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1 1 V. Hraška, P. Murín, Surgical Management of Congenital Heart Disease I, DOI:10.1007/978-3-642-24169-7_1, © Springer-Verlag Berlin Heidelberg 2012 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) 20 Patient Characteristics 20 Specific Steps of Operation 21 Arterial Switch Operation with a Coronary Pattern (2R; AD, Cx) 24 Patient Characteristics 24 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) 26 Patient Characteristics 26 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

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Page 1: Complete Transposition 1 of the Great Arteriesextras.springer.com/2012/978-3-642-24168-0/DVD-1/01_Chap.pdf · 6 V. Hraška, P. Murín. Arterial Switch Operation in Simple Transposition

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V. Hraška, P. Murín, Surgical Management of Congenital Heart Disease I, DOI:10.1007/978-3-642-24169-7_1, © Springer-Verlag Berlin Heidelberg 2012

Contents

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) 20 ◙ Patient Characteristics 20 ◙ �Specific�Steps�of�Operation� 21

◙ Arterial Switch Operation with a Coronary Pattern (2R; AD, Cx) 24 ◙ Patient Characteristics 24 ◙ 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) 26

◙ Patient Characteristics 26 ◙ �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

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Introduction

Complete transposition of the great arteries is the most common form of neo-natal cyanotic heart disease. Transposition is the result of malformation of the conus arteriosus. This complex always has discordant ventriculoarterial align-ment, 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 fol-lows: 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 long-term 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 de-crease�the�incidence�of�left�ventricular�outflow�tract�obstruction,�thus�preserv-

◙ 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

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31 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�pro-vides excellent long-term outcomes. However, there is the constant hazard of neoaortic� valve� regurgitation� and� recurrent� left� ventricular� outflow� tract� ob-struction. In the past, patients with an unfavorable intracardiac anatomy, such as an inlet ventricular septal defect or atrioventricular valve anomalies, under-went single-ventricle palliation. Recently, even in these patients, biventricular correction has become possible using the Bex–Nikaidoh procedure. This op-eration 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 major-ity 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 transposi-tion 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 compli-cated 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

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are�hemodynamically�significant.�The�ventricular�septal�defect�can�occur�any-where 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�hy-poplastic 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 obstruc-tion, 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 attach-ment�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 clas-sification�system�is�used,�irrespective�of�the�relationship�of�the�arterial�trunks.�The Leiden convention numbers the facing sinuses in the aorta from the per-spective 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�si-nus 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 in-dicates 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

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51 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.

Complete Transposition of the Great Arteries, 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,� intra-cranial bleeding, etc.), the operation must be postponed and there is no ven-tricular 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 hypother-mia�(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 ven-tricular septal defect, an angled cannula and direct cannulation of the superior

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Arterial Switch Operation in Simple Transposition with a Coronary Pattern (1AD; 2R, Cx)

Patient Characteristics

Age at operation: 8 daysDiagnosis: 1. Transposition of the great arteries

(1AD; 2R, Cx)2. Secundum atrial septal defect3. Patent ductus arteriosusHistory:1. Prenatally diagnosed

2. After birth, balloon atrial septostomy was performed and treatment with prostaglandins commenced

3. Elective surgeryProcedure:1. Arterial switch operation2. Direct closure of the atrial septal defect

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 SurgeryThe position of the great vessels is switched, and the coronaries are transferred to the neoaorta. Defects on atrial and ventricular levels are closed.

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Specific Steps of Operation

Clip1

Preoperative�findings.

Clip2

After subtotal removal of the thymus and harvest-ing of the pericardium, the coronary arteries are examined closely, and the proximal epicardial course�is�identified.

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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 proxi-mal 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.

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Clip5

Harvesting of the button of the right coronary and circumflex�artery.

Clip6

Harvesting of the left anterior descending artery.

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Clip7

Implantation of the left anterior descending coro-nary 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.

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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.

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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 ven-tricular�outflow�tract/right�ventricular�outflow�tract.

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fullversion

Arterial Switch Operation in Situs Inversus with a Coronary Pattern (1R; 2AD, Cx)

Patient Characteristics

Age at operation: 5 daysDiagnosis: 1. Situs inversus2. Transposition of the great arteries

(1R; 2AD, Cx)3. Secundum atrial septal defect4. Patent ductus arteriosus History:1. Prenatally diagnosed

2. Prostaglandins administered after birth; no septo stomy was needed

3. Elective surgeryProcedure:1. Arterial switch operation2. Direct closure of the atrial septal defect3. Transection of the patent ductus arteriosus

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Specific Steps of Operation

Clip1

Preoperative�findings.

Clip2

External anatomy of the heart.

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151 Complete Transposition of the Great Arteries

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.

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Clip5

Harvesting the button of the right coronary artery.

Clip6

Creation of circular opening on the anterior wall of the neoaortic root.

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171 Complete Transposition of the Great Arteries

Clip7

Implantation of the button of the right coronary artery.

Clip8

Implantation of the button of the left coronary artery.

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Clip9

Reconstruction of the neopulmonary trunk with an autologous pericardial patch.

Clip10

End-to-end anastomosis of the neoaortic root and the ascending aorta.

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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.

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fullversion

Arterial Switch Operation with a Coronary Pattern (1R; AD, Cx)

Patient Characteristics

Age at operation: 4 daysDiagnosis: 1. Transposition of the great arteries (1R; AD,

Cx) 2. Small coronary artery for sinus node comes

from sinus 23. Secundum atrial septal defect4. Patent ductus arteriosus

History:1. Prenatally diagnosed2. Prostaglandins administered after birth; no

septostomy was needed3. Elective surgeryProcedure:1. Arterial switch operation2. Direct closure of the atrial septal defect3. Transection of the patent ductus arteriosus

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Specific Steps of Operation

Clip1

External anatomy of the heart.

Clip2

Mobilization of the right and left coronary arteries.

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Clip3

Implantation of the button of the right and left coronary arteries.

Clip4

Reconstruction of the neopulmonary artery.

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Clip5

Completed arterial switch.

fullversion

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Arterial Switch Operation with a Coronary Pattern (2R; AD, Cx)

Patient Characteristics

Age at operation: 2 yearsDiagnosis: 1. Double outlet right ventricle – Taussig–Bing

heart (2R; AD, Cx) 2. Subpulmonary ventricular septal defect3. Hypoplastic aortic arch4. Patent ductus arteriosusHistory:1. At the age of 6 days, patch plasty of the

hypoplastic aortic arch, transection of the patent ductus arteriosus, atrial septectomy,

and pulmonary artery banding (at a different institution) were performed.

2. At the age of 4 months, ballooning of the re-coarctation was performed.

3. Elective surgeryProcedure:4. Arterial switch operation5. Patch closure of the ventricular septal defect6. Resection of the subaortic obstruction7. Debanding8. Direct closure of the atrial septal defect

Specific Steps of Operation

Clip1

Preoperative angiography.

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251 Complete Transposition of the Great Arteries

Clip2

The operation is conducted in a similar fashion to that of the arterial switch operation for simple transposition. First, the typical subpulmonary ven-tricular septal defect is closed with a patch, work-ing 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

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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

Age at operation: 2.5 monthsDiagnosis: 1. Transposition of the great arteries

(1Cx; 2R, AD)2. Inlet ventricular septal defect3. Mid-muscular ventricular septal defect4. Straddling of tricuspid valve5. Mild subvalvar pulmonary stenosis6. Mild valvar pulmonary stenosis7. Crisscross heart with side-by-side position

of the great vessels and l-position of the aorta

8. Secundum atrial septal defect9. Patent ductus arteriosus

History:1. Prenatally diagnosed2. After birth, balanced circulation due to mild to

moderate left ventricular outflow tract ob-struction

3. Elective surgeryProcedure:1. Arterial switch operation2. Patch closure of ventricular septal defects3. Detachment and reposition of straddled papil-

lary muscle of the tricuspid valve4. Resection of subpulmonary obstruction5. Commissurotomy of pulmonary valve6. Transection of patent ductus arteriosus 7. Direct closure of the atrial septal defet

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Specific Steps of Operation

Clip1

Preoperative�findings.

Clip2

After subtotal removal of the thymus and harvest-ing 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 thor-oughly�dissected�free,�including�the�first�branches�in the hilum of the lung, to allow mobility of the vessels.

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Clip3

Intracardiac anatomy is evaluated.

Clip4

Transection of the muscle bar.

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Clip5

Patch closure of ventricular septal defect and de-tachment of straddling papillary muscle of tricus-pid valve.

Clip6

Reattachment of the papillary muscle of tricuspid valve.

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Clip7

Transection of the aorta and harvesting of the but-ton�of�the�circumflex�artery.

Clip8

Harvesting of the left anterior descending artery and the right coronary artery.

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Clip9

Transection of pulmonary artery and Lecompte maneuver.

Clip10

Commissurotomy of pulmonary valve (neoaortic valve).

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Clip11

Implantation�of�the�circumflex�coronary�artery.

Clip12

Implantation of the button with the right and left anterior descending coronary arteries.

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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.

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Clip15

Postoperative echocardiogram.

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Complete Transposition of the Great Arteries 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 anat-omy 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�opera-tion 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�stent-ing�of�the�patent�ductus�arteriosus)�before�the�final�operation.�However,�a�prop-erly chosen treatment plan has the potential to minimize the number of pallia-tions 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 tech-nique�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�innomi-nate artery. Both vena cavae are cannulated. The use of an angled cannula and

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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 SurgeryCorrection of transposition of the great arteries is provided by an intraventricu-lar 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 subaor-tic 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 intra-ventricular tunnel.

Extensive dissection and mobilization of the pulmonary arteries is needed to�obtain�a�tension-free�right�ventricular�outflow�tract�reconstruction.�The�pul-monary 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�ob-struction 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 pul-monary 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.

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371 Complete Transposition of the Great Arteries

Patient Characteristics

Age at operation: 7 monthsDiagnosis: 1. Transposition of the great arteries with left

ventricular�outflow�tract�obstruction2. Type 1 conal ventricular septal defect 3. Valvar and subvalvar pulmonary stenosis 4. Atrial septal defect5. Patent ductus arteriosusHistory:1. At the age of 10 days, stenting of the ductus

arteriosus

2. At the age of 7 months, semi-elective surgery due to progressive cyanosis

Procedure:1. REV operation2. Direct connection of the pulmonary artery

with a right ventriculotomy and patch enlarge-ment�of�the�right�ventricular�outflow�tract�and�the left pulmonary artery

3. Direct closure of the atrial septal defect4. Stent removal

Specific Steps of Operation

Clip1

Preoperative echocardiogram and angiogram.

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Clip2

External anatomy of the heart.

Clip3

Right ventriculotomy.

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Clip4

Intracardiac anatomy.

Clip5

Creation of the intraventricular tunnel.

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Clip6

Transection of the great arteries.

Clip7

Closure of the pulmonary valve and the Lecompte maneuver.

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411 Complete Transposition of the Great Arteries

Clip8

End-to-end anastomosis of the ascending aorta.

Clip9

Direct connection of the pulmonary artery with the ventriculotomy and stent removal.

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Clip10

Pericardial patch plasty of the right ventricular outflow�tract�and�the�left�pulmonary�artery.

Clip11

Final outcome.

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431 Complete Transposition of the Great Arteries

Bex–Nikaidoh Procedure

The Goal of SurgeryThe Bex–Nikaidoh procedure creates better alignment of the right and left ven-tricular�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 ven-tricular 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 coro-nary ischemia when the position of the great vessels is not optimal.

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Patient Characteristics

Age at operation: 8 weeksDiagnosis: 1. Transposition of the great arteries

(1 AD, Cx; 2R)2. Inlet type of ventricular septal defect3. Valvar and subvalvar pulmonary stenosis 4. Secundum atrial septal defectHistory:1. At the age of 10 days, balloon atrial septec-

tomy

2. At the age of 8 weeks, semi-elective surgery due to progressive cyanosis

Procedure:1. Bex–Nikaidoh operation2. Direct connection of the pulmonary artery

with the right ventriculotomy and patch enlargement�of�the�right�ventricular�outflow�tract

3. Direct closure of the atrial septal defect

Specific Steps of Operation

Clip1

Preoperative echocardiogram.

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451 Complete Transposition of the Great Arteries

Clip2

Mobilization of the great vessels.

Clip3

Being on pump, the proximal aspects of the coro-nary arteries are extensively mobilized.

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Clip4

Harvesting the aortic root.

Clip5

Transection of both great vessels and transection of the outlet septum.

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Clip6

Excision of pulmonary valve.

Clip7

Posterior translocation of the aortic root.

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Clip8

Reconstruction of the aorta.

Clip9

Patch closure of the ventricular septal defect.

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491 Complete Transposition of the Great Arteries

Clip10

Downsizing�of�the�right�ventricular�outflow�tract.

Clip11

During rewarming, an anterior autologous pericar-dial patch is utilized to augment the main pul-monary artery and complete the right ventricle to pulmonary artery connection.

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Clip12

Final outcome.

fullversion

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Recommended ReadingBautista-Hernandez V, Marx GR, Bacha EA et al (2007) Aor-tic root translocation plus arterial switch for transposition of the�great�arteries�with�left�ventricular�outflow�tract�obstruction:�intermediate-term results. J Am Coll Cardiol 49:485–490

Bex JP, Lecompte Y, Baillot F et al (1980) Anatomical correction of transposition of the great arteries. Ann Thorac Surg 29:86–88

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 out-flow�tract�reconstruction:�Rastelli,�REV,�or�Nikaidoh�procedure?�J Thorac Cardiovasc Surg 135:331–338

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 256–278

Kreutzer�C,�De�Vive�J,�Oppido�G�et�al�(2000)�Twenty-five–year�experience with Rastelli repair for transposition of the great ar-teries. J Thorac Cardiovasc Surg 120:211–223

Lecompte Y, Vouhé P (2003) Réparation à l’Etage Ventriculaire (REV procedure): not a Rastelli procedure without conduit. Oper Tech Thorac Cardiovasc Surg 8:150–159

Losay J, Touchot A, Serraf A et al (2001) Late outcome after arterial switch operation for transposition of the great arteries. Circulation 104(Suppl1):121–126

Morell VO, Wearden PD (2008) Nikaidoh operation for trans-position of the great arteries with a ventricular septal defect and pulmonary stenosis. MMCTS. doi:10.1510/mmcts.2006.002337

Morell VO, Wearden PD (2008) Technique of aortic transloca-tion for the management of transposition of the great arteries with a ventricular septal defect and pulmonary stenosis. Oper Tech Thorac Cardiovasc Surg 13:181–187

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 Saun-ders, Philadelphia, pp 2153 –2163

Nikaidoh H (1984) Aortic translocation and biventricular out-flow�tract�reconstruction:�a�new�surgical�repair�for�transposition�of the great arteries associated with ventricular septal defect and pulmonary stenosis. J Thoracic Cardiovasc Surg 88:365–372

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 19:283–289

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 cardiol-ogy, 3rd edn. Churchill-Livingstone, Philadelphia, pp, 795–817

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 20:816–823

Weyand K, Haun C, Blaschczok H et al (2010) Surgical treat-ment of transposition of the great arteries with ventricular septal defect� and� left� ventricular� outflow� tract� obstruction:�midterm-results. World J Pediatr Congenital Heart Surg 2:163–169