the valve commissures. The valve is usually malformed, as seen here. The pulmonary confluence, held by a Derra clamp, if fully dissected. The duct or ligament is sutured and divided to increase mobility.
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Video 7 A French manoeuvre is performed in most cases.
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A sizing dilator is introduced in the pulmonary orifice (Video 8). This step has two objectives:
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Video 8 A Hegar dilator is introduced across the pulmonary valve in the left ventricular cavity and identified from the ventriculotomy. Forceps point out to a well-developed conal septum, pushed forward by the dilator (then grabbed by forceps).
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- to protect the mitral valve from damage; and, mainly
- to perfectly expose the portion of ventricular septum interposed between the VSD, distally, and the aortic orifice, cranially; this muscular structure corresponds to the conal septum (Schematic 1).
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Schematic 1 Introduction of a Hegar dilator through the pulmonary valve into the left ventricle. This maneuver exposes, pushing it towards the surgeon, the conal septum; it also protects the anterior leaflet of the mitral valve during muscle resection. Three lines of incision (dotted line) are made to remove this part of the septum interposed between the VSD and the aortic orifice. (Reproduced from Lecompte Y, Vouhé P. Réparation à l'Etage Ventriculaire (REV Procedure): not a Rastelli without conduit. Operative Techniques in Thoracic and Cardiovascular Surgery: A Comparative Atlas 2003;8:150–159 with permission from Elsevier, Inc.)
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It should be kept in mind that the pulmonary orifice usually lies on a lower plane than the aortic one. Accordingly, the septal incision must be slightly oblique and not perpendicular to the septal surface (Schematic 2).
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Schematic 2 The subaortic line of incision is oblique, as the plane of the pulmonary orifice is usually lower than the aortic one. A straight incision may injure the pulmonary orifice or a major coronary artery branch. (Reproduced from Lecompte Y, Vouhé P. Réparation à l'Etage Ventriculaire (REV Procedure): not a Rastelli without conduit. Operative Techniques in Thoracic and Cardiovascular Surgery: A Comparative Atlas 2003;8:150–159 with permission from Elsevier, Inc.)
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After resection of the conal septum (Video 9), a wide pathway is created, necessary to the placement of a tunnel patch as short and straight as possible, between the left ventricle and the aorta.
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Video 9 Resection of the conal septum. Three incisions are performed: one anterior and vertical; one posterior, similarly vertical; one horizontal, under the aortic valve, joining the two former ones. An eleven-blade scalpel or a microsurgery knife (Sharpoint, Surgical Specialties Corporation, Reading, PA, USA – MMCTSLink 175) is used. The zoom vision shows the area of resected muscle, just anterior to the mitral valve anterior leaflet.
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A round patch is tailored (Video 10). The patch suture follows the muscular margins of the VSD (Schematic 3), starting from its posterior portion, then continues on the right side of the aortic orifice and then on its anterior part (Video 11).
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Video 10 Sizing of a circular ventricular patch, that has a diameter equal to the distance between the posterior edge of the VSD and the anterior portion of the aortic annulus. The patch is shaped as sandwich of Dacron Sauvage (Impra Inc., a subsidiary of C.R. Bard, Inc., Tempe, AZ, USA – MMCTSLink 176) [right side] and heterologous pericardium [left side].
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Schematic 3 The construction of the intraventricular tunnel begins at the inferior border of the VSD, passing the sutures either through the septal leaflet of the tricuspid valve or a muscular rim which may separate the defect from the atrioventricular valve. Interrupted, pledgetted sutures are often used here. The suture line runs towards the right side of the aortic valve; pushing the transected aorta towards the right ventricle often helps to assure perfect exposure of the upper part of the suture line, around the aortic valve. (Reproduced from Lecompte Y, Vouhé P. Réparation à l'Etage Ventriculaire (REV Procedure): not a Rastelli without conduit. Operative Techniques in Thoracic and Cardiovascular Surgery: A Comparative Atlas 2003;8:150–159 with permission from Elsevier, Inc.)
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Video 11 The fixation of the patch is started from the posterior portion of the VSD, then the suture line is carried on the right side of the aortic orifice and to its anterior part.
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It is mandatory, in most cases, to trim the anterior portion of the patch (Video 12), that should reach the anterior border of the ventricular septal defect without traction or redundancy. Leaving too much patch tissue does not protect from the danger of tunnel stenosis; on the contrary, a redundant patch always bulges in the subaortic region, despite the opposite pressure gradient between the ventricles.
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Video 12 The patch is then trimmed, greatly reducing its left margin.
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The intracardiac steps are finished. The native pulmonary orifice is obliterated (Schematic 4). A danger inherent to this manoeuvre is to distort a coronary artery branch, coursing in proximity to the orifice. The coronary artery course is checked with a fine sound before and after suturing the pulmonary orifice.
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Schematic 4 The pulmonary orifice is closed directly, paying attention to the coronary arteries that run nearby. If a French Manoeuvre is used, it is mandatory to resect a generous wedge of ascending aorta, in order to create large space in the mediastinum for the anteriorly placed pulmonary outflow tract. (Reproduced from Lecompte Y, Vouhé P. Réparation à l'Etage Ventriculaire (REV Procedure): not a Rastelli without conduit. Operative Techniques in Thoracic and Cardiovascular Surgery: A Comparative Atlas 2003;8:150–159 with permission from Elsevier, Inc.)
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A large portion of the ascending aorta is excised, and the aortic stumps are directly anastomosed (Video 13). The aortic clamp is now removed.
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Video 13 Suture of the native pulmonary orifice in two over-and-over layers, occasionally reinforced by pledgetted interrrupted sutures. The patency of the nearby crossing left coronary artery is verified from the aorta. A generous wedge resection of a segment of ascending aorta is performed, in order to create sufficient space in the anterior mediastinum for the anteriorly translocated pulmonary pathway. The aortic stumps are re-anastomosed.
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The pulmonary trunk is incised anteriorly (Video 14) and reimplanted on the upper part of the ventriculotomy (Schematic 5). In order to limit the amount of early postoperative pulmonary regurgitation, a fine polytetrafluoroethylene (PTFE) monocusp valve is constructed (Video 15) and sutured to the margins of the ventricular incision [10].
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Video 14 The pulmonary trunk is incised anteriorly and sutured to the upper part of the ventriculotomy.
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Schematic 5 Reconstruction of the pulmonary outflow tract. (A) anterior incision of the pulmonary trunk and direct anastomosis of its posterior half to the upper ventriculotomy; (B) monocusp polytetrafluoroethylene valve sutured to the borders of the ventriculotomy; (C) closure of the pulmonary outlow tract with a sandwich patch attached to the entire ventricular incision.
The width of the patch (equal to length of the monocusp valve free edge) is tailored to obtain an outflow tract diameter 1.5 times the ‘normal’ value by body weight. (Reproduced from Lecompte Y, Vouhé P. Réparation à l'Etage Ventriculaire (REV Procedure): not a Rastelli without conduit. Operative Techniques in Thoracic and Cardiovascular Surgery: A Comparative Atlas 2003;8:150–159 with permission from Elsevier, Inc.)
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Video 15 A monocusp PTFE valve (PTFE Pericardial Membrane, W.L. Gore & Associates Inc., Flagstaff, AZ, USA – MMCTSLink 41) is tailored and sutured to the ventriculotomy edges.
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The final step is the anterior implantation of a patch (Video 16) to widen the right ventricular outflow tract (Video 17).
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Video 16 A sandwich patch (Dacron on the outside) is again used to close off the ventriculotomy anteriorly. Its width is equal to 1.5 times the ‘normal’ diameter of the pulmonary valve by body weight.
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Video 17 Repair is now complete.
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Temporary atrial and ventricular electrodes are routinely implanted, as well as a left atrial line. Discontinuation of bypass follows the usual steps.
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Results
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Two hundred and five patients were operated on by two of the authors between 1980 and 2003; their mean age at surgery was 21 months, ranging from 3.6 to 192 months. One hundred and twenty-three patients (60%) had undergone previous surgery, for a total of 145 procedures.
Twenty-four patients died early after surgery (hospital mortality: 12%). Out of 181 early survivors, one hundred and seventy-one patients were available for follow-up: ten patients, living in distant countries, could not be traced.
Actuarial survival by the Kaplan–Meier method was 85% at 25-year follow-up interval (Graph 1 ).
By the cumulative method analysis [11], six possible outcomes at 25-year follow-up interval were evaluated: alive without reoperation, reoperation for right ventricular outflow tract obstruction (RVOTO), reoperation for LVOTO, reoperation for residual VSD, reoperation for other causes, and death. Results were 45%, 33%, 5%, 6%, 6.5%, 6.5%, respectively (Graph 2).
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Graph 2 Cumulative method analysis.
Redo, re-intervention (any type); RVOTO, right ventricular outflow tract obstruction; LVOTO, left ventricular outflow tract obstruction; VSD, recurrent/residual ventricular septal defect.
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There were thirteen late deaths: the causes are indicated in Table 1. Reoperation was necessary in 36 patients for recurrent RVOTO and in three patients because of residual LVOTO. Reoperation resulted in two late deaths.
One hundred and thirty-nine patients are asymptomatic (79%, NYHA class I) and fourteen patients (8%), are mildly symptomatic with arrhythmias or dyspnoea on major efforts (NYHA class II).
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Discussion
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The classic repair of transposition of the great arteries with VSD and pulmonary stenosis has been represented by the Rastelli operation. Anatomic contraindications, complex shape of the intraventricular patch, need for an extracardiac conduit, are known drawbacks of this procedure, causing disappointing late results even in the best hands [12,13,14]. The REV procedure provides an answer to many of these concerns: contraindications such as tricuspid or mitral abnormalities are overcome, the intraventricular patch is straight and does not bulge in the right ventricular cavity, an extracardiac conduit in not needed.
A comparison of results with the Rastelli and REV operations is shown in Table 2.
Recently, a procedure originally described by Bex in 1980 [15] and adopted subsequently by Nikaidoh [16] has raised a strong interest; now named ‘aortic translocation procedure’ by Morell et al. [17], it combines principles of the Ross and arterial switch procedure and, occasionally of the REV itself [18,19,20]. Results are encouraging in terms of overall survival; unfortunately, complications, common after the originating procedures, are reported, such as coronary problems, aortic valve insufficiency, conduit degeneration [18, 19,20,21]. While the enthusiasts of this procedure claim that it eliminates a formidable complication such as late subaortic obstruction, it should be borne in mind that such a problem has been virtually eliminated by the REV procedure. Larger series and longer follow-up intervals will be required to demonstrate the superiority of the aortic translocation procedure over the REV operation, which nowadays represents, in our mind, the gold standard for repair of these complex anomalies.
More than 20 years after the operation was first performed, we can say that the technique allows us to hope, despite the complexity of the anatomy, in long-term results similar to those of tetralogy of Fallot.
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References
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- Sidi D, Lecompte Y. Transposition and malposition of the great arteries with ventricular septal defect. In: Moller JH, Hoffman JE, editors. Structural heart disease. Churchill Livingstone 1999:363–373.
- Lecompte Y, Neveux JY, Leca F, Zannini L, Tu TV, Duboys Y, Jarreau MM. Reconstruction of the pulmonary outflow tract without a prosthetic conduit. J Thorac Cardiovasc Surg 1982;84:727–733.[Abstract]
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- Bex JP, Lecompte Y, Baillot F, Hazan E. Anatomical correction of transposition of the great arteries. Ann Thorac Surg 1980;29:86–88.[Abstract]
- Nikaidoh H. Aortic translocation and biventricular outflow tract reconstruction. A new surgical repair for transposition of the great arteries associated with a ventricular septal defect and pulmonary stenosis. J Thorac Cardiovasc Surg 1984;88:365–372.[Abstract]
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- Jacobs ML. Discussion of Hu SS. J Thorac Cardiovasc Surg 2008;135:337–338.[Free Full Text]
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Summary
Introduction