MANAGEMENT OF CC TGA

1. Management of cc - TGA

2. Clinical features
• Depends on associated anomalies
• Vsd – 80%
– Only in 20% increased pulmonary blood flow (PBF)
– With some degree of ps
– Variable presentation
– Rarerly in infancy – cyanosis
– Chilhood or 2nd decade
– 3,4,5 th decades with rv (systemic ventricular) failure

3. • Clinical features
• Complete heart block is the most common
arrhythmia in patients with L-TGA with signs and
symptoms of bradycardia, fatigue, and poor exercise
tolerance.
• Progressive fibrosis of conduction system with
advancing age, which increases the risk of complete
heart block (progressive incidence of 2 percent per
year ) and re-entrant tachyarrhythmias including
Wolff-Parkinson-White (WPW) syndrome.

4. • Unoperated Natural History
• Early natural history is significantly affected by the severity of
associated lesions and surgical management.
• Although there have been repeated case reports of long-term
survival with ccTGA, this is likely unusual .
• Beauchesne et al. followed 44 unoperated patients for 144
months and found that most (59%) had grade 3 or greater
systemic atrioventricular valve regurgitation and that many of
these demonstrated significant systemic RV dysfunction and
were symptomatic.

5. • Presbitero et al. have followed 18 patients, again pointing to
systemic atrioventricular valve regurgitation and ventricular
dysfunction as major concerns.
• Graham et al. In a large multi-institutional study, found that,
patients without associated lesions had a lower occurrence
rate of heart failure and systemic ventricular dysfunction than
those with associated lesions at a given age, these problems
tended to increase in frequency with advancing age in both
groups.

7. • Electrocardiography —
• In L-TGA, the interventricular septum is depolarized in the
opposite direction of normal.
• Q waves in the right precordial leads and an absence of Q
waves in the left-sided precordial leads
• These electrocardiographic findings may be misinterpreted as
an inferior myocardial infarction
• In addition, patients may also have varying degrees of AV
heart block due to abnormalities of the conduction system.
• As noted above, the risk of CHB rises over time with a 2
percent per year increase in incidence

9. • Chest radiograph —
• 25 % of patients with L-TGA will have mesocardia or
dextrocardia
• In those patients with levocardia (normal location),
the leftward positioned aorta usually results in a
prominence in the upper left border of the
mediastinum

10. congenitally corrected transposition of the great arteries, a nonrestrictive
ventricular septal defect, and increased pulmonary blood flow.
A septal notch (unmarked arrow, lower right) appears just above the left
hemidiaphragm.
The ascending aorta (AAo) is convex at the left base,
the dilated posterior pulmonary trunk causes rightward displacement of
the superior vena cava (SVC).

11. echocardiography
• Atrial situs solitus
• Atrio ventricular discordance
• Lt sided av - valve more towards apex
• Direct chordal attachments to septum
• Coarse trabeculated endocardial surface of ventricle
to left side
• Ventriculo arterial discordance
• Rt av - valve and pulmonary valve in continuity
• Presence of associated anomalies

12. Radiological inv
• Cardiac ct
– Anatomy
– Coronary anatomy
• Cardiac mri
– Particularly lv mass, volumes
– Valve regurgitant fractions

13. Angiography
• Profile septum
• Location and no of vsd’s
• Nature of ps
• Tricuspid valve fn
• Measure PVRI
• Shunt fraction
• Ventricular edp.

14. • Medical Management
• Medical management involves the usual modalities
for cardiac failure, such as inhibition of angiotensin-
converting enzymes, diuretics, and control of
arrhythmias with pacing to raise the heart rate when
necessary.

15. Indications for intervention in CC-TGA
The presence of corrected TGA is not an indication for a
reparative operation
WITHOUT ASSOCIATED DEFECTS
Complete heart block
-WITH ASSOCIATED DFECTS
1. Ventricular septal defect
2. VSD & Important PS
3. Left-sided tricuspid incompetence

16. • CHB
• Dual chamber AV sequential pacemeker is indicated in any
symptomatic patient with AV block.

17. • CORRECTIVE SURGICAL MANAGEMENT
• Overview — There has been a paradigm shift from
• conventional repair/Physiological Repair of the associated cardiac lesions
while maintaining the congenitally corrected atrioventricular and
ventriculoarterial discordance to an
• “anatomic” repair, which makes the morphologic left ventricle become
the systemic pump and the morphologic right ventricle the pulmonary
ventricle.

18. • Isolated L-TGA –
• the choice of anatomic repair is controversial.
• complex procedures that require substantial time on bypass .
• In contrast, there are data that show adults with isolated L-TGA without
anatomic repair are at-risk for systemic heart failure, although the risk is
lower than patients described above with associated lesions .
The final decision is individualized based on a review of the patient’s
potential for heart failure without anatomic correction versus the
potential complications of the anatomic repair, and the preference of the
family.

19. • Suggested guide for deciding surgical approach
• Pediatric patients with L-TGA and
– significant ventricular septal defect [VSD],
– left ventricular (LV) outflow tract obstruction, and/or
– Ebstein-like malformation of the tricuspid valve
• should be considered for anatomic repair as they are at greatest risk for
developing systemic heart failure with conventional repair.
•

20. • Patients who are diagnosed with L-TGA beyond childhood eventually
present with systemic ventricular dysfunction or failure, systemic tricuspid
valve regurgitation, or arrhythmias.
• When systemic tricuspid regurgitation is present, these adults should be
referred for systemic AV valve replacement before they have morphologic
right ventricular failure or progressive dysfunction (systemic right
ventricular EF less than 40 percent).
• PA banding has been found to improve systemic AV valve regurgitation in
select patients.
• Cardiac transplantation or ventricular assist device placement should be
considered in patients with persistent heart failure refractory to these
measures and medical management.

21. • conventional repair/Physiological Repair
• Associated cardiac anomalies can be repaired with this approach, albeit
that the morphologically right ventricle remains as the pump to the
systemic circulation.
– ventricular septal defects, if present, can be closed,
– obstruction within the left ventricular outflow tract can be relieved by
either resection or placement of a valved conduit, and the
– tricuspid valve, if leaking, can be repaired or replaced.

22. • ventricular septal defect
• Usually the VSD is perimembranous in position-
• NO right ventriculuotomy and no damage to systemic av valve
- incision in the right atrium,, through the morphologically mitral valve either
displacing septal leaflet or cutting the annulus .
• Some muscular outlet defects can be
– closed via the pulmonary trunk and the pulmonary valve.
• The conduction system passes in anterocephalad fashion around the
pulmonary outflow tract.
• To avoid damaging the conduction system, either continuous or interrupted
sutures are placed on the morphologically right ventricular margin of the
defect superiorly, and from the morphologically left ventricular side of the
margin inferiorly
• PA banding to prevent pulomonary overcirculation and PAH –May help for
anatomic repair in future.

23. • LVOTO
• Mostly subvalvular posteriorly located overlied by RV anteriorly.
• Conduction system runs on left ventricular side of septum- any tension on
septum can damage it.
• Incisions placed across the attachments of the pulmonary valvar leaflets
• the ventriculotomy is placed towards the apex of the ventricle
– careful resection of accessory tissue, or
– open pulmonary valvotomy, but in general
– a valved conduit is placed from the morphologically left ventricle to
the pulmonary arteries so as to relieve the obstruction.
• Valved conduits will not last forever, and most will need to be changed.
• It is wise, therefore, to close the pericardium with a membrane to protect
the heart during sternal re-entry at reoperations.

24. • Morphologically tricuspid valve abnormalities
• Repair or replacement of the morphologically tricuspid valve sometimes
has to be done as part of physiological repairs when there is severe
tricuspid valvar regurgitation.
• with problems of continuing cardiac failure, since often the
morphologically right ventricle is failing by the time such surgery is
entertained.
• In addition, particularly in younger patients where there is marked
dysplasia of the valvar leaflets, repair can be extremely difficult, if not
impossible.
• Under these circumstances, replacement may well be necessary.
• Difficulties are Similar to ebstein anamoly

25. Classic Operation of CC-TGA
1. Repair of ventricular septal defect
2. Repair of coexisting VSD & PS
· Extracardiac conduit
· Without extracardiac conduit
3. Correction of incompetent tricuspid valve
· Repair ( annuloplasty )
· Replacement
4. Fontan-type repair
Straddling, A-V canal , hypoplastic ventricle

26. INDICATIONS FOR OPERATION
• CCTGA per se is not a definitive indication for a reparative operation.
• When VSD coexists.
• When VSD and important pulmonary stenosis coexist
• When important left-sided tricuspid regurgitation coexists
• When complete heart block develops
• (1) presence of straddling tricuspid chordae (increasing risk of
postoperative complete heart block), (2) AV septal defect, and (3)
presence of left-sided tricuspid regurgitation may be considered by some
to be indications for a Fontan, rather than biventricular repair.

27. Repair of Coexisting Ventricular
Septal Defect
• Preparations for operation, median sternotomy, and placing pericardial
stay sutures are as usual.
• It is done where the large VSD is only associated anomaly and it keeps RV
as systemic ventricle and has disadvantage of increased incidences of late
RV failure.
• Different approaches to repair the VSD includes:
1. Through Right-Sided Mitral Valve
2. Through Aorta
3. Through Left-Sided Tricuspid Valve

29. Through Right-Sided Mitral Valve
• Cardiopulmonary bypass (CPB) is established in the usual manner.
• The right atrium is opened through an oblique incision.
• The VSD is examined through the right-sided mitral valve.
• When exposure is suboptimal, an incision is made in the base of the mitral
valve septal leaflet near the superior commissure and through the base of
the commissural tissue into the mural leaflet.
• Location of anterior AV node and bundle of His arching over the
subpulmonary outflow tract and passing anterior to the VSD are
conceptualized.
• VSD repair is made by sewing into place a properly sized patch of either
glutaraldehyde-treated autologous pericardium or double-velour-knitted
polyester, keeping sutures on left (RV) side of defect anterosuperiorly,
anteriorly, and as much as possible inferiorly.

32. Through Aorta
• An attractive alternative approach is closing the VSD through the aorta,
which allows the patch to be sutured into place from the RV (left-sided)
aspect of the septum.
• this may reduce prevalence of perioperative complete heart block.

33. Through Left-Sided Tricuspid Valve
• When isolated dextrocardia complicates CCTGA and VSD, the VSD can be
repaired through a left-sided incision in the usually large left-sided left
atrium.
• Exposure through the left-sided tricuspid valve usually allows good
exposure, and surgically induced heart block should be avoidable because
suturing is all on the RV (left) side of the septum.

34. Repair of Coexisting Ventricular Septal
Defect and Pulmonary Stenosis
• valvotomy is performed as for isolated pulmonary valve stenosis .
• Obstructing fibrous subvalvar tags are excised.
• A subvalvar fibrous membrane can be excised, except at the
anteroinferior angle.
• Aneurysm of the membranous ventricular septum is excised and the
deficiency closed as part of VSD repair.
• Muscle must never be removed from the rightward (medial) aspect of the
right-sided LV outflow tract or from the anterior part adjacent to the
pulmonary "anulus," because the His bundle lies there.

35. • it has seemed reasonable not to revert to CPB and place a valved
extracardiac conduit if the PLV/RV in operating room is less than about
0.85, considering that the right-sided ventricle and valve are a
morphologic LV and mitral valve.

36. Placing Valved Extracardiac Conduit
• When pulmonary stenosis is so severe that the
patient is cyanotic
• if simple procedures are unsatisfactory or
postrepair PLV/RV is too high, a valved
extracardiac conduit is used.

37. • a site is chosen for attaching the conduit to the right-sided LV by
examining the LV interior through the mitral valve. A site is chosen on the
anterior wall, but rather inferior and away from any papillary muscles. Left
ventriculotomy is then made.
• There is reasonable flow across the native LV-pulmonary trunk outflow
tract, it can be left intact, creating an end-to side anastomosis of conduit
to pulmonary trunk. This results in LV ejection via two routes: native tract
and conduit.
• More commonly, when a conduit is required, obstruction is severe;
therefore the pulmonary trunk is transected at the valve level, proximal
stump oversewn, and conduit connected end to end to distal pulmonary
trunk.

39. • Estimating length and lie of the conduit is important to avoid its
compression by the sternum.
• the conduit must be of sufficient length to prevent kinking, and the valve
must lie away from the LV so it is not distorted.
• The conduit curves to the right around the right atrium and atrial
appendage.
• Typically conduit is positioned to right side of the midline and ascending
aorta.
• A composite conduit is usually constructed using a distal valved allograft
and a proximal polytetrafluoroethylene or polyester tube.
• Distal position of valved conduit component within the composite allows
the valve to be positioned more posteriorly in mediastinum, thereby
avoiding compression and distortion of valve with sternal closure.

40. Transanular Patch
• Doty and colleagues have proposed using a posteriorly placed transanular
patch across the pulmonary valve "anulus“ in this situation.
• average gradient across the repair was 40 mmHg.

41. Correction for Regurgitant Left-Sided
Tricuspid Valve
• When important left-sided tricuspid valve regurgitation coexists, repair
and anuloplasty are only occasionally successful, but should be attempted
if it seems feasible.
• Valve replacement is the same as for a left-sided mitral valve.
• The replacement device is either sewn in with interrupted pledgeted
mattress sutures or simple interrupted sutures. A continuous suture
technique is not desirable when there is absence of a welldefined anulus.

42. Results

43. Morphologic Right Ventricle
Supporting Systemic Circulation.
• Early (hospital) death:
• for CCTGA and VSD, hospital mortality has been 5% or less .
• When performed for CCTGA with coexisting VSD and important
pulmonary stenosis, it has been 10% to 20%.
• When performed for coexisting left-sided tricuspid valvar regurgitation
requiring valve replacement, it has been 15% to 25%.

45. • Anatomic repair —
• Associated lesions remains the major determinant regarding surgical
repair.
• The poor late outcome results associated with conventional repair of L-TGA
has led to anatomic correction to make the morphologic LV the systemic
pump and the morphologic RV the pulmonary ventricle.

46. 1. Morphologic LV that is prepared (ie, sufficiently hypertrophied or
“trained”) to take over the workload of the systemic ventricle,
thereby minimizing the likelihood of postoperative LV failure.
2. Current LV/RV pressure ratio greater than 0.7
3. Unobstructed LV-PA & RV-Ao connections
4. Balanced ventricular & AV valve sizes
5. Septatable heart, without AV valve straddling
6. Translocatable coronary arteries
7. Competent mitral valve with good LV function
(Karl TR, et al. ATS 1997)
Proposed Patient Selection Criteria

47. • Anatomical Correction
• The current choice of surgical intervention for anatomic correction of the
ventricles is largely dependent on the
– presence of subpulmonary obstruction and the
– anatomy of the VSD:
• morphologically left ventricle is restored to pumping the systemic
circulation by
– double-switch operation-combining atrial and arterial switch
procedures, or
– atrial switch along with ventricular rerouting.

48. • Double -switch operation
– Where competent and non-stenotic valves.
• Atrial switch along with ventricular rerouting/ Senning-Rastelli procedure.
– Where there is pulmonary stenosis or atresia, usually in association
with a large ventricular septal defect, the atrial switch is combined
with tunnelling of the morphologically left ventricle to the aorta.
– A valved conduit is then placed from the morphologically right
ventricle to the pulmonary arteries.

49. • Pulmonary artery banding or left ventricular “training” —
• Not needed in significant PS/pulmonaryhypertension/unrestrictive VSD.
• In these patients, the LV is already functioning at pressure levels
consistent with what will be required as the systemic ventricle.
• However, in other patients in whom the LV is not initially ready to become
the systemic ventricle, PA banding is used to “train” the LV.

50. • Pulmonary artery banding or left ventricular “training” —
• In L-TGA patients with a LV that is not ready to function as the systemic
ventricle, placement of a band on the pulmonary artery (PA) is used to
increase the afterload of the morphologic LV.
• This exposure to near systemic pressure increases the LV posterior wall
thickness (ie, left ventricular “training”).
• Altering the left and right ventricular pressure ratio may also reduce the
right ventricular sphericity and improve the geometry of the right ventricle
prior to anatomic correction .

51. • Although pediatric cardiac surgical centers use varying measurements to
determine if a morphologic LV is adequately prepared for the systemic
circulation, most published reports suggest a morphologic LV pressure of
66 to 80 percent of systemic pressure is sufficient.
• In addition, others recommend that normal LV mass and thickness for
systemic function using echocardiography and/or magnetic resonance
imaging be required prior to anatomic correction .
• Risk factors for failure of PA band retraining include
– mild LV dysfunction before banding,
– development of significant LV dilation and dysfunction, and
– postoperative development of tricuspid regurgitation .

52. • In several case series, the median time from PA banding to the double
switch procedure ranged from 2 to 14.5 months .
• Typically, PA banding appears to be more successful in patients less than
13 years of age, and the younger the patient, the shorter interval required
for training.
• Patients older than 16 years of age appear to be unlikely to achieve
sufficient LV function to proceed to anatomic correction.

53. • Pulmonary artery banding for Large ventricular septal defect —
• In infants with a large unrestrictive VSD, an increase in pulmonary blood
flow may result in heart failure in the first few weeks of life as the
pulmonary vascular resistance falls.
• The placement of a PA band can be considered in patients who are
refractory to medical management.
• Creating increased resistance to the pulmonary circuit will reduce
pulmonary blood flow, improve the symptoms of heart failure, and
promote weight gain.
• Promotion of growth is desired as anatomic surgical correction is easier to
perform in a larger infant.

54. • Double switch operation —
• The double switch (DS) operation
consists of an atrial switch procedure
that creates an intra-atrial baffle
(Mustard or Senning procedure) and an
arterial switch operation (ASO).
• The intra-atrial baffle diverts the
deoxygenated systemic venous return
into the subpulmonary ventricle and
oxygenated pulmonary venous return
to the subsystemic ventricle.
• The ASO involves transection of both
great arteries, and then translocation of
the vessels to the opposite root similar
to the ASO procedure performed for D-
TGA requiring coronary artery transfer.

55. • Relocation of the pulmonary trunk may be achieved by transposing the
pulmonary arteries anterior to the reconstructed aorta, or they may be
left in posterior position.
• In general, if the aorta is more or less anterior to the pulmonary trunk,
then the pulmonary arteries are relocated anteriorly.
• If the arterial trunks are more side-by-side, then we leave the pulmonary
arteries behind the newly reconstructed aorta.
• At the end of the procedure, it is important to check on the reconstruction
using transoesphageal or epicardial echocardiography, confirming the
patency of the venous and arterial pathways as well as ensuring adequate
ventricular function

56. • After a DS procedure, normal concordance is established with systemic
deoxygenated blood baffled across the tricuspid valve into the
morphologic right ventricle and flow into the pulmonary artery.
• In addition, the oxygenated pulmonary venous return is baffled from the
left atrium across the mitral valve into the morphologic left ventricle and
then pumped across the neo-aorta to the systemic circulation

57. • This operation is a technically difficult and challenging procedure with a
long cardiopulmonary bypass time.
• Therefore, identifying the ideal surgical timing is a complex issue.
• Various centers report a median age at the time of surgery that ranges
from 7 months to 3.2 years and a median weight of 9.6 to 14.7 kg.
• Early hospital mortality is reported to range from 0 to 7.4 percent, and
reported event-free survival rates are between 70 to 85 percent at 10
years .
• In addition, coronary artery transfer is required. As a result, in patients
undergoing this surgical intervention, delineating the coronary anatomy is
mandatory.

58. • Senning-Rastelli procedure —
• In patients with L-TGA that have a VSD and LV outflow tract obstruction,
the Senning-Rastelli (SR) procedure is typically used.
• In this intervention, the intra-atrial baffle (Senning tunnel) is created and a
baffle is placed in VSD so that the blood from the LV is directed into the
aorta, and a conduit is placed between the right ventricle and pulmonary
artery (Rastelli procedure).
• The Rastelli procedure requires a sizable and appropriately located VSD so
that the baffle can be placed to redirect blood flow into the aorta.
• The intermediate-term results show improved survival of this group
compared with the patients undergoing a double switch operation same
used for D-TGA, VSD, and LV outflow tract obstruction.
• Long-term, conduits become stenotic as they do not grow as the child
grows. As a result, patients who undergo a Senning-Rastelli procedure
require serial conduit replacements.

59. • Ventricular Rerouting Combined
with Atrial Redirection
• The atrial switch is performed in
the same manor as for the double-
switch procedure
• An incision is made in the
morphologically right ventricle,
permitting creation of an
intraventricular tunnel between the
ventricular septal defect and the
aorta.
• In creating this tunnel, care has to
be taken to avoid any subaortic
stenosis.
• The repair is completed by placing
a valved conduit from the right
ventriculotomy to the pulmonary
arteries.

60. • Outcome and complications — Because these procedures were initially
introduced in the 1990s, there are limited long-term outcome data.
Nevertheless, several case series have provided information regarding
mortality and morbidity.
• Mortality —
• The following case series demonstrate comparable mortality rates to that
seen with conventional repair. It remains to be seen if long-term survival
improves
• In a large case series of 113 patients from an English pediatric cardiac
surgical center of patients undergoing anatomic repair from 1991 to 2011,
actuarial survivals at 1, 5, and 10 years were 88, 84, and 84 percent in the
DS group (n = 68), and 92, 92, and 77 percent in the SR group (n = 45),
respectively .
• Early deaths occurred in five patients in the DS group, and no patients in
the SR group

61. • Morbidity —
• The complications associated with anatomic correction in patients with L-
TGA are primarily due to conduction abnormalities (ie, complete heart
block and arrhythmias), left ventricular dysfunction, and neo-aortic
regurgitation.
• In addition, some of the baffle-associated complications seen in patients
with D-TGA who undergo ASO repair may also occur in patients with L-TGA
who undergo DS operation.

62. • Conduction abnormalities —
• New onset complete heart block and atrial arrhythmias are common
complications postoperatively .
• In the previously mentioned English case series of 113 patients.
• After anatomic correction, pacemaker insertion was required in 10 of the
68 patients who underwent DS and in 5 of the 45 patients with SR
procedure. In this cohort, tachyarrhythmias were observed in four patients
preoperatively and developed in four patients postoperatively (three in
the DS group and one in the SR group).

63. • Left ventricular dysfunction — .
• Morphologic LV dysfunction -reported in 14 to 18 percent of patients .
• Due to the small numbers of patients, it is currently difficult to determine
with certainty the underlying cause or risk factors of postoperative LV
dysfunction.
• In the previously mentioned English case series, 16 of the 113 patients (14
percent) developed LV dysfunction postoperatively, all of whom were in
the DS group .

64. • Neo-aortic regurgitation —
• Patients who have undergone DS appear to be at greater risk for neo-
aortic regurgitation than patients who have undergone SR procedure.
• In the case series from England, 70 percent of patients after DS repair had
at least mild aortic insufficiency (AI) at follow-up, including six patients
with severe AI requing AVR.

65. • Risk factors for neo-aortic root dilation were previous pulmonary arterial
banding and ASO performed in a later era
• A change in surgical technique is a likely explanation for the association
between surgery in a more recent era with neo-aortic root dilation,
possibly related to the increased size of the coronary “buttons” taken for
the translocation.
• In this current era, pulmonary arterial banding is very rare as complete
repairs are typically performed in the first week of life, thereby reducing
the frequency neo-aortic regurgitation.

66. • Coronary artery stenosis or insufficiency
• The incidence of coronary events continues to be bimodal with the
majority of events (89 percent) occurring in the first three months
following the ASO .
• These tend to be related to “kinking” or other anatomic obstructions to
coronary perfusion. Unexplained ventricular dysfunction or poor
hemodynamics should prompt early evaluation of the coronaries in the
postoperative setting.
• Risk factors for the development of coronary events include type of
coronary anatomy (presence of a single coronary orifice ) and the
occurrence of a major intraoperative event (coronary translocation
difficulty, left ventricular dysfunction, cardiac arrest, or temporary
mechanical support at the end of the intervention).

67. • Baffle-associated complications —
• Although there are limited reports of baffle-associated complications in
patients with L-TGA undergoing anatomic repair, they have been reported
frequently in adult patients undergoing D-TGA surgical repair
• Obstruction at the right atrial and superior vena caval junction is a
recognized complication of the Mustard procedure. The clinical
presentation may include chylothorax, upper extremity edema, or facial
plethora.
• Pulmonary venous obstruction is a complication more commonly
associated with the Senning procedure. Pulmonary venous congestion
may be an early manifestation. Progressive obstruction may be seen later
and may present with symptoms of reactive airway disease.

68. • Reintervention —
• Surgical reintervention is common in patients who undergo either a SR
procedure or DS operation as illustrated by the following findings from the
previously mentioned English case series :
• In the SR group (n = 45), 34 reinterventions were performed in 16 patients
including 14 right ventricular-pulmonary artery conduit changes or
ballooning.
• In the DS group (n = 68), 41 reinterventions were performed in 13 patients
including six aortic valve replacements, and surgical and catheter
reinterventions of the Senning pathway in 14 patients.

69. • FOLLOW-UP CARE —
• Longitudinal follow-up care is required in all patients with levo- or left-
transposition of the great arteries (L-TGA) by a cardiologist with expertise
in congenital heart disease.
• Clinicians need to know the potential complications following the various
surgical repairs and in unoperated patients.
• Follow-up routine care includes focused history, physical examination, and
detailed imaging study by echocardiography and/or magnetic resonance
imaging (MRI).

70. • History —:
• Episodes of syncope or palpitations that may suggest an underlying
arrhythmia or complete heart block
• Increasing exercise intolerance suggestive of declining systemic ventricular
function or increasing pulmonary artery obstruction
• Exertional chest pain may suggest coronary artery insufficiency.
• Edema of the face and upper extremities suggest superior venal caval
obstruction due to a baffle complication seen in the Senning procedure
• Dyspnea may suggest systemic atrioventricular (AV) valve regurgitation or
systemic ventricular dysfunction in the adult patient that is unoperated

71. • Physical examination —
• Vital signs, particularly the pulse, to determine any irregularity that
suggests an underlying arrhythmia
• Cardiac auscultation to detect any murmur (eg, pulmonary stenosis, aortic
or tricuspid insufficiency) or gallop (eg, failure)
• Examination for signs of cardiac failure including pulmonary congestion,
peripheral edema, and hepatomegaly

72. • Tests — Routine testing includes electrocardiography and
echocardiography.
• Electrocardiography (ECG) is performed yearly to detect and diagnose
arrhythmias. ECG is essential to look for complete heart block as there is a
2 percent annual risk for the development of complete heart block
• Holter or event recorder monitoring may be useful in patients with a
history suggestive of arrhythmia.
• Routine echocardiography is used to assess ventricular function, detect
pulmonary artery stenosis, and evaluate competency of the neo-aortic
valve. Evaluation of the systemic and pulmonary venous baffles can also
be performed with echocardiography.
• Angiography remains the preferred modality to diagnose coronary artery
occlusions in patients who undergo the arterial switch operation

73. • Cardiac magnetic resonance imaging is an excellent tool to quantify
ventricular function.
• It should be used when evaluating adults who have not undergone repair,
and can be used to accurately assess left ventricular thickness and
function in those patients who have undergone PA banding.
• This diagnostic modality is also helpful in identifying fibrosis and scar
formation.

74. • Endocarditis prophylaxis —
• Prophylactic antibiotics for endocarditis are recommended for patients
who have surgical repairs that include the use of prosthetic material (eg,
heart valve), prior episode of endocarditis, and those with high-risk lesions
for endocarditis (eg, unrepaired cyanotic heart disease or with a residual
defect such as a patch margin VSD).

75. • PREGNANCY —
• In general, women with a systemic ventricular ejection fraction that is less
than 40 percent and/or have a New York Heart functional class III and IV
should be counseled against pregnancy as the added volume load of
pregnancy is typically not well tolerated.
• In a one study of 22 women with L-TGA, 50 of the 60 pregnancies resulted
in live births including one preterm birth at 29 weeks gestation. None of
the infants had congenital heart disease.
• There were no pregnancy-related deaths but one woman developed heart
failure due to worsening systemic atrioventricular valve regurgitation.
• In addition, one woman with 12 pregnancies resulting in 10 live births
subsequently developed endocarditis and heart failure

76. Morphologic Left Ventricle Supporting
Systemic Circulation
• Early (hospital) death:
• several studies suggest that early outcomes are as good or better with
more complicated "double switch" procedures that assign the
morphologic LV to the systemic circulation.
• patients with structurally abnormal tricuspid valves, mortality was 11%
following "anatomic repair," and 33% following "physiologic repair“.
• Early mortality ranged from 0% to 14%.

77. • Time-related survival:
• Limited late follow-up is available in this patient group

78. Modes of Death
• A few patients die suddenly. this is due to sudden appearance of complete
heart block with ventricular asystole or fibrillation.
• RV dysfunction.

79. Incremental risk factors for CCTGA

80. Postrepair Complete Heart Block
• In all reported series, prevalence has been 15% to 30%.
• chordal straddling or insertion on the septal crest (usually from the left-
sided tricuspid valve) increases the probability of producing complete
heart block at the time of VSD repair.

83. Development of Tricuspid Valve
Regurgitation
• Immediately after simple, classic repair of CCTGA with VSD, left-sided
tricuspid valve regurgitation sometimes appears.
• Operations assigning the morphologic LV to the systemic circulation result
in improved tricuspid valve function.
• Tricuspid valve regurgitation also may be associated with development of
complete heart block.
• When the RV is placed in the pulmonary circulation, tricuspid valve
function typically improves, often without specifically surgically addressing
the valve.

84. Functional Status
• Most surviving patients with CCTGA consider themselves to have normal
functional capacity.
• 78% to 83% were in New York Heart Association (NYHA) functional class I,
and the remainder in class II.

85. Ventricular Function
• When surgery involves placing the morphologic LV in the systemic
circulation, early follow-up and midterm follow-up studies demonstrate
both well-maintained LV and RV function but converse is not true.

86. "Double Switch" Procedures
Combined with Bidirectional Superior
Cavopulmonary Anastomosis
• Use of the bidirectional superior cavopulmonary anastomosis, as part of
operations for CCTGA when the morphologic LV is placed in the systemic
circulation has a number of specific advantages:
• It may benefit the small or poorly functioning RV
• It importantly reduces complexity of the atrial baffle procedure
• It eliminates complications related to the superior limb of the atrial baffle
• It reduces flow across an RV-pulmonary trunk conduit
• It likely increases conduit longevity
• This reduces myocardial ischemia time because the cavopulmonary
anastomosis can be performed during rewarming after aortic clamp
removal and myocardial reperfusion is established.

88.  Problems of physiologic repair
• Progressive tricuspid regurgitation
• Right ventricular dysfunction
• Atrioventricular dysfunction
• Conduit related problems
Congenitally Corrected TGA

89. Tricuspid regurgitation
• Volume load on the right ventricle
• Low incidence with naturally occurring
pulmonary stenosis
• Movement of interventricular septum
Congenitally Corrected TGA

90. Congenitally Corrected TGA
Tricuspid valve abnormality
• In IVS
Preop. 38% postop. 60%
• In VSD
Preop. 90% postop. 56%
• In VSD+PS
Preop. 36% postop. 36%

91. Causes of Tricuspid Regurgitation
• Structural alteration of tricuspid valve component
Congenitally abnormal tricuspid valve
Adherence of septal leaflet or chordae to VSD patch
Asynchronous papillary muscle contraction with RBBB
Supraventricular or ventricular arrhythmia
• Abnormal function of structurally normal valve
Dilated annulus
Distraction of papillary muscles
Right ventricular or papillary muscle dysfunction
Congenitally Corrected TGA

92. 1. Ventricle shape
Cylindric vs. crescent-shaped cavity
2. Contraction pattern
Concentric vs. bellow-like contraction
3. Pumping action
Pressure pump vs. low pressure-volume pump
4. Coronary artery supply
Two system vs. one system
5. Embryology
Primitive ventricle vs. bulbus cordis
6. Papillary muscles
Two papillary vs. small & numerous (septophylic)
Characteristics of Both Ventricles LV Vs RV
The long-term systemic workload results in progressive tricuspid regurgitation
that increases volume overload and contributes to ventricular dysfunction and
failure. Increase the vulnerability of this ventricle to ischemia, particularly
when hypertrophy is present