The Rastelli procedure has been traditionally used for repair

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En-bloc Rotation of the Truncus Arteriosus A Technique for Complete Anatomic Repair of Transposition of the Great Arteries/Ventricular Septal Defect/Left Ventricular Outflow Tract Obstruction or Double Outlet Right Ventricle/Left Ventricular Outflow Tract Obstruction Rudolf Mair, MD The Rastelli procedure has been traditionally used for repair of transposition of the great arteries (TGA) with ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO), or double outlet right ventricle with subpulmonary VSD and LVOTO. 1 The Rastelli procedure in its original version involves an intracardiac baffle, which forms the left ventricular outflow tract from the VSD to the aortic valve. This results in a long intracardiac tunnel and has an inherent tendency to obstruction. A homograft or xenograft is also needed for reconstruction of the right ventricular outflow tract. Resection of the conal septum and direct anastomosis between the main pulmonary artery and the right ventricle as in the Lecompte procedure, 2 or reparation a l etage ventriculaire (REV), can minimize these problems but does not completely solve them. Although there is growth potential in the pulmonary artery after a REV procedure, there is no valve in the right ventricular outflow tract. Therefore, one can expect similar problems as follow tetralogy repair with a transannular patch. Aortic translocation (Nikaidoh procedure) brings the aorta directly over the left ventricular outflow tract. 3 The pulmonary valve is also removed in this procedure. In its original Department of Cardiovascular Surgery, General Hospital Linz, Linz, Austria. Address reprint requests to Rudolf Mair, MD, Department of Cardiovascular Surgery, General Hospital Linz, Krankenhausstr. 9, A-4020 Linz, Austria. E-mail: Rudolf.mair@akh.linz.at version, it does not place a valve in the right ventricular outflow tract and does not include a backwall to the pulmonary artery. In many patients, the right coronary artery has to be transferred anteriorly, as reported by Dr. Nikaidoh himself, as well as other authors. 3 In many patients suitable for the Rastelli, REV, or Nikaidoh procedures, the stenosis in the left ventricular outflow tract is subvalvar. The pulmonary valve is often bicuspid or dysplastic. However, the valve may be appropriate for use in the right ventricular outflow tract. En-bloc rotation of the complete truncus arteriosus (the aortic and pulmonary roots) enables one to bring the aortic root over the left ventricular outflow tract, and the pulmonary root over the right ventricular outflow tract. This procedure was first published by Yamagishi and coworkers in a case report in 2003. 4 They used a monocusp to enlarge the pulmonary root. In our series of en-bloc rotation of the truncus arteriosus, we have preserved the native pulmonary valve in six of eight patients. 5 The pulmonary valve was either bicuspid or dysplastic but competent and with a sufficient orifice. The remaining two patients in our series required a pulmonary transannular patch. Subvalvar enlargement of the pulmonary root was usually adequate to relieve LVOTO. The coronary anatomy is critical in this procedure. An important coronary artery crossing the right ventricular outflow tract close to the aortic root makes this procedure impossible. 1522-2942/09/$-see front matter 2009 Published by Elsevier Inc. doi:10.1053/j.optechstcvs.2009.02.002 45

46 R. Mair Operative Technique Figure 1 The aorta is cross-clamped. Cardioplegia is administered; the right atrium is opened, and the patent foramen ovale is closed. The aorta is cut transversely at the location of the cardioplegia needle. Both coronaries are excised as U-shaped buttons and mobilized in the same manner as in an arterial switch procedure. Ao. aorta; MPA main pulmonary artery.

En-bloc rotation of the truncus arteriosus 47 Figure 2 The pulmonary artery is cut transversely at the same height as the aorta. The pulmonary valve and VSD are inspected. Marking stitches are placed: two in the anterior part of the right ventricular outflow tract and two precisely at the opposite side behind the pulmonary artery. Similar to a Ross procedure, a transverse incision is performed in the RVOT between the anterior marking stitches and is continued step by step around the aortic valve. At the area of the conal septum, the external incision stops at both sides. The conal septum is incised from inside parallel to the former incision and great attention is paid not to injure the pulmonary valve. This is especially important in TGA/VSD/LVOTO, as there is no subpulmonary conus. As soon as the external incision and the transection of the conal septum are connected on both sides, the external incision is continued around the pulmonary root. In the case of TGA, the transection goes through the area of pulmonary-mitral fibrous continuity and comes close to the posterior cusp of the pulmonary valve. If the patient has a double outlet right ventricle, there will be a subpulmonary conus, making the excision of the pulmonary root somewhat easier. Ao. aorta; MPA main pulmonary artery.

48 R. Mair Figure 3 Following en-bloc excision of the truncus arteriosus, the residual part of the conal septum, which comprises the superior border of the VSD, is carefully inspected for insertion of a tricuspid valve papillary muscle. An incision in the conal septum is made anterior to the papillary muscle, so that the latter stays on the side of the tricuspid valve. m. muscle; VSD ventricular septal defect.

En-bloc rotation of the truncus arteriosus 49 Figure 4 The truncus arteriosus is now reimplanted after rotation by 180, so that the aortic root lies exactly over the LVOT (marking stitches). Reimplantation is started at the posterior marking stitch with a continuous suture using 6-0 absorbable monofilament material. It is continued anteriorly up to both ends of the transected conal septum. The residual part of the subaortic circumference is now filled with a VSD patch. We use a glutaraldehyde-treated pericardial patch, which is implanted by a continuous suture using 6-0 nonabsorbable monofilament material. Reconstruction and appropriate enlargement of the subaortic part of the LVOT is now finished. The absorbable suture used for the truncus arteriosus is now continued a few millimeters anteriorly around the right ventricular outflow tract on each side, to make reimplantation of the coronaries more straightforward.

50 R. Mair Figure 5 Reimplantation of the coronaries: usually the left coronary artery (LCA) is reimplanted first, followed by the right coronary artery (RCA). In most cases the distances between the origins of the coronary arteries and their reimplantation sites are very short. In our experience, only one trapdoor-plasty was necessary to gain some length. In all other cases reimplantation has been done by simple direct anastomosis. LCA left coronary artery; MPA main pulmonary artery; RCA right coronary artery; VSD ventricular septal defect.

En-bloc rotation of the truncus arteriosus 51 Figure 6 A Lecompte maneuver is performed, and the ascending aorta and original aortic root are reconnected by an end-to-end anastomosis using a continuous suture of 6-0 absorbable monofilament material. The left heart is accurately deaired and the cross-clamp is removed. Usually the heart starts beating immediately. The color of the ventricles is observed, and the coronaries should be properly filled with blood. There should be neither tension nor kinking of the coronaries.

52 R. Mair Figure 7 The pulmonary valve is now inspected and sized. In many cases, it is bicuspid, and in other cases, it is tricuspid and dysplastic. However, in the majority of cases it is appropriate for use in the right ventricular outflow tract. In only two of the eight patients in our series, a transannular patch was necessary. The left atrial vent is now placed in the right atrium or right ventricle. The suture line between the pulmonary part of the truncus arteriosus and the right ventricular outflow tract is then completed from both sides. Usually the pulmonary root is too small to fill the right ventricular outflow tract, so that subvalvar patch enlargement is necessary. An incision is made from the base of the pulmonary root up into the commissure of the pulmonary valve, carefully preserving the valve. If the pulmonary valve is bicuspid, only one incision is necessary; this is usually located in the midline. If the valve is tricuspid, two incisions have to be made, one under each anterolateral commissure (triangles). These incisions are filled with triangular pericardial patches.

En-bloc rotation of the truncus arteriosus 53 Figure 8 The final step is the anastomosis between the main pulmonary artery and the pulmonary root. It is performed with absorbable monofilament suture material (6-0). Following closure of the right atrium, pulmonary artery and left atrial lines are placed, as well as atrial and ventricular pacing wires. Cardiopulmonary bypass is weaned. Cannulae are removed; chest tubes are placed, and the chest is closed in standard fashion. The procedure begins with a median sternotomy, partial resection of the thymus, and opening of the pericardium by harvesting a pericardial patch. The anatomy is assessed carefully. The coronaries are inspected. To date, we have done this procedure only if the coronary anatomy is usual for transposition. We do not do it if the circumflex coronary artery originates from the right coronary. A major coronary artery crossing the right ventricular outflow tract close to the aortic root (right coronary artery from left anterior descending coronary artery, or left anterior descending coronary artery from right coronary artery) is also a contraindication to this procedure. Cardiopulmonary bypass is instituted. Arterial cannulation is done in the ascending aorta or proximal aortic arch. Bicaval venous cannulation is used. A left atrial vent is inserted. A line for cardioplegia is put into the ascending aorta at the place of the planned transection, as in an arterial switch procedure. Conclusions To date, our results with this procedure are very encouraging. Since 2003 we have a series of eight patients. All of them survived the procedure. All patients have growth potential in the reconstructed right and left ventricular outflow tracts. Two patients came to us via a human aid organization from Albania, so they are not in our follow-up program. None of the six patients who have been followed for up to 5 years has a gradient in the left ventricular outflow tract. One patient has an echocardiographic gradient across the pulmonary valve of approximately 40 mmhg. This has not increased over the last 3 years. Another patient shows a gradient at the pulmonary bifurcation of 36 mm Hg. To date she has not needed any intervention. One patient needed a permanent pacemaker for second-degree heart block 2 months after the operation.

54 R. Mair References 1. Rastelli GC, Wallace RB, Ongley PA: Complete repair of transposition of the great arteries with pulmonary stenosis. Circulation 39:83-95, 1969 2. Lecompte Y, Neveux JY, Leca F, et al: Reconstruction of the pulmonary outflow tract without prosthetic conduit. J Thorac Cardiovasc Surg 84: 727-733, 1982 3. Nikaidoh H: Aortic translocation and biventricular outflow tract reconstruction. J Thorac Cardiovasc Surg 88:365-372, 1984 4. Yamagishi M, Shuntoh K, Matsushita T, et al: Half-turned truncal switch operation for complete transposition of the great arteries with ventricular septal defect and pulmonary stenosis. J Thorac Cardiovasc Surg 125: 966-968, 2003 5. Mair R, Sames-Dolzer E, Vondrys D, et al: En bloc rotation of the truncus arteriosus-an option for anatomic repair of transposition of the great arteries, ventricular septal defect and left ventricular outflow tract obstruction. J Thorac Cardiovasc Surg 131:740-741, 2006