D TGA

transposition of great arteries

1. D–Transposition

2. D -TRANSPOSITION Atrioventricular concordance with ventriculo -arterial discordance aorta arises from a morphological RV, and the PA arises from a morphological LV Abnormal spatial relationship of the great arteries two circulations in parallel

3. dextroposition of the bulboventricular loop ( ie , the position of the RV, which is on the right side). aorta =right and anterior great arteries are parallel rather than crossing

4. Basic Embryological defect Abnormal development, growth, and absorption of the distal infundibulum (conus) normal conus is subpulmonary , left sided, and anterior= prevents fibrous continuity between the pulmonary and tricuspid valve rings infundibulum is usually subaortic , right sided, and anterior= prevents fibrous continuity between the aortic and tricuspid valve rings

5. Embryology 1 . Spiral aortico -pulmonary septum forms but does not spiral or twist during its partitioning of the truncus arteriosus a. Aorta arises from right ventricle b. Pulmonary trunk arises from the left ventricle 2. a . Systemic – unoxygenated – repeatedly re-circulated b. Pulmonary - oxygenated - repeatedly re-circulated

6. 4 truncus and 2 conal cushions develop. Dextro - sinistro cushions of both conus and truncus fuse to form Conotruncal septum. Intercalated cushions play an role in formation of semi lunar valves Embryology - Septation of conus and truncus .

7. Embryology - Septation of conus and truncus. Because the cushions are dextro -superior and sinistro inferior in truncus and dextro -dorsal and sinistro -ventral in conus union forms a spiral septum than true lineal relation.

8. Aorta will be in connection with RV and PA with LV. There are two rotations one at conoventricular junction and other at Conotruncal junction. Both rotations are counterclockwise around 110º Embryology - Rotation and absorption.

9. Conoventricular rotation brings aorta in continuation with LV and PA with RV. Conotruncal rotation brings the normal position of aorta in relation to PA ( left and posterior to PA) Embryology - Rotation and absorption.

10. selective Resorption of conal septum coni which gets selectively resorbed during development that respective artery is drawn over LV .

11. Absence of both conoventricular and Conotruncal rotation Persistence of sub aortic and complete resorption of sub pulmonic coni

12. abnormal growth and development of the SA infundibulum and the absence of growth of the SP infundibulum. AV is protruded superiorly and anteriorly by the development of the SA infundibulum, placing it above the anterior RV Failure of development of the SP infundibulum prevents the normal morphogenetic movement of the PV from posterior to anterior and further results in abnormal PV to MV ring fibrous continuity .

13. Jatene =TGA and VSD. LECOMPTE = direct anastomosis of both great arteries without interposition of a tube when the pulmonary bifurcation is transferred in front of the distal ascending AA.

14. Epidemiology 20.1 to 30.5 per 100,000 live births 60 % to 70 %= male 5% to 7% of all CHD males than in females =3:1

15. Pathophysiology

16. physiologic L-to-R shunt = volume of the PV blood recirculating through the lungs without having passed through the body physiologic R-to-L shunt -volume of SV blood reentering the systemic circulation without having passed through the lungs.

17. The net volume of blood passing from the pulmonary circulation (LA, LV, PA) to the systemic circulation (RA, RA, aorta) represents the anatomic L-to-R shunt The effective systemic blood flow ( i.E. , Oxygenated PV return perfusing the systemic capillary bed)

18. The net volume of blood passing from the systemic circulation to the pulmonary circulation represents the anatomic R-to-L shunt The effective PBF (SV return perfusing the pulmonary capillary bed).

19. deficient oxygen supply to the tissues excessive right and LV workload . incompatible unless mixing at some anatomic level.

20. Anatomy TGA {S,D,D} – situs solitus (S) ( D) looping of the ventricles anterior and rightward (D) aorta

21. Great artery relationship aortic root - anterior or anterior and to the right of the pulmonary trunk in a slightly oblique relationship (S,D,D) Less commonly- aorta may be positioned anterior and to the left (S,D,L). rare - aorta is posterior

22. Atria normal internal anatomy. Right atria is larger , particularly when IVS is intact . Almost always=PFO 5% - true secundum ASD sinus and AV nodes =usual locations.

23. Right Ventricle hypertrophied , and large 90 % = subaortic conus (infundibulum ) less wedging of the pulmonary trunk between mitral and tricuspid valves atrioventricular (AV) valves may be at virtually the same level 10% of hearts with TGA and IVS= subaortic conus in the RV is absent or very hypoplastic .

24. Left Ventricle infrequently contains an infundibulum ( conus PV-MV fibrous continuity exists RV wall is considerably thicker than normal at birth and increases in thickness with age.

25. IVS is intact and no PS -- LV wall is of normal thickness at birth less than normal thickness within a few weeks of birth thin wall by age 2 to 4 VSD is present = LV wall thickness increases slightly less than in the normal heart but remains well within the normal range during the first year of life LV cavity is the usual ellipsoid in shape at birth but soon becomes banana shaped.

26. Conduction System AV node and bundle of His lie in a normal position, although the AV node is abnormally shaped and may be partly engulfed in the right trigone LBB originates more distally from the bundle of His Damage to the bifurcation of the bundle at VSD closure – CHB

27. Coronary Arteries aortic sinuses that face the pulmonary trunk , regardless of the interrelationships of the great arteries LAD and LCx arise as a single trunk ( LCA ]) from aortic sinus 1 and distribute in a normal manner RCA = sinus 2 and follows this artery's usual course . single coronary artery= sinus 2 .

28. Usual =single ostium in the center of the sinus may arise from a double-barreled ostium consisting of two ostia immediately adjacent to each other and constituting essentially a single ostium At times, the LCA or LAD passes forward between aorta and pulmonary trunk in an intramural course to emerge anteriorly .

29. conus artery frequently arises separately and from its own ostium in sinus 1. ==considerable part of the anterior wall of the infundibulum of the RV. sinus node artery = atrial switch (Mustard or Senning ) = arises from the RCA close to its origin and passes superiorly and rightward, usually partly embedded in the most superior portion of the limbus of the atrial septum, where it can be damaged if this portion of the atrial septum is widely excised

30. Coexisting Anomalies 50 % =no other anomaly except a PFO or a PDA. VSD =- 40% to 45%. - perimembranous ( conoventricular 33%) - AV canal (inlet septum 5%) - muscular (27%) - malalignment (30%) - conal septal hypoplasia type (5%)

31. Ventricular Septal Defect Conoventricular defects of the several different varieties some hearts with conoventricular VSDs=outlet ( conal , infundibular) septum is malaligned and fails to insert within the Y of the septal band septum may be displaced leftward= LVOTO Rightward=n RV ( subaortic ) obstruction.

32. conal septum is displaced to the right= pulmonary trunk may be biventricular in origin and over a juxtapulmonary VSD and may be associated with subaortic stenosis or aortic arch obstruction (arch hypoplasia, coarctation , or interruption). Occasionally the VSD is juxta -aortic and associated with a malaligned but nondisplaced conal septum . The conal septum may be absent and the VSD is then juxta -arterial (doubly committed).

33. LV Outflow Tract Obstruction subpulmonary obstruction dynamic or anatomic 0.7 % of patients with TGA and intact ventricular septum 20 % of patients born with TGA and VSD apparent or develop after birth in other patients, overall prevalence of 30 % to 35%. Dynamic type of LVOTO, developing in patients with TGA and intact ventricular septum= leftward bulging of the muscular ventricular septum secondary to higher RV than LV pressure.

34. Subpulmonary Stenosis or LVOTO Fixed - Circumferrential fibrous membrane /diaphragm - Fibromuscular ridge - Herniating tricuspid leaflet tissue - Anomalous MV septal attachments - Tissue tags from membranous septum Dynamic-associated with SAM

35. Subaortic Obstruction Rightward and anterior displacement of the infundibular septum - hypoplasia - coarctation - interruption Asso . RV hypoplasia & tricuspid valve anomalies

36. Aortic Obstruction discrete ( coarctation , or less often interrupted aortic arch) distal arch hypoplasia. 7 % to 10% of TGA and VSD. more frequent when the VSD is juxtapulmonary and the pulmonary trunk is partly over the RV in association with rightward and anterior displacement of the infundibular septum and with some subaortic narrowing. associated coarctation = underdevelopment of the RV sinus is more common.

37. Patent Ductus Arteriosus PDA-more common Persistence of a large PDA for more than a few months = increased prevalence of pulmonary vascular disease.

38. PDA is present at age 1 week =half thereafter the prevalence falls rapidly . When patent, the ductus is small (less than 3 mm in diameter) in two thirds of patients =little influence on natural history . Large = LV output is increased and hypoxia lessens =heart failure acute and often early closure of the ductus --sudden increase in hypoxia and clinical deterioration = decreased mixing at ductus level but also at atrial level because of the fall in left atrial pressure that results from decreased pulmonary venous return.

39. TV anomalies 31 % Functionally imp 4% Ratio of tricuspid to mitral anulus circumference is less than 1 in 50% normal hearts this ratio -greater than 1

40. TV anomalies Straddling/overriding of chordae Overriding of the tricuspid annulus Abnormal chordal atatchments Dysplasia Accessory tissue Double orifice

41. MV anomalies 20% Functionally imp 4% Cleft anterior mitral valve leaflet anomalous papillary muscles and chordae Straddling redundant tissue tags

42. Juxtaposition of atrial appendages Both appendages or left + part of right are adjacent 2-6% dextrocardia , VSD, bilateral infundibulum, right ventricular hypoplasia and tricuspid stenosis or atresia. Imp in BAS

43. Right Aortic Arch 5 % more common when there is an associated VSD than when the IVS associated leftward juxtaposition of the atrial appendages.

44. Bronchopulmonary Collateral Circulation > 30% of infants with TGA under 2 years of age functionally and freely communicate with the pulmonary vascular bed proximal to the pulmonary capillary bed Potential intercirculatory (systemic-to-pulmonary) mixing pathway, accelerated and more widespread PVD Persistence of a significant BPC circulation after surgical repair - large enough left-to-right shunt – CCF - warrant catheter embolization

45. contribution to PBF from the BPC circulation enters the pulmonary vascular circuit distal to the usual catheter sampling sites true mixed PA saturation present at the precapillary level cannot be sampled falsely high PA oxygen saturation and blood flow calculation will result A modest BPC Circulatation (20%) to the pulmonary precapillary blood flow can result in 30% overestimation of PBF.

46. Fetal circulation compatible with normal fetal survival and relatively normal gestational development. course of fetal circulation is modified right side of the heart ejects blood directly into the ascending aorta RV ejects into the descending aorta via the PDA in normal cardiac and CNS structures similar in size and weight to control values increased numbers and size of pancreatic islet cells increased weight of the adrenal cortex

47. Neonatal transition After birth, the PVR falls PBF and LA pressures increase more or less normal neonatal transitional physiology SVR increases because of removal of the low-resistance placental circulation With TGA= RA pressures are increased( contrast to normal) and the similarity of atrial pressures tends to keep the PFO open (incompetent valve), with resulting bidirectional shunting TGA with IVS- ductus arteriosus is often widely patent after birth . Early after birth, when PVR is still high, there is bidirectional ductal flow : systole , LV–PA–ductus–-descending aorta diastole , aorta–ductus–PA.

48. Natural history 1 st week-30% 1 st month-50% 1 st year-90% Depends on the degree of shunting Moderate PS improves survival

49. Survival all varieties 55 % survive 1 month 15% survive 6 months 10 % survive 1 year . Mean life expectancy = 0.65 year 4 years for those who survive to 12 months 6 years for the few who survive for 18 months Thereafter , life expectancy declines rapidly.

50. ] TGA and essentially intact ventricular septum 80% at 1 week only 17% at 2 months 4 % at 1 year better when there is a true ASD. TGA and important VSD 91 % at 1 month 43% at 5 months 32 % at 1 year lower when the patient has a very large Qp .

51. large VSD and aortic obstruction ( coarctation , interrupted arch) Lethal all patients die within a few months with severe heart failure . In patients with TGA, VSD, and LVOTO, early survival is still better, reaching 70% at 1 year and 29% at 5 years, because in many LVOTO is only moderate initially. Leibman and colleagues found that PDA increased risk of early death in all subsets of patients. This is particularly the case when the ductus is large.

52. CLINICAL COURSE IN COMPLETE TGA CYANOSIS, HYPOXEMIC DETERIORATION, HEART FAILURE WITH EARLY DEATH Inter circulatory mixing

53. CLINICAL FEATURES Symptoms and clinical presentation - degree of mixing high degree of mixing and large PBF- Qp , SaO2 may be near normal, and unless there is pulmonary venous hypertension, symptoms are minimal . When mixing is minimal= SaO2 is low and symptoms of hypoxia are severe Adequate mixing =communications of reasonable size at atrial, ventricular, or great artery levels Factors that reduce Qp , such as LVOTO and increased PVR= reduce mixing and increase cyanosis.

54. Reverse differential cyanosis TGA with a PDA and PA-to-aorta shunting complex TGA malformation including an aortic arch anomaly, such as COA or IAA . TGA with suprasystemic PVR.

55. CLINICAL SPECTRUM TGA (IVS or small VSD) with increased PBF and small ICS TGA (VSD large) with increased PBF and large ICS TGA (VSD and LVOTO), with restricted PBF TGA (VSD and PVOD), with restricted PBF

56. Essentially TGA IVS (Poor Mixing) infants with out a VSD or with a VSD 3 mm or less in diameter. PFO or naturally occurring ASD + Cyanosis - half these infants within the first hour of life 90 % within the first day and is rapidly progressive. critically ill with tachypnea and tachycardia and dies from hypoxia and acidosis without appearance of frank heart failure.

57. rapid downhill course is usually obviated with a naturally occurring ASD of adequate size because cyanosis is less severe. surviving infants, appearance of moderate or severe dynamic LVOTO -- increasing cyanosis and hypoxic spells, even after an adequate atrial septostomy patients are of average birth weight and in good general condition although with severe cyanosis Clubbing of fingers and toes is absent and generally does not appear unless the infant survives to about age 6 months There is mild increase in heart and respiratory rates

58. The heart is not hyperactive, and the liver is barely palpable faint mid- systolic ejection-type murmur is present along the midleft sternal edge in less than half these infants more prominent with organic or dynamic LVOTO, first appearing at age 1 or 2 months with the dynamic form and then gradually increasing in intensity . The second heart sound is unremarkable (often apparently single or narrowly split).

59. ECG often normal at birth. By the end of the first week -- persistence of an upright T wave in right precordial leads indicates abnormal RV hypertroph y, and right-axis deviation predominates. When important LVOTO is present or PVR elevated, ECG evidence indicates biventricular hypertrophy .

60. CXR • An oval- or egg-shaped cardiac silhouette with a narrow superior mediastinum • Mild cardiac enlargement • Moderate pulmonary plethora first week of life-CXR may be normal, or occasionally cardiac enlargement may be more marked. narrow mediastinum - great artery positions and by shrinkage of the thymus, usually associated with stress, and the plethora is caused by the increase in Qp . Plethora is less marked -LVOTO.

61. CXR TGA/IVS (a) oval or egg-shaped cardiac silhouette with narrow superior mediastinum (b) mild cardiomegaly (c) increased pulmonary vascular markings

62. D TGA CHEST X RAY IN DIAGNOSIS OF CARDIAC CONDITIONS

63. Large VSD, Large PDA, or Both (Good Mixing ) Presentation -latter half of the first month mild cyanosis signs of HF -PVH and myocardial failure Tachycardia , tachypnea, important liver enlargement, and moist lung bases heart is more active larger than in the poor-mixing group large VSD =moderate-intensity pansystolic murmur along the lower left sternal edge that may not be present initially.

64. apical middiastolic murmur or gallop rhythm narrow splitting accentuation of p2 large PDA-continuous murmur, bounding pulses, and an apical middiastolic murmur are =less than half the patients, even when the ventricular septum is intact.

65. more cardiomegaly more plethora wider superior mediastinum than in the poor-mixing group. ECG =BVH persistent large VSD=Q wave in V6. Isolated LV hypertrophy =RV hypoplasia with tricuspid valve overriding Development of PVD = reduction in Qp and less plethora, particularly in the peripheral lung fields, as well as reduced heart size, but these features generally appear after the neonatal period.

66. coarctation with VSD and PDA= femoral pulses are usually normal because the coarctation is preductal and ductus arteriosus large . Rarely, differential cyanosis can occur, with cyanosis confined to the upper torso.

67. Large VSD and LVOTO (Poor Mixing without High PBF) least common of the three TGA groups decreased Qp and poor mixing PVH and associated symptoms and signs do not develop Heart failure is -- not present. cyanosis is severe from birth.

68. heart is not overactive pulmonary ejection murmur single S2 apical gallop or middiastolic murmur near normal-sized heart with normal or ischemic lung fields . ECG- biventricular hypertrophy.

69. TGA VSD Pulmonary Vascular Disease simple TGA= PVOD rarely develops in the first few months of life After 6 to 24 months=prevalence increases to 10% to 30%. TGA and moderate or large VSD= PVD develops more rapidly , as it does in those with persistently large PDA Among those dying at about age 6 months, 25% have developed severe pulmonary vascular disease (grade 3 or greater), and 50% of infants dying by age 12 months have developed it.

70. Echocardiography dynamic LVOTO leftward deviation of the VS abnormal fluttering and premature closure of the PV SAM of the mitral leaflet prolonged diastolic apposition of the AML to the septum. coronary arteries=number , origin, major branching pattern, , intramural course.

71. Coronary artery anatomy; Morphologic details of pulmonary or subpulmonary obstruction; VSD number, site, and size; Great vessel alignments in relation to the VSD and outlet septum; Type and severity of aortic arch abnormalities Inter atrial communication

72. The RAA lies posterior and to the left of the GA and anterior to the LA. The posterior portion of the atrial septum (a) is oriented normally; the anterior portion (b) is oriented transversely and parallel to the anterior chest wall. T he atrial septum curves toward the right. The LA and RPV wrap around the posterosuperior aspect of the RA

73. Cardiac Catheterization and Cineangiography SBF and PBF and pressures, including those across the LVOT. Using appropriate views, cineangiography demonstrates the cardiac connections and great artery positions position and number of VSDs site of any LVOTO the size and function of AV valves, size and function of both ventricles, and presence of other cardiac anomalies.

74. Indications of cardiac catheterization hemodynamically unstable and rquire BAS. physiologic and anatomic data is required concerning coronary artery, the VSD, degree of LVOTO. complex cardiac anomalies like CoA, interrupted aortic arch. pulmonary vascular resistance in TGA with VSD

75. oxygen saturation in the pulmonary artery is always higher than in the aorta .

76. TV - selective RV injection in the frontal or RAO views by noting intra-atrial bulging of the leaflets during ventricular systole and during diastole by the negative silhouette of the orifice as nonopacified blood enters the ventricle Continuity of the anterior leaflet of the MV with the PV = four-chamber long-axial or LAO views in diastole when the anterior leaflet is noted to form the posterior wall of the LVOT.

77. Catheterization PAP PBF(and PVR ); ( overestimate PBF and underestimate PVR) Coronary artery anatomy; Morphologic details of pulmonary or subpulmonary obstruction; VSD number, site, and size; Great vessel alignments in relation to the VSD and outlet septum; Type and severity of aortic arch abnormalities

78. Coronary artery anatomy Selective transvenous CAG or antegrade aortic root angiography with distal balloon occlusion of the ascending aorta. balloon angiographic catheter introduced transvenously , is positioned in the ascending aorta balloon is inflated with CO2 and stabilized in the ascending aorta. 0.5 and 1.0 mL/kg of contrast medium are injected over 1 second balloon is deflated immediately after the injection.

79. Balloon occlusion aortography with extreme caudal angulation of the anterior-posterior camera, - laid-back aortogram . visualization of both the proximal ostia and distal distribution of the coronary arteries in the same plane of view

80. Medical management hemodynamic stabilization correction of physiological aberrations caused by cyanosis and poor perfusion . Correction of acid base balance, maintainance of normothermia , prevention of hypoglycemia.

81. Management Airway protection, Oxygen and ventilatory care Acid-base correction, PGE 1 infusion, 0.01-0.02 microg /kg infusion Apnea , fever , hypotension Antibiotics,

82. PGE1 infusion to maintain patencty of ductus BAS

83. Palliative procedures D-TGA/IVS –poor I.C.M.despite patent PDA- Balloon atrial septostomy, surgical septectomy partial venous correction, D-TGA/IVS/ICM depends on PDA-but it is closing PGE1 infusion. D-TGA/IVS/LVOTO- systemic-to-pulmonary shunts. D-TGA/VSD(multiple) - pulmonary artery banding,

84. Balloon Atrial Septostomy profound hypoxemia when corrective surgery must be delayed . catheter should be advanced across the foramen ovale into the LA or a PV and the position of the tip established with certainty in the LA prior to proceeding posterior position of the tip in the lateral or LAO view or entry of the catheter into a PV

85. balloon is inflated with diluted angiographic contrast medium to 12 to 15 mm diameter rapidly withdrawn across the atrial septum with an abrupt, short tug balloon and interatrial septum are displaced toward the inferior vena cava, and the septum primum flap of the fossa ovalis is ruptured as the balloon is carried in a single movement from the left atrium to the right atrial-inferior vena caval junction .

86. The catheter should be advanced immediately and the balloon pushed cephalad out of the IVC orifice into the RA toward the SVC to verify crossing the septum and to avoid obstruction to IVC return while the balloon is being deflated. This same procedure should be repeated several times with increasing balloon volumes so that withdrawal of the balloon, inflated tensely to a diameter of 15 mm, is achieved without much resistance being perceived at the atrial septum level.

87. COMPLICATIONS Atrial wall, Pulmonary vein, inferior vena caval perforation or tears or AV valve damage. Intracardiac rupture of the balloon. Air embolism CNS complications

88. Difficulties older infants, particularly those with TGA/VSD=thickened interatrial flap =catheter equipped with an extendable blade markedly thick atrial septum, a new defect (separate from the foramen ovale ) - transseptal needle and dilated with an 8- to 15-mm-diameter balloon angioplasty catheter ( Brockenbrough angioplasty).

89. Surgical Creation of ASD Blalock & Hanlon operation or one of its modifications – historical footnote excision of the posterior aspect of the IAS . oxygen saturation levels modestly higher than BAS mortality risks <3% to 5%.

90. Pulmonary Artery Banding large VSD without LVOTO To prevent Heart failure Pulmonary vascular disease Present Indications complex/multiple VSDs Coexisting medical conditions that cause a delay in surgery To train LV before switch in TGA/IVS

91. Partial Venous Return Repair ( Baffes ) connecting the IVC to the LA with a homograft or synthetic conduit and concurrently detaching the right pulmonary veins and directly transferring them to the RA. This results in an obligate, effective shunt at the atrial level. Subsequently, some of these patients had a modified Mustard type of atrial repair to achieve complete physiologic correction.

92. Aims of surgery 1.To make the parallel circulations into series. So that oxygenated blood goes to aorta and deoxygenated blood goes to pulmonary trunk. 2.Correction of other cardiac anomalies like VSD, PDA, TR, AORTIC OBSTRUCTION, LVOTO. 3.To provide a near normal functional status to patients.

93. Definitive Repair The atrial level : Senning or Mustard sx Great artery level : Arterial switch operation or jatene operation

94. Atrial switch Removal of the atrial septum redirection of the SV pathways to LV PV blood to RV Senning operation= rerouting by infolding of the atrial walls Mustard =synthetic or pericardial tissue modifications of the Mustard = trouser-shaped baffle, with the legs anastomosed to the SVC & IVC inflows.

95. early post-operative survival exceeding 95%.

96. PHYSIOLOGIC CORRECTION (ATRIAL SWITCH) atrial switch operations may be d elayed for a few weeks to several months after birth (and balloon atrial septostomy ).

97. A right atriotomy is made in front of and parallel to the caval veins and extended into the atrial appendage. This exposes the atrial septum The atrial septum is now incised anteriorly , superiorly, and inferiorly to form a septal flap as large as possible, which remains fixed posteriorly between the caval entrances. The wall between the coronary sinus (CS) and the left atrium may be incised to provide a large posterior lip for the inferior septal flap suture line and direct coronary sinus return to the newly formed systemic venous return chamber. The systemic venous chamber and conduit to the mitral valve is formed posteriorly by the repositioned septal flap and the conduit then is completed anteriorly by suturing the posterior right atrial free wall flap anteriorly to the anterior septal limbus . A left atriotomy is made as long as possible in the internal atrial groove (exposing the orifices of the right pulmonary veins). The pulmonary venous chamber and pathway to the tricuspid valve are completed with the suturing of the anterior right free wall atrial flap over the right pulmonary veins to the anterior lip of the left atriotomy .

98. The patch should not touch the lateral atrial wall 8/12/2017

99. Final 8/12/2017

100. Results and Sequelae of Physiologic Correction 10-year survival rates of 85% to 90%. ( a ) residual intra-atrial shunts, (b) caval and pulmonary venous obstructions, (c) right ventricular dysfunction, (d) tricuspid valve insufficiency, and (e) arrhythmia.

101. Trivial leaks -10% to 20% of patients, Significant leaks requiring reoperation -(1 to 2%). RV dysfunction to some extent and dysrhythmias =late concerns

102. Progressive loss of NSR and increase in atrial rhythm disturbances Gradual time-related decrease in sinus rhythm sinus rhythm at 1 year was 72%, at 5 years 56%, at 10 years 50%, and at 13 years 43 %

103. Arterial switch operation ( Jatene operation) Advantages Fewer long-term complications Arrhythmias RV dysfunction Baffle stenosis Tricuspid regurgitation (TR). LV as systemic ventricle & MV as Systemic AV valve

104. functional adequacy of the LV ?? Adequate LV muscle mass . Early infancy Nonrestrictive PDA , Surgically remediable or dynamic LVOTO Delayed decrease in PVR and persistent PAH A large, nonrestrictive VSD . Definitive early (neonatal) one-stage arterial repair >> early palliation with PA banding and later arterial switch surgery.

105. Pre requisite LV pressure should be near systemic levels switch should be performed shortly after birth (i.e., before 2 weeks of age). LV pressure is low= PA banding, either with or without a shunt, for 7 to 10 days ( rapid , two-stage switch operation) or for 5 to 9 months

106. absolute LV systolic pressure that is appropriate for age, A LV pressure at cardiac catheterization = > 70% systemic levels (left to RV ratio >0.7), or LV muscle mass that is within the normal range for BSA EMPIRICAL CRITERIA FOR LV SIZE

107. Pre-op Coronary artery pattern amenable to transfer to the neoaorta without distortion or kinking. Risk is high when the left main or LAD coronary artery passes anteriorly between the aorta and the PA. LV inflow and outflow tracts must be free of significant structural abnormality RVOT should be free of significant stenosis.

108. Anatomic variants that may impact operative mortality include intramural course of a coronary artery retropulmonary course of the left coronary artery Multiple VSDs Coexisting abnormalities of the aortic Straddling AV valves Longer duration of global myocardial ischemic (cross-clamp) prolonged circulatory arrest times

109. great arteries are transected reanastomosis of the distal aortic segment to the proximal pulmonary artery ( neo aortic root ). Transfer of the coronary arteries to this pulmonary segment =excision from the aortic sinus with a cuff of adjacent aortic wall proximal aortic segment ( neopulmonary root) connected to the distal PA segment Maneuver of lecompte - passes the anterior aorta posterior to the bifurcation of the pulmonary artery

110. aorta is transected , pulmonary trunk is transected just proximal to its bifurcation aortic button around the orifice of the left main coronary artery is excised from its sinus , and this is inserted into the left facing sinus of the neoaorta ( originally,pulmonary trunk).

111. Arterial switch

112. Complications PA stenosis at the site of reconstruction - 5 % to 10% CHB- 5% to 10%. AR late complication > 20% of patients especially PA banding unequal size of the pulmonary cusps that leads to eccentric coaptation Coronary artery obstruction myocardial ischemia, infarction, and even death.

113. TGA with Low LV Pressure PA banding .. Long preparation period. Rapid two stage switch. LV function may be extremely impaired following banding .( systemic-to-PA shunt is frequently placed to ensure adequate PBF ) interval period between banding and correction = low output syndrome. Clinical improvement coincides with improvement in LV function such that anatomic correction can be performed within 7 to 10 days in most cases.

114. OTHER INNOVATIVE APPROACHES Percutaneously adjustable band Partial balloon occlusion of the MPA with a percutaneously placed balloon-tipped cathete r Systemic-to-PA shunting alone Primary arterial switch with LV assist in the perioperative period.

115. Anatomic Correction without Coronary Translocation DAMUS , KAYE, AND STANSEL arterial level repair without coronary translocation. children with TGA and coronary artery patterns not suitable for transfer patients with DORV ( Taussig bing type) with severe subaortic stenosis.

116. MPA is transected and anastomosed to the ascending aorta. coronary arteries are perfused in a retrograde fashion native aortic valve may be left intact VSD (if present) is closed to direct lv blood to the native pulmonary ( neoaortic ) valve RV to PA conduit is placed to establish a normal series circulation

117. Innovative Techniques for anatomic correction with out Coronary Translocation Creation of aortopulmonary tunnel ( aubert procedure) Baffling the LV outflow to the nontranslocated coronary ostia with a patch of native aorta or pericardium. The entire aortic root may be translocated to the left ventricle with biventricular outflow tract reconstruction.

118. Transposition of the Great Arteries (VSD and LVOTO) with Restricted Pulmonary Blood Flow Neonates with TGA, VSD, and severe PS or atresia have diminished PBF. They represent a relatively small proportion (5% to 8%) of the neonatal TGA population. Clinical findings are similar to those in the infant with TOF with severe PS or atresia , and the cyanosis is extreme from birth

119. Surgery for TGA with Associated LVOTO In some neonates, a palliative systemic-to-PA shunt (Gore-Tex interposition shunt or classic BT shunt) may be performed, with intracardiac correction carried out at a later age. Alternatively, corrective surgery can be performed in early infancy

120. RASTELLI OPERATION intraventricular repair ++ extracardiac RV to PA conduit. TGA with large VSD and extensive LVOTO - complete bypass of the LVOTO and an anatomic correction of the transposition pathology operative survival =95 % midterm survival =90 %

121. complications Unfavorable anatomic variants, -- restrictive VSD anomalous TV connections to the infundibular septum that prevent baffling the LV to the anterior aorta. Residual VSD, Late unexpected death, myocardial dysfunction . ??Functional longevity of the valved conduits. Improved results are noticeable with fresh or cryopreserved homograft- valved conduits compared with the previously used dacron heterograft structures

122. Complications conduit obstruction (especially in those containing porcine heterograft valves) complete heart block (rarely occurs). This conduit needs to be replaced as the child grows.

123. REV Applicable to younger patients , Avoidence of Prosthetic extrcardiac conduit, Avoidence of intracardiac Tunnel Obstruction. REV approach allows Complete repair earlier in infancy, Is feasible in patients with anatomic contraindications to the rastelli operation, reduce the need for reoperation and the prevalence of residual pulmonary outflow tract obstruction The lifelong implications of pulmonary regurgitation following this newer operative approach require continued investigation

124. This operation involves Performing a high, anterior RV incision Radical excision of the outlet septum to create an unobstructed anterior RV cavity ; Establishing a short and direct intraventricular tunnel from the LV to the aorta Closure of the pulmonary artery orifice ; Reimplantation of the transected (and usually anteriorly translocated ) PA directly onto the RV outflow cavity without a prosthetic conduit

125. Surgery for TGA/VSD and PVOD PVR>10 U or grade 4 (H-E) histologic changes is a CI to VSD closure.

126. SURGICAL OPTIONS DTGA Anatomy Surgical options Comments TGA/IVS Physiologic repair    Senning or Mustard Usually elective, neonatal-1 yr Anatomic repair (primary)    Arterial switch ( Jatene ) Neonatal period, usually within 2 wk of age TGA/IVS with prolonged low LV pressure Physiologic repair     Senning or Mustard Usually elective, 1 mo to “1 yr Anatomic repair (delayed)    Two-stage arterial switch Long preparation period ( Yacoub ) Rapid two-stage switch (Jonas) TGA/VSD Physiologic repair     Senning or mustard with VSD closure Poor long-term results Anatomic repair    Arterial switch with VSD closure Usually neonatal repair; PAB occasionally (multiple VSDs) Interventricular baffle repair Not all VSDs suitable Damus -“Kaye-“ Stansel : VSD closure ( LVto’PA ); proximal PA to Ao anastomosis ; RV to distal PA conduit Used when coronary translocation impossible aortic valve closure

127. TGA/VSD/PS VSD closure ( LV to Ao ), RV to PA conduit ( Rastelli ) Palliative systemic-to-pulmonary shunt frequently performed Conduit replacement frequently necessary VSD closure ( LV to Ao ), anterior translocation of PA with direct connection to RV: REV procedure ( Lecompte ) Long-term pulmonary regurgitation TGA/PVOD Physiologic repair, palliative Anatomic repair, palliative Symptomatic improvement