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The Rastelli Operation

      The Rastelli operation was conceived by Gian Carlo Rastelli as a surgical repair for children born with transposition of the great arteries (TGA), ventricular septal defect (VSD), and pulmonary stenosis.
      • Rastelli GC
      A new approach to “anatomic” repair of transposition of the great arteries.
      The operation is based on a re-direction of ventricular outflows: an intracardiac baffle tunnels the left ventricle to the aorta, and an external valved conduit connects the right ventricle and the pulmonary artery. This was the first operation described for TGA that incorporated the left ventricle as the systemic ventricle. This procedure was first successfully performed by Dr. Robert Wallace at the Mayo Clinic on July 26, 1968.
      • Rastelli GC
      • Wallace RB
      • Ongley PA
      Complete repair of transposition of the great arteries with pulmonary stenosis: A review and report of a case corrected by using a new surgical technique.
      The term “Rastelli procedure” has become a generic phrase applied to patients who undergo an operation that includes a VSD closure with an extracardiac ventricle to pulmonary artery conduit. This includes patients with truncus arteriosus, VSD with pulmonary atresia, and double outlet right ventricle, among others.
      This article will address the specific operative techniques of the Rastelli operation in its classical application for a patient with D-Transposition of the Great Arteries (D-TGA), VSD, and pulmonary stenosis. These patients present with cyanosis in the newborn period and are often maintained on a prostaglandin E1 infusion. This procedure keeps the ductus arteriosus patent to keep their saturations acceptable in the newborn period. The typical patient with this anatomy has as the initial palliative procedure a modified Blalock-Taussig shunt. Following the modified Blalock-Taussig shunt, they have pulmonary blood flow both through the stenotic pulmonary valve and across the shunt. This process often allows them to grow to an age of 1 to 1½ years and a weight of 6 to 10 kg. The Rastelli operation may then be performed as the second procedure. In the past (or for patients who grow slowly), a second, modified Blalock-Taussig shunt may be performed as part of the overall strategy of patient treatment. However, in our experience, this has become less and less common, and most of these patients will undergo the Rastelli operation as their second procedure.

      Surgical Technique

      The operation illustrated in the following drawings is based on a procedure performed on a 1½-year-old child born with TGA, VSD, and pulmonary stenosis. At 7 days of age, the child underwent a 4-mm Gore-Tex (W.L. Gore & Associates, Phoenix, AZ) modified Blalock-Taussig shunt from the innominate artery to the right pulmonary artery through a median sternotomy approach. The illustrations are all depicted from the surgeon's view. At preoperative evaluation, cardiac catheterization showed a right ventricular pressure of 90/10 mm Hg, with a simultaneous left ventricular pressure of 92/10 mm Hg. The lateral view of the right ventriculogram showed a large but slightly restrictive VSD and the transposed aorta originating from the right ventricle. An injection in the shunt showed prompt filling of right and left pulmonary arteries. The echocardiogram confirmed the intracardiac anatomy and showed normal atrioventricular valves. This operation is routinely performed with a transesophageal echocardiogram in place for preoperative and postoperative analysis. The valve conduit that is inserted is a Carpentier-Edwards porcine valved conduit (Edwards Lifesciences, Irvine, CA). The conduit should be pre-clotted (ie, the size should be selected, the conduit washed, and blood drawn from the patient before heparinization to preclot the valve).
      Figure thumbnail fx1
      1Cardiopulmonary bypass, shunt ligation, and division. The heart has been exposed through a repeat median sternotomy incision. The patient is on cardiopulmonary bypass with a distal aortic cannula and bicaval venous cannulation. The superior vena cava (SVC) has been cannulated via the right atrial appendage. The inferior vena cava (IVC) has been cannulated at the right atrial IVC junction. The aorta is being retracted to the left. The shunt is being clipped and divided. Two hemoclips are placed proximally, I distally. It is important to divide the shunt so that there is no traction on the pulmonary artery (causing late pulmonary artery stenosis) as the child grows. The shunt should be clipped immediately on commencing cardiopulmonary bypass to prevent a “steal” of blood into the pulmonary circulation with resultant low systemic perfusion. Ao, aorta; RA, right atrium; RPA, right pulmonary artery; RV, right ventricle.
      Figure thumbnail fx2
      2Aortic cross-clamp and cardioplegia. This is the beginning of the aortic cross-clamp time. The aortic cross-clamp is in place, and cold blood cardioplegia is being injected into the ascending aorta. Note that a vent has been placed in the right superior pulmonary vein (RSPV). This vent crosses through the left atrium, across the mitral valve, and into the left ventricle to decompress the heart. This process will also provide a bloodless field at the time of ventricular septal defect (VSD) closure.
      Figure thumbnail fx3
      3Right ventriculotomy. (A) The right ventricular (RV) opening is being enlarged. An incision has been made in the right ventricular outflow tract. Muscle bundles are resected on both sides of the incision. This ensures that there will not be stenosis at the orifice of the right ventricular outflow to the conduit. As illustrated, the muscle bundles are excised with a scissors. (B) The right ventricular incision must be placed so that it does not interfere with major epicardial coronary vessels. These usually should be identified at the time of the initial Blalock-Taussig shunt or by cardiac catheterization. The incision in the right ventricle should also not be placed too close to the base of the heart where the great vessels are located because there might be inadvertent injury to the aortic valve.
      Figure thumbnail fx4
      4Enlarging the ventricular septal defect (VSD). (A) The use of a #11 blade to resect the anterosuperior margin of the septum to provide unobstructed flow from the left ventricle to the aorta is shown. The area to be resected is indicated by the dotted lines. Note in the Boston series there was no relationship between VSD enlargement and postoperative atrioventricular (AV) block.
      • Kreutzer C
      • DeVive J
      • Oppido G
      • et al.
      Twenty-five year experience with Rastelli repair for transposition of the great arteries.
      The conduction system should be away from this excision site, clockwise to the muscle of Lancisi. (B) A dilator can be used to compare the size of the aortic valve with the size of the created VSD.
      Figure thumbnail fx5
      5Ventricular septal defect (VSD) suture placement. The perimeter of the VSD is surrounded with interrupted pledgetted sutures (4-0 or 5-0). The large size of the VSD and the fact that what is being constructed is an intracardiac baffle tunneling the left ventricle to the aorta necessitates a long suture line. This frequently requires anywhere from 20 to 25 interrupted sutures. At the posterior-inferior aspect of the VSD, relatively superficial bites must be taken to avoid the conduction system between the muscle of Lancisi and the tricuspid valve annulus.
      Figure thumbnail fx6
      6Dacron patch insertion. (A) A large Dacron (Medox Medical Inc., Oakland, New Jersey) patch is cut to act as a baffle directing the left ventricular blood to the aorta. It is imperative that this patch be large enough to prevent early or late left ventricular outflow tract stenosis. The size of the patch can be assessed with a dilator. All the sutures are sequentially passed through the edge of the Dacron patch, which is lowered into the field, and the sutures are all tied and cut. Some surgeons have preferred using a Dacron conduit that has been cut longitudinally. (B) Dacron patch in place with all the sutures tied.
      Figure thumbnail fx7
      7Oversewing the pulmonary valve. The main pulmonary artery (MPA) in these patients is usually posterior and to the left. It must be transected and oversewn as it is committed to the left ventricle that is now also directed to the aorta. Our practice has been to oversew the proximal pulmonary artery stump with running Prolene (Ethicon, Inc., Somerville, NJ) suture in 2 layers, the first being a mattress suture and the second an over-and-over suture. This suture should incorporate the pulmonary valve leaflets so that thrombus does not form above the leaflets and act as a potential nidus for clot formation that could lead to systemic emboli. This suture line is reinforced with pledgetted sutures.
      Figure thumbnail fx8
      8Closing the patent foramen ovale. The right atrium is opened with a small oblique incision and the patent foramen ovale (PFO) is either closed with a single mattress suture, 2 mattress sutures, or with a running Prolene suture. The heart at this point may be de-aired through the patent foramen ovale and the cross-clamp removed. Alternatively, a portion of the conduit can be constructed with the cross-clamp still in place.
      Figure thumbnail fx9
      9Conduit preparation. The conduit used in this operation is a Carpentier-Edwards porcine valved conduit (Edwards Lifesciences, Irvine, CA), manufactured with the valve inside a Dacron conduit. The conduit is preclotted by immersing it in blood drawn from the patient (usually the right atrium) before heparinization. The conduit should be cut so that the distance between the valve and the pulmonary artery is as short as possible. The proximal portion of the conduit is cut in a bevel shape that will mirror the opening created in the right ventricular outflow tract. This procedure allows the pulmonary valve to be distal and away from the sternotomy incision where it might be compressed.
      Figure thumbnail fx10
      10Distal conduit anastomosis. The conduit is being anastomosed to the distal pulmonary artery with the aortic cross clamp off. This anastomosis is being performed with running Prolene suture, in a parachute fashion. Ao, aorta; RA, right atrium; RV, right ventricle.
      Figure thumbnail fx11
      11Proximal conduit anastomosis. This anastomosis is facilitated by using a Teflon (DuPont, Wilmington, Delaware) felt strip along the edge of the ventriculotomy. Prolene suture is used to anastomose the ventricle to the proximal conduit. The Teflon felt helps to ensure that the sutures do not pull through the right ventricular muscle and improves hemostasis at the conclusion of the procedure. (A) The first suture is a horizontal mattress suture with the knot tied inside the conduit (ie, Ebert technique).
      Figure thumbnail fx12
      11a(B) The anastomosis is a running Prolene suture that includes the conduit, Teflon felt, and right ventricle muscle.
      Figure thumbnail fx13
      12De-airing. The conduit is almost completely sutured in place. The right ventricle and the main pulmonary artery are de-aired through this opening by partially occluding the venous return just before completing the anastomosis.
      Figure thumbnail fx14
      13Finished conduit. The conduit, when filled with blood, distends out as shown. All the cannulae have been removed.

      Outcomes

      The 2 largest reported series reviewing the outcome of the Rastelli operation for TGA are from Boston Children's Hospital and the Mayo Clinic.
      • Kreutzer C
      • DeVive J
      • Oppido G
      • et al.
      Twenty-five year experience with Rastelli repair for transposition of the great arteries.
      • Dearani JA
      • Danielson GK
      • Puga FJ
      • et al.
      Late results of the Rastelli operation for transposition of the great arteries.
      Kreutzer and colleagues reviewed the results of the Rastelli repair for TGA at Boston Children's Hospital in 101 patients operated on from 1973 to 1998.
      • Kreutzer C
      • DeVive J
      • Oppido G
      • et al.
      Twenty-five year experience with Rastelli repair for transposition of the great arteries.
      Median age and weight at the time of the Rastelli operation were 3.1 years and 12.8 kg, respectively. Pulmonary stenosis was present in 70% of the patients, pulmonary atresia in 20%. Operative mortality was 7%, with no operative deaths in the last 7 years of the study. Risk factors for early death included straddling tricuspid valve and longer aortic cross-clamp times. Median length of hospital stay was 9 days, and 6% percent of patients had postoperative atrioventricular block. VSD enlargement with resection of the anterosuperior margin of the defect was not associated with either complete or transient atrioventricular block. Eleven patients (10%) required reoperation for left ventricular outflow tract obstruction. Nine patients had late arrhythmias, and there were 5 late sudden deaths. Conduit replacement was performed in 44 of 101 patients, 50% of these reoperations occurred by 7 years postoperatively. Overall, the 10-year survival was 70%.
      The Mayo Clinic experience for patients with TGA who underwent a Rastelli operation was reported by Dearani and associates in 2001.
      • Dearani JA
      • Danielson GK
      • Puga FJ
      • et al.
      Late follow-up of 1095 patients undergoing operation for complex congenital heart disease utilizing pulmonary ventricle to pulmonary artery conduits.
      They studied 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. The early results in this series were complicated by patients with significant pulmonary hypertension. Evolving techniques of cardioplegia, cardiopulmonary bypass, and postoperative care have significantly improved the early mortality. In this series, the early mortality decreased from 24% for the years 1968 through 1977 to 4% from 1988 to 1997. Sixty-four percent of the patients were males, 36% female. Age at operation ranged from 1 to 44 years, with a mean age of 9.7 years and a median age of 8 years. The most common prior palliative operation was a Blalock-Taussig shunt. The causes of late death were sudden death and/or arrhythmia in 17 patients (11%), congestive heart failure in 12, and pulmonary hypertension in 4. In a comprehensive review of 1,095 patients from the Mayo Clinic undergoing a ventricle to pulmonary artery conduit, early mortality for conduit replacement in 306 patients operated on between 1964 and 2001 was 4.9%, with a 1.7% mortality for conduit replacement since 1989.
      • Dearani JA
      • Danielson GK
      • Puga FJ
      • et al.
      Late follow-up of 1095 patients undergoing operation for complex congenital heart disease utilizing pulmonary ventricle to pulmonary artery conduits.

      Conclusion

      The Rastelli operation remains the procedure of choice at many institutions throughout the world for children with TGA, VSD, and pulmonary stenosis. The early mortality of this procedure has been reduced to less than 5% at most institutions.
      • Kreutzer C
      • DeVive J
      • Oppido G
      • et al.
      Twenty-five year experience with Rastelli repair for transposition of the great arteries.
      • Dearani JA
      • Danielson GK
      • Puga FJ
      • et al.
      Late results of the Rastelli operation for transposition of the great arteries.
      These patients will require reoperation for conduit change, although these procedures may be performed with low morbidity and mortality.
      • Dearani JA
      • Danielson GK
      • Puga FJ
      • et al.
      Late follow-up of 1095 patients undergoing operation for complex congenital heart disease utilizing pulmonary ventricle to pulmonary artery conduits.
      Attention to adequate resection of the anterosuperior portion of the ventricular septum to enlarge the VSD has nearly eliminated the need for reoperation for left ventricular outflow tract obstruction in most series. The incidence of late sudden death from arrhythmia remains to be explained and is a potentially problematic long-term complication.

      References

        • Rastelli GC
        A new approach to “anatomic” repair of transposition of the great arteries.
        Mayo Clin Proc. 1969; 44: 1-12
        • Rastelli GC
        • Wallace RB
        • Ongley PA
        Complete repair of transposition of the great arteries with pulmonary stenosis: A review and report of a case corrected by using a new surgical technique.
        Circulation. 1969; 39: 83-95
        • Kreutzer C
        • DeVive J
        • Oppido G
        • et al.
        Twenty-five year experience with Rastelli repair for transposition of the great arteries.
        J Thorac Cardiovasc Surg. 2000; 120: 211-223
        • Dearani JA
        • Danielson GK
        • Puga FJ
        • et al.
        Late results of the Rastelli operation for transposition of the great arteries.
        Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2001; 4: 3-15
        • Dearani JA
        • Danielson GK
        • Puga FJ
        • et al.
        Late follow-up of 1095 patients undergoing operation for complex congenital heart disease utilizing pulmonary ventricle to pulmonary artery conduits.
        Ann Thorac Surg. 2003; 75: 399-411