Complete Transposition of the Great Arteries

Transcription

1 1 Complete Transposition of the Great Arteries Contents Introduction 2 Anatomy 3 Complete Transposition of the Great Arteries, with or without Ventricular Septal Defect 5 Indication for Surgery 5 Approach and Cardiopulmonary Bypass Strategy 5 Arterial Switch Operation 6 The Goal of Surgery 6 Arterial Switch Operation in Simple Transposition with a Coronary Pattern (1AD; 2R, Cx) 6 Patient Characteristics 6 Specific Steps of Operation 7 Arterial Switch Operation in Situs Inversus with a Coronary Pattern (1R; 2AD, Cx) 13 Patient Characteristics 13 Specific Steps of Operation 14 Arterial Switch Operation with a Coronary Pattern (1R; AD, Cx) Patient Characteristics Specific Steps of Operation 21 Arterial Switch Operation with a Coronary Pattern (2R; AD, Cx) Patient Characteristics Specific Steps of Operation 24 Arterial Switch Operation in Complex Transposition with Crisscross Heart, Multiple Ventricular Septal Defects, Straddling of Tricuspid Valve, Mild Subvalvar and Valvar Pulmonary Stenosis, and with a Coronary Pattern (1Cx; 2R, AD) Patient Characteristics Specific Steps of Operation 27 Complete Transposition of the Great Arteries with Ventricular Septal Defect and Left Ventricular Outflow Tract Obstruction 35 Indication for Surgery 35 Approach and Cardiopulmonary Bypass Strategy 35 REV (Réparation à l étage ventriculaire) Operation 36 The Goal of Surgery 36 V. Hraška, P. Murín, Surgical Management of Congenital Heart Disease I, DOI: / _1, Springer-Verlag Berlin Heidelberg

2 2 V. Hraška, P. Murín Patient Characteristics 37 Specific Steps of Operation 37 Bex Nikaidoh Procedure 43 The Goal of Surgery 43 Patient Characteristics 44 Specific Steps of Operation 44 Recommended Reading 51 Introduction Complete transposition of the great arteries is the most common form of neonatal cyanotic heart disease. Transposition is the result of malformation of the conus arteriosus. This complex always has discordant ventriculoarterial alignment, such that the aorta arises entirely or largely from the right ventricle, and the pulmonary artery arises entirely or largely from above the left ventricle. The general categories of complete transposition of the great arteries are as follows: complete transposition of the great arteries with intact ventricular septum (simple form), complete transposition of the great arteries with a ventricular septal defect (complex form), and complete transposition of the great arteries with a ventricular septal defect and left ventricular outflow tract obstruction. The surgical method of choice for complete transposition of the great arteries without left ventricular outflow tract obstruction is an arterial switch operation, performed during the first weeks of life. Currently, the low operative mortality (<5%), low incidence of reintervention (<10%), and promising functional longterm outcome have been well documented. The optimal treatment strategy for complete transposition of the great arteries combined with a ventricular septal defect and left ventricular outflow tract obstruction remains challenging due to its great range of anatomical variability and unsatisfying long-term results. The Rastelli operation has been the method of choice for the past four decades. The procedure can be performed with low early mortality. However, substantial late morbidity and mortality associated with conduit obstruction, left ventricular outflow tract obstruction, and arrhythmias have been reported. An alternative operation, the réparation à l étage ventriculaire (REV), has the potential to decrease the incidence of left ventricular outflow tract obstruction, thus preserv-

3 1 Complete Transposition of the Great Arteries ing left ventricular function and improving the long-term outcome. In selected patients with a resectable left ventricular outflow tract obstruction, an arterial switch operation with surgery for left ventricular outflow tract obstruction provides excellent long-term outcomes. However, there is the constant hazard of neoaortic valve regurgitation and recurrent left ventricular outflow tract obstruction. In the past, patients with an unfavorable intracardiac anatomy, such as an inlet ventricular septal defect or atrioventricular valve anomalies, underwent single-ventricle palliation. Recently, even in these patients, biventricular correction has become possible using the Bex Nikaidoh procedure. This operation offers many advantages such as straight connections between the left ventricle and the aorta, and the right ventricle and the pulmonary artery. On the other hand, the long-term outcome is unclear. Single-ventricle palliation, i.e., total cavopulmonary connection, remains a valuable option in any form of intracardiac anatomy, independent of the coronary pattern, ventricular septal defect location, or atrioventricular valve morphology. The long-term functional limits of single-ventricle palliation are very well documented. Anatomy The most important feature of complete transposition of the great arteries is the muscular subaortic conus, which widely separates the transposed aortic valve from both atrioventricular valves. Usually, absence of a subpulmonary conus permits direct pulmonary mitral fibrous continuity; therefore, the aortic valve lies at a higher level than the pulmonary valve. The subaortic conus determines the relationship of the aorta to the pulmonary trunk. In the majority of patients with an intact ventricular septum, the aortic root is to the right of the pulmonary trunk, and both arteries have nearly the same diameter. The ventricular septum is much straighter than usual, and the pulmonary valve is not wedged as deeply between the mitral and tricuspid valves, as is the aortic valve in the normal heart. In nearly 75% of the cases of complete transposition of the great arteries, the patients have this simple form, with isolated ventriculoarterial discordance, an intact ventricular septum, a patent foramen ovale, and patent ductus arteriosus. The anatomical situation can be complicated by the presence of a ventricular septal defect, i.e., an obstruction within the left ventricular outflow tract. The presence of a ventricular septal defect is relatively common (40%); however, only approximately 20% of these defects 3

4 4 V. Hraška, P. Murín are hemodynamically significant. The ventricular septal defect can occur anywhere within the interventricular septum. The type of ventricular septal defect with anterior malalignment, where the conal septum deviates into the right ventricular outflow tract (into the subaortic area), can be associated with a hypoplastic aortic annulus and arch-related problems, and/or underdevelopment of the right ventricle. The posterior deviation of the conal septum, with or without overriding of the aortic valve, can produce a subpulmonary obstruction, with hypoplasia of the pulmonary annulus or a bicuspid pulmonary valve, with or without stenosis. Tunnel-like left ventricular outflow obstructions or subpulmonary membranes develop with time and are not usually apparent in newborns. Other rarer forms of stenosis are produced by anomalous attachment of the tension apparatus of the mitral valve across the outflow tract or by aneurysms of fibrous tissue tags bulging into the outflow tract. One should keep in mind the dynamic type of left ventricular outflow tract obstruction caused by the septum bulging to the left due to higher pressure in the systemic right ventricle. Precise echocardiogram diagnosis of the mechanism of the obstruction is crucial in decision-making. The abnormal position of the aortic root, with a variable relationship to the pulmonary trunk, in complete transposition of the great arteries determines the origin and the course of the coronaries. To account for this variability, a classification system is used, irrespective of the relationship of the arterial trunks. The Leiden convention numbers the facing sinuses in the aorta from the perspective of an individual standing within the aorta and facing the pulmonary artery. Sinus 1 is on the observer s right side, and sinus 2 is on the left side. For the most common coronary pattern in complete transposition of the great arteries, the sinus 1 is anatomically leftward and posterior, and gives origin to the anterior descending (AD) and circumflex coronary (Cx) arteries. The sinus 2 is rightward and posterior, giving origin to the right coronary artery (R). The usual coronary artery pattern is labeled 1AD, Cx; 2R. The second, most frequently seen coronary pattern, with circumflex artery arising from sinus 2 and passing posterior to the pulmonary trunk, is labeled 1AD; 2R, Cx. To describe the epicardial course of the major coronary branches, the pathways of the branches around the great vessels are specified. An anterior course indicates passing anterior to the aorta; a posterior course indicates posterior to the pulmonary artery. Even an intramural coronary can pass between the great vessels. A supplemental descriptive classification specifies the origin of the coronaries, taking into consideration the position of the origin of the coronary

5 1 Complete Transposition of the Great Arteries in the sinus, proximity to commissures, separate or remote origins, etc. The most risky coronary pattern is the intramural left coronary artery. From outside, the arrangement seems to be normal; however, clear inspection from inside the aorta usually shows that the main stem of the left coronary artery passes behind the posterior commissures of the aortic valve and takes its origin from sinus 2, which also gives rise to the right coronary artery. The extreme example is the single coronary ostium with an intramural left coronary artery. omplete Transposition of the Great Arteries, C with or without Ventricular Septal Defect Indication for Surgery Diagnosis is indication for surgery. In simple complete transposition of the great arteries, the patent ductus arteriosus is kept open by prostaglandins, and balloon atrial septostomy is performed soon after birth, unless there is free communication on an interatrial level. If there is a ventricular septal defect, balloon atrial septostomy is usually not necessary. The operation is scheduled within the first 2 weeks of life. If, for whatever reason (prematurity, intracranial bleeding, etc.), the operation must be postponed and there is no ventricular septal defect, the patency of the ductus arteriosus is maintained by prostaglandins; alternatively, the patent ductus arteriosus can be stented. It is probably still safe to perform an arterial switch operation in infants with intact ventricular septum and closed duct within up to 6 8 weeks of life, if mechanical support of circulation is available. Otherwise, one should consider left ventricle training. Approach and Cardiopulmonary Bypass Strategy The heart is approached through a median sternotomy. The standard technique of cardiopulmonary bypass with full flow and mild hypothermia (32 C) is used, unless reconstruction of the arch is required, when moderate hypothermia (28 C) is preferable. The aortic cannula is in a high position, close to the base of the innominate artery. Both vena cavae are cannulated. If there is a ventricular septal defect, an angled cannula and direct cannulation of the superior 5

6 6 V. Hraška, P. Murín vena cava is preferable; otherwise, straight cannulas are used. A left ventricular vent (sump sucker) is inserted through the interatrial septum after opening of the right atrium. Arterial Switch Operation The Goal of Surgery The position of the great vessels is switched, and the coronaries are transferred to the neoaorta. Defects on atrial and ventricular levels are closed. rterial Switch Operation in Simple Transposition A with a Coronary Pattern (1AD; 2R, Cx) Patient Characteristics Age at operation: 8 days Diagnosis: 1. Transposition of the great arteries (1AD; 2R, Cx) 2. Secundum atrial septal defect 3. Patent ductus arteriosus History: 1. Prenatally diagnosed 2. After birth, balloon atrial septostomy was performed and treatment with prostaglandins commenced 3. Elective surgery Procedure: 1. Arterial switch operation 2. Direct closure of the atrial septal defect

7 1 Complete Transposition of the Great Arteries 7 Specific Steps of Operation Clip1 Preoperative findings. Clip2 After subtotal removal of the thymus and harvesting of the pericardium, the coronary arteries are examined closely, and the proximal epicardial course is identified.

8 8 V. Hraška, P. Murín Clip3 The pulmonary trunk is marked at the site where the coronaries will be transferred. Extensive circumferential dissection of the great vessels is performed. The aorta is mobilized up to the proximal arch. The pulmonary arteries are thoroughly dissected free, including the first branches in the hilum of the lung, to allow mobility of the vessels. Clip4 The aortic cross-clamp is applied, and antegrade cold crystalloid cardioplegia is delivered. Both great vessels are transected.

9 1 Complete Transposition of the Great Arteries 9 Clip5 Harvesting of the button of the right coronary and circumflex artery. Clip6 Harvesting of the left anterior descending artery.

10 10 V. Hraška, P. Murín Clip7 Implantation of the left anterior descending coronary artery. Clip8 Implantation of the button with the right and circumflex coronary arteries. In order to avoid distortion or kinking of the circumflex artery, the button must be implanted higher than normal on the neoaorta.

11 1 Complete Transposition of the Great Arteries 11 Clip9 Reconstruction of the neopulmonary trunk with an autologous pericardial patch and the neoaortic anastomosis. Clip10 Closure of the atrial septal defect, de-airing of the left part of the heart through the left appendage, and reconstruction of the pulmonary bifurcation on the beating heart during rewarming.

12 12 V. Hraška, P. Murín Clip11 Placement of the left atrial line through the left atrial appendage and insertion of pacing wires. Clip12 Postoperative echocardiogram findings showed good biventricular function, unobstructed left ventricular outflow tract/right ventricular outflow tract.

13 1 Complete Transposition of the Great Arteries 13 fullversion rterial Switch Operation in Situs Inversus A with a Coronary Pattern (1R; 2AD, Cx) Patient Characteristics Age at operation: 5 days Diagnosis: 1. Situs inversus 2. Transposition of the great arteries (1R; 2AD, Cx) 2. Prostaglandins administered after birth; no septo stomy was needed 3. Elective surgery Procedure: 1. Arterial switch operation 3. Secundum atrial septal defect 2. Direct closure of the atrial septal defect 4. Patent ductus arteriosus 3. Transection of the patent ductus arteriosus History: 1. Prenatally diagnosed

14 14 V. Hraška, P. Murín Specific Steps of Operation Clip1 Preoperative findings. Clip2 External anatomy of the heart.

15 1 Complete Transposition of the Great Arteries 15 Clip3 Dissection of the pulmonary arteries, transection of the patent ductus arteriosus, aortic cross-clamp, delivery of cardioplegia. Clip4 Harvesting of the button of the left coronary artery.

16 16 V. Hraška, P. Murín Clip5 Harvesting the button of the right coronary artery. Clip6 Creation of circular opening on the anterior wall of the neoaortic root.

17 1 Complete Transposition of the Great Arteries 17 Clip7 Implantation of the button of the right coronary artery. Clip8 Implantation of the button of the left coronary artery.

18 18 V. Hraška, P. Murín Clip9 Reconstruction of the neopulmonary trunk with an autologous pericardial patch. Clip10 End-to-end anastomosis of the neoaortic root and the ascending aorta.

19 1 Complete Transposition of the Great Arteries 19 Clip11 Closure of the atrial septal defect, de-airing of the left part of the heart through the left appendage, and reconstruction of the pulmonary bifurcation on the beating heart during rewarming. Clip12 Completed arterial switch.

20 20 V. Hraška, P. Murín fullversion Arterial Switch Operation with a Coronary Pattern (1R; AD, Cx) Patient Characteristics Age at operation: 4 days Diagnosis: 1. Transposition of the great arteries (1R; AD, Cx) 2. Small coronary artery for sinus node comes from sinus 2 History: 1. Prenatally diagnosed 2. Prostaglandins administered after birth; no septostomy was needed 3. Elective surgery 3. Secundum atrial septal defect Procedure: 1. Arterial switch operation 4. Patent ductus arteriosus 2. Direct closure of the atrial septal defect 3. Transection of the patent ductus arteriosus

21 1 Complete Transposition of the Great Arteries 21 Specific Steps of Operation Clip1 External anatomy of the heart. Clip2 Mobilization of the right and left coronary arteries.

22 22 V. Hraška, P. Murín Clip3 Implantation of the button of the right and left coronary arteries. Clip4 Reconstruction of the neopulmonary artery.

23 1 Complete Transposition of the Great Arteries 23 Clip5 Completed arterial switch. fullversion

24 24 V. Hraška, P. Murín Arterial Switch Operation with a Coronary Pattern (2R; AD, Cx) Patient Characteristics Age at operation: 2 years and pulmonary artery banding (at a different Diagnosis: 1. Double outlet right ventricle Taussig Bing institution) were performed. heart (2R; AD, Cx) 2. At the age of 4 months, ballooning of the recoarctation was performed. 2. Subpulmonary ventricular septal defect 3. Elective surgery 3. Hypoplastic aortic arch Procedure: 4. Arterial switch operation 4. Patent ductus arteriosus History: 1. At the age of 6 days, patch plasty of the 5. Patch closure of the ventricular septal defect 6. Resection of the subaortic obstruction hypoplastic aortic arch, transection of the 7. Debanding patent ductus arteriosus, atrial septectomy, 8. Direct closure of the atrial septal defect Specific Steps of Operation Clip1 Preoperative angiography.

25 1 Complete Transposition of the Great Arteries 25 Clip2 The operation is conducted in a similar fashion to that of the arterial switch operation for simple transposition. First, the typical subpulmonary ventricular septal defect is closed with a patch, working through the tricuspid valve, and the prominent conal septum is partially transected to release the subaortic obstruction of the right ventricular outflow tract. The clip demonstrates the coronary artery transfer and reconstruction of the aorta and pulmonary artery. fullversion

26 26 V. Hraška, P. Murín rterial Switch Operation in Complex Transposition with A Crisscross Heart, Multiple Ventricular Septal Defects, Straddling of Tricuspid Valve, Mild Subvalvar and Valvar Pulmonary Stenosis, and with a Coronary Pattern (1Cx; 2R, AD) Patient Characteristics Age at operation: 2.5 months Diagnosis: 1. Transposition of the great arteries (1Cx; 2R, AD) 2. Inlet ventricular septal defect History: 1. Prenatally diagnosed 2. After birth, balanced circulation due to mild to moderate left ventricular outflow tract obstruction 3. Mid-muscular ventricular septal defect 3. Elective surgery 4. Straddling of tricuspid valve 5. Mild subvalvar pulmonary stenosis Procedure: 1. Arterial switch operation 6. Mild valvar pulmonary stenosis 2. Patch closure of ventricular septal defects 7. Crisscross heart with side-by-side position 3. Detachment and reposition of straddled papil- of the great vessels and l-position of the aorta lary muscle of the tricuspid valve 4. Resection of subpulmonary obstruction 8. Secundum atrial septal defect 5. Commissurotomy of pulmonary valve 9. Patent ductus arteriosus 6. Transection of patent ductus arteriosus 7. Direct closure of the atrial septal defet

27 1 Complete Transposition of the Great Arteries 27 Specific Steps of Operation Clip1 Preoperative findings. Clip2 After subtotal removal of the thymus and harvesting of the pericardium, the external anatomy is examined closely. The pulmonary trunk is marked at the site where the coronaries will be transferred. Extensive circumferential dissection of the great vessels is performed. The aorta is mobilized up to the proximal arch. The pulmonary arteries are thoroughly dissected free, including the first branches in the hilum of the lung, to allow mobility of the vessels.

28 28 V. Hraška, P. Murín Clip3 Intracardiac anatomy is evaluated. Clip4 Transection of the muscle bar.

29 1 Complete Transposition of the Great Arteries 29 Clip5 Patch closure of ventricular septal defect and detachment of straddling papillary muscle of tricuspid valve. Clip6 Reattachment of the papillary muscle of tricuspid valve.

30 30 V. Hraška, P. Murín Clip7 Transection of the aorta and harvesting of the button of the circumflex artery. Clip8 Harvesting of the left anterior descending artery and the right coronary artery.

31 1 Complete Transposition of the Great Arteries 31 Clip9 Transection of pulmonary artery and Lecompte maneuver. Clip10 Commissurotomy of pulmonary valve (neoaortic valve).

32 32 V. Hraška, P. Murín Clip11 Implantation of the circumflex coronary artery. Clip12 Implantation of the button with the right and left anterior descending coronary arteries.

33 1 Complete Transposition of the Great Arteries 33 Clip13 The neoaortic anastomosis. Clip14 Closure of the atrial septal defect, de-airing of the left part of the heart through the left appendage, and reconstruction of the neopulmonary trunk and pulmonary bifurcation on the beating heart during rewarming.

34 34 V. Hraška, P. Murín Clip15 Postoperative echocardiogram. fullversion

35 1 Complete Transposition of the Great Arteries omplete Transposition of the Great Arteries C with Ventricular Septal Defect and Left Ventricular Outflow Tract Obstruction Indication for Surgery The morphology and severity of the left ventricular outflow tract obstruction and position and size of the ventricular septal defect determine the treatment options for transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction. Intraventricular rerouting is possible if there is a committed and nonrestrictive ventricular septal defect. This anatomy is present in a majority of patients; therefore, the Rastelli operation or the REV procedure is more frequently used. Only a few patients have a resectable left ventricular outflow tract obstruction that allows performance of an arterial switch operation and closure of the ventricular septal defect. The remaining patients have a noncommitted and/or restrictive ventricular septal defect and a small right ventricle, which prevents intraventricular rerouting. If there is no significant straddling of the atrioventricular valves, the Bex Nikaidoh operation might be used; otherwise, the Fontan pathway is an option. Early development of cyanosis, due to a severe left ventricular outflow tract obstruction, often requires palliation (modified Blalock Taussig shunt or stenting of the patent ductus arteriosus) before the final operation. However, a properly chosen treatment plan has the potential to minimize the number of palliations necessary. The arterial switch operation, and the Bex Nikaidoh and REV operations can be performed early in life as a primary correction, thus avoiding palliations and the negative impact of hypoxia, obstruction, and volume overloading over a prolonged period. Corrective operation is accomplished in the majority of patients during the first year of life. Approach and Cardiopulmonary Bypass Strategy The heart is approached through a median sternotomy. The standard technique of cardiopulmonary bypass is used, with full flow and mild hypothermia (32 C). The aortic cannula is in a high position, close to the base of the innominate artery. Both vena cavae are cannulated. The use of an angled cannula and 35

36 36 V. Hraška, P. Murín direct cannulation of the superior vena cava are preferable. A left ventricular vent is inserted through the right upper pulmonary vein. REV (Réparation à l étage ventriculaire) Operation The Goal of Surgery Correction of transposition of the great arteries is provided by an intraventricular tunnel, which connects the left ventricle with the aortic annulus through the ventricular septal defect. The right ventricular outflow tract is developed by direct connection of the pulmonary artery with the right ventriculotomy. The critical step in the intracardiac part of the procedure is the construction of a straight intraventricular tunnel, with the aim of reducing the risk of subaortic obstruction and left ventricular dysfunction. Usually, resection of the conal septum is required in order to commit the ventricular septal defect toward the aorta. Earlier indication for operation, before ventricular septum hypertrophy develops, facilitates the technical aspect of resection and creation of the intraventricular tunnel. Extensive dissection and mobilization of the pulmonary arteries is needed to obtain a tension-free right ventricular outflow tract reconstruction. The pulmonary trunk is transected just above the stenotic valves, and the ascending aorta is divided. Subsequently, the pulmonary artery is translocated anteriorly to the ascending aorta (Lecompte maneuver) and is directly connected with the ventriculotomy. If there is no anteroposterior arrangement of the great vessels, the Lecompte maneuver can be avoided. In many patients, the reconstructed right ventricular outflow tract has growth potential, providing 50 60% freedom from right ventricular outflow tract obstruction in 10 years of follow-up. We prefer to reconstruct the right ventricular outflow tract using only an autologous pericardium patch, without insertion of a monocusp valve. There are two reasons for this choice. First, the monocusp valve is prone to calcification, degeneration, and subsequent valvular dysfunction, and has a tendency to obstruct the right ventricular outflow tract. Second, pulmonary insufficiency is well tolerated if there is no increased pulmonary resistance. In contrast to the Rastelli operation, the risk of compression of the translocated pulmonary artery by the sternum is minimal, even in dextrocardia. Shortening of the ascending aorta and mobilization of the pulmonary artery beyond the pericardial reflection minimize undue traction on the pulmonary artery bifurcation.

37 1 Complete Transposition of the Great Arteries 37 Patient Characteristics Age at operation: 7 months Diagnosis: 1. Transposition of the great arteries with left ventricular outflow tract obstruction 2. Type 1 conal ventricular septal defect 2. At the age of 7 months, semi-elective surgery due to progressive cyanosis Procedure: 1. REV operation 2. Direct connection of the pulmonary artery 3. Valvar and subvalvar pulmonary stenosis with a right ventriculotomy and patch enlarge- 4. Atrial septal defect ment of the right ventricular outflow tract and 5. Patent ductus arteriosus the left pulmonary artery History: 1. At the age of 10 days, stenting of the ductus 3. Direct closure of the atrial septal defect 4. Stent removal arteriosus Specific Steps of Operation Clip1 Preoperative echocardiogram and angiogram.

38 38 V. Hraška, P. Murín Clip2 External anatomy of the heart. Clip3 Right ventriculotomy.

39 1 Complete Transposition of the Great Arteries 39 Clip4 Intracardiac anatomy. Clip5 Creation of the intraventricular tunnel.

40 40 V. Hraška, P. Murín Clip6 Transection of the great arteries. Clip7 Closure of the pulmonary valve and the Lecompte maneuver.

41 1 Complete Transposition of the Great Arteries 41 Clip8 End-to-end anastomosis of the ascending aorta. Clip9 Direct connection of the pulmonary artery with the ventriculotomy and stent removal.

42 42 V. Hraška, P. Murín Clip10 Pericardial patch plasty of the right ventricular outflow tract and the left pulmonary artery. Clip11 Final outcome.

43 1 Complete Transposition of the Great Arteries 43 fullversion B ex Nikaidoh Procedure The Goal of Surgery The Bex Nikaidoh procedure creates better alignment of the right and left ventricular outflow tracts, while correcting the transposition anatomy by posterior aortic translocation. After harvesting the aortic root from the right ventricle, the outlet septum is transected in order to relieve the left ventricular outflow tract obstruction. The divided outlet septum offers excellent visualization of the atrioventricular valve attachments, as well as the ventricular septal defect borders. Therefore, enlargement of the left ventricular outflow tract with the patch can be performed easily, even if there is an inlet and/or a restrictive ventricular septal defect or atrioventricular valve straddling. The translocated aorta is directly committed to the left ventricle. There is no intraventricular baffle with the inherent propensity to postoperative left ventricular outflow tract obstruction, as is typically seen in the Rastelli operation. After the Lecompte maneuver, the right ventricular outflow tract is reconstructed, either by direct right ventricle to pulmonary artery anastomosis or by an orthotopically placed pulmonary homograft. In either case, the pulmonary outflow is less likely to be compressed by the sternum. Modifications to this technique include individual coronary transfer during translocation in order to avoid the possibility of coronary ischemia when the position of the great vessels is not optimal.

44 44 V. Hraška, P. Murín Patient Characteristics Age at operation: 8 weeks Diagnosis: 1. Transposition of the great arteries (1 AD, Cx; 2R) 2. Inlet type of ventricular septal defect 2. At the age of 8 weeks, semi-elective surgery due to progressive cyanosis Procedure: 1. Bex Nikaidoh operation 2. Direct connection of the pulmonary artery 3. Valvar and subvalvar pulmonary stenosis with the right ventriculotomy and patch 4. Secundum atrial septal defect enlargement of the right ventricular outflow History: 1. At the age of 10 days, balloon atrial septec- tract 3. Direct closure of the atrial septal defect tomy Specific Steps of Operation Clip1 Preoperative echocardiogram.

45 1 Complete Transposition of the Great Arteries 45 Clip2 Mobilization of the great vessels. Clip3 Being on pump, the proximal aspects of the coronary arteries are extensively mobilized.

46 46 V. Hraška, P. Murín Clip4 Harvesting the aortic root. Clip5 Transection of both great vessels and transection of the outlet septum.

47 1 Complete Transposition of the Great Arteries 47 Clip6 Excision of pulmonary valve. Clip7 Posterior translocation of the aortic root.

48 48 V. Hraška, P. Murín Clip8 Reconstruction of the aorta. Clip9 Patch closure of the ventricular septal defect.

49 1 Complete Transposition of the Great Arteries 49 Clip10 Downsizing of the right ventricular outflow tract. Clip11 During rewarming, an anterior autologous pericardial patch is utilized to augment the main pulmonary artery and complete the right ventricle to pulmonary artery connection.

50 50 V. Hraška, P. Murín Clip12 Final outcome. fullversion

51 1 Complete Transposition of the Great Arteries 51 Recommended Reading Bautista-Hernandez V, Marx GR, Bacha EA et al (2007) Aortic root translocation plus arterial switch for transposition of the great arteries with left ventricular outflow tract obstruction: intermediate-term results. J Am Coll Cardiol 49: Morell VO, Wearden PD (2008) Technique of aortic translocation for the management of transposition of the great arteries with a ventricular septal defect and pulmonary stenosis. Oper Tech Thorac Cardiovasc Surg 13: Bex JP, Lecompte Y, Baillot F et al (1980) Anatomical correction of transposition of the great arteries. Ann Thorac Surg 29:86 88 Nido del PJ (2005) Transposition of the great arteries (complex forms). In: Sellke FW, del Nido PJ, Swanson SJ (eds) Sabinston and Spencer surgery of the chest, vol. 2, 7th edn. Elsevier Saunders, Philadelphia, pp Dearani JA, Danielson GK, Puga FJ et al (2001) Late results of the Rastelli operation for transposition of the great arteries. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 4:3 15 Gittenberger-de Groot AC, Sauer U et al (1983) Coronary artery anatomy in transposition of the great arteries: a morphologic study. Pediatr Cardiol 4(Suppl):S15 S24 Hu SS, Liu ZG, Li SJ et al (2008) Strategy for biventricular outflow tract reconstruction: Rastelli, REV, or Nikaidoh procedure? J Thorac Cardiovasc Surg 135: Jaggers JJ, Cameron DE, Herlog R et al (2000) Transposition of the great arteries. Annal Thor Surg 69(Suppl):S205 S235 Jonas RA (2004) Transposition of the great arteries. In: Jonas RA (ed) Comprehensive surgical management of congenital heart disease Arnold, London, pp Kreutzer C, De Vive J, Oppido G et al (2000) Twenty-five year experience with Rastelli repair for transposition of the great arteries. J Thorac Cardiovasc Surg 120: Lecompte Y, Vouhé P (2003) Réparation à l Etage Ventriculaire (REV procedure): not a Rastelli procedure without conduit. Oper Tech Thorac Cardiovasc Surg 8: Losay J, Touchot A, Serraf A et al (2001) Late outcome after arterial switch operation for transposition of the great arteries. Circulation 104(Suppl1): Morell VO, Wearden PD (2008) Nikaidoh operation for transposition of the great arteries with a ventricular septal defect and pulmonary stenosis. MMCTS. doi: /mmcts Nikaidoh H (1984) 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 Thoracic Cardiovasc Surg 88: Prêtre R, Gendron G, Tamisier D et al (2001) Results of the LeCompte procedure in malposition of the great arteries and pulmonary obstruction. Eur J Cardiothorac Surg 19: Rastelli GC, Wallace RB, Ongley PA (1969) 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 39:83 95 Salih C, Brizard CH, Penny DJ et al (2010) Transposition. In: Anderson RH, Becker EJ, Penny D et al (eds) Pediatric cardiology, 3rd edn. Churchill-Livingstone, Philadelphia, pp, Wetter J, Belli E, Sinzobahamvya N et al (2001) Transposition of the great arteries associated with ventricular septal defect: surgical results and long-term outcome. Eur J Cardiothorac Surg 20: Weyand K, Haun C, Blaschczok H et al (2010) Surgical treatment of transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction: midtermresults. World J Pediatr Congenital Heart Surg 2: