DR.ANKIT JAIN

PROF.A.SAXENA

SURGICAL MANAGEMENT OF

TGA

Prevelance D-TGA accounts for 5% to 7% of all congenital heart

defects

Prevalence is 0.2 per 1,000 live births with male

preponderance of 2:1 to 3:1

Sibling recurrence rates is 0.27% and 2%, in simple and

complex forms

HISTORY

The TGA was first described by Mathew Baillie in 1797, in

book "The Morbid Anatomy of Some of the Most Important

Parts of the Human Body

The term transposition was 1st used by Farre 1814

Meaning that aorta and pulmonary trunk were placed (positio)

across (trans) the ventricular septum

EMBRYOLOGY Most accepted theory is abnormal development of the B/L

subarterial conus

Normally both the subaortic conus and subpulmonary

conus are present initially and both great arteries are placed

above RV

Subaortic conus resorb and aorta migrates inferior and

posterior to lie over LV

Contd.. Subpulmonary conus persists so that PA remains stationary

over the right ventricle

In D-transposition, the subpulmonary conus resorbs

So pulmonary artery moves inferior and posterior with thepulmonic valve in fibrous continuity with the mitral valve

Failure of the septum to spiral

1. Straight septum

2. Parallel arrangement of RVOT and LVOT

d- and l- transposition Describe the spatial relationship between the aorta and the

pulmonary trunk

In d-transposition, the aortic valve lies to the right of the

pulmonary valve

This is the most frequent arterial arrangement In TGA

Other possibilities of arterial distribution exist in this

setting, thus the two concepts are not synonyms

ARTERIAL RELATIONSHIP

ASSOCIATED LESIONS

In 75% of the cases, the ventriculoarterial discordance is an

isolated finding simple transposition with PFO or ASD

VSD (25% to 40%)

LVOT Obstruction (25%)

Aortic arch anomalies

Coronary abnormalties

Atrioventricular Valve Abnormalties

ASSOCIATED LESIONS Data from series of 260 patients undergoing operation at GLH, 1964-1984

VSD VSD (20-25%)

Anterior malaligned VSD , increases the probability that

the patient will have aortic arch anomalies

Posterior malaligned VSD is associated with LVOT

obstruction

Overridding and straddling of tricuspid valve is associated

LVOT OBSTRUCTION Present in up to 25 percent of patient

1.Dynamic LVOT obstruction

Patients with IVS

ASO alone is curative

It is rare in neonates because of high PVR

2. Fixed LVOT obstruction

TGA/VSD have high incidence of severe anatomical

LVOT obstruction

OTHERS

PDA present in 50% at two weeks of life

Aortic obstruction - rare in IVS, occurs in 7~10% with

VSD

Right aortic arch 5%, more common with VSD

Leftward juxtaposition of the atrial appendage 2.5%, more

common with underdevelopment of RV

Right ventricular hypoplasia

HISTORY OF TREATMENT Surgery for TGA started in 1950 by Blalock and Hanlon at

Johns Hopkins, closed method of atrial septectomy

Edwards, modified it in 1964 by resuturing the septum to

connect the right pulmonary veins to the RA

In 1953, Lillehei and Varco described a “partial physiologic

correction” (anastomosis of RPV to RA , and IVC to LA a

technique known as the “Baffes operation)

CONTD.. Major step in Palliation of TGA was , BAS in 1966 by

Rashkind and Miller in Philadelphia

Park introduced Blade atrial septectomy in 1975

Switching the venous return at atrial level Senning in

1959

The Mustard procedure, in which the atrial septum is

excised and a pericardial baffle used to redirect blood was

devised to create larger atria (1964)

CONTD.. Rastelli procedure for anatomic repair of

TGA/VSD/LVOTO in 1969

Jatene in Brazil introduce ASO in 1975 (TGA/VSD)

1980 REV for TGA /VSD/LVOTO

1977 Yacoub et al. two stage repair

1983 Quaegebeur and Castaneda, primary repair in neonate

1988 Boston group, rapid two-stage ASO

1982-Lecompte introduced his manoeuvre to directly

anastomose the PA’s to the neo-pulmonary “the french

connection

WHEN TO OPERATE There is no clear guidelines to inform the surgical decision

regarding the timing of complete repair

A recent study by anderson et al tried to find out the

optimal timing of ASO

METHODS In a Study of 140 selected infants with D-TGA undergoing

ASO

The authors analyzed the influence of age at surgery onearly “major” morbidity

Major morbidity was defined as

Cardiac arrest, ECMO support, delayed sternal closure,infection, CNS insult, reoperation or readmission at 30days

The median age at operation was 5 days (range: 1 to 12days)

Anderson et al arterial switch operation improves outcomes and reduces costs for neonates with transposition of the great arteries. J Am Coll Cardiol 2014;63:481

RESULTS Decreasing probability of major morbidity between 1 and 3

days with increase after 3 days

ASO between 1 and 3 days had an incremental benefit of

decreasing morbidity (46%) for every day later that surgery

was performed

Opposite was true for neonates undergoing switch after 3 days

of age

CONTD..

In the “older” cohort, there was an incremental increase in

major morbidity (47%) for every day later that surgery was

performed.

The authors inferred that, ASO is ideally performed on day

of life 3

ADVANTAGES OF DELAYING ASO

1) Transition from fetal to neonatal circulation

2) Reduction in pulmonary vascular resistance

3) Kidney and liver function improvement

4) Initiation of enteral nutrition

5) Evaluation for other congenital anomalies

6) Family preparation for surgery

Harms A good Spo2 may be associated with paradoxically low

cerebral oxygen delivery

Cerebral SvO2 is significantly lower than predicted from

the Spo2 in neonates with a run-off lesion

Even a few days delay in ASO may increase CNS injury

ASO The great arteries are divided

Coronary button transfer

Lecomptes maneuver

Great artries reconstruction

Closer of any intracardiac communication

Considerations in ASOCoronary anatomy

Usual course

Intramural course

Anterior/ posterior looping

Single coronary

Relationship and size of the great arteries

Associated cardiac defects

–VSD

–Atrioventricular valve anomalies

–Aortic arch obstruction

–Subaortic stenosis

Left ventricular regression

GREAT ARTERIES TRANSECTION

Transection of arterial trunks

–Aorta

Transacted just above ST junction

–PA

Transacted as far distal near bifurcation to

accommodate coronaries

Coronary Transfer

Origin,Course and the presence of infundibular branches

are identified

Coronary buttons are harvested with a large cuff of aorta

extending well into the SOV

Proximal coronary arteries are mobilised to avoid tension

and distortion without sacrificing the infundibular branches

LM is inserted into the left facing sinus

RCA is inserted into the rt facing sinus

COMMON CORONARY PATTERNS

CORONARY ANOMALIES

The most common pattern (67% of cases) LMCA

arising from the left facing sinus and branching

into LAD/LCx

The 2ND MC (16% of cases) is the LAD arising

from the left facing sinus and the RCA /LCx

arising from the right sinus

So the LCx courses posterior to the pulmonary

artery and there are chances of kinking of LCx

TRAPDOOR CORONARY TRANSFER

Pulmonay trunk transected as distally as possible

Coronary artery will be transferred by creating a trapdoor

flap in neoaorta

This will prevent too far implantation of coronary button

on right-lateral aspect of neoaorta

Coronary button is also positioned more cephalad than in

usual case

TRAPDOOR CORONARY TRANSFER

INTRAMURAL CORONARY

ASO IN PATIENTS WITH INTRAMURAL CORONARIES

LECOMPTES MANEUVER

MORTALITY

Two recent studies demonstrate a hospital

survival rate of >98%

30-day mortality rate for the ASO at <3% with a

1-year survival rate of >96%

University College London. National Institute for Cardiovascular Outcomes

Research, June 20, 2014

Early mortality after ASO

Early mortality is always due to difficulty with coronary

artery transfer

Coronaries are transferred with an margin of 2-3 mm sinus

aorta known as coronary button

Preoperative knowledge of course of coronary is important

to prevent coronary damage during button excision

Metton O Intramural coronary arteries and outcome of neonatal arterial switch operation. Eur J Cardiothorac Surg 2010;37:1246-

OUTCOME AND PREDICTORS OF EARLY MORTALITY

RISK FACTORS

THE “LATE” ASO

TGA+IVS > 1 month

LV is regressed

1977Yacoub introduce PA band to increase the LV mass

and a BTS to relieve the cyanosis

They waited months after procedure

In1994 boutin and jonas found that LVH occurs rather

rapidly and LV mass doubles within a week of PA banding

CONTD.. LV mass increases most rapidly in the first 2 days after

band placement, with an exponential decrease in the growth

rate after that

Disadvantage of long interval 2 stage

1. Band caused scarring

2. Neo-aortic valve incompetance

3. Adhesions caused coronary transfer difficult

LV PREPRATION INDICATORS LV RV pressure ratio greater than 65%

LVEDV >90% of normal.

LVEF >50%

LVEDPWT >4 mm or safely >4.5mm (BSA<.5M2)

Predictive LV wall stress <120 x 103 dynes/cm

LV Mass >70 gm/m2

Nakazawa circulation 1988 ,78, 124-131

Procedure of the two stage ASO

BTS performed followed by a PA band to achieve 75% of

the systemic pressure

Second stage-shunt take down and debanding done

Adhesions usually not a problem as the procedure is being

performed within 7 days

LATE PRIMARY ASO ASO has been successfully performed beyond the neonatal

period up to age 9 month in patients with TGA and IVS

Such patients are more likely to require postoperative

mechanical support

Kang N, de Leval MR, Elliott M, Tsang V, Kocyildirim E, Sehic I, et al. Extending the boundaries of the

primary arterial switch operation in patients with transposition of the great arteries and intact ventricular

septum. Circulation 2004;110:II123-7

AIIMS DATA –A.K BISOI ET AL IJCTVS 2006

Favoured primary aso >21 days

Age 25 – 70 days

Gr A (RTS ASO)-11pt

Gr B(ASO)-- 15 pt

Gr A–3/11 died after first stage

1. 8/11(73%) sucessfully trained

2. 5/11 survival( 45%)

Gr B –13/15 survived(86%)

2 deaths due to cardiac failure

Drawbacks of RTS

First stage puts lot of strain on the ventricle

Potential problems can arise of tightness of PA

band and overflow/blockage of the shunt.

In case of any such event there is danger to life

Evident from the 27% mortality after 1ST Stage

Surgical outcome of primary aso > 6 weeks

Jan 2003- june 2009

55 children ( 42 days to 9 years )

Mortality – 7 pts ( 13% )

Children who had severly regressed LV ( banana shaped )

were operated with integrated ECMO-CPB

Children with regressed lv required longer ventilatory time

and inotropes

AIIMS DATA –A.K BISOI ET AL EJCTVS 2010

ADVANTAGES

1.No time lag to initiate ECMO

2.Enable LV Retraining Under normoxemia condition

3.Early initiation of ECMO prevent end organ damage

d-TGA,VSD,LVOTO Primary palliation by a BTS if LVOTO is not resectable

If resectable then ASO with LVOT resection should be

performed

Where LVOT is not resectable –options

Rastelli

REV or Lecompte

Rastelli procedure

It is done for d-TGA ,LVOTO and a large S/A VSD

Not suitable for non-comitted VSD’s

VSD is closed routing the Aorta to the LV with or without

VSD enlargement

PV is closed from the RV or is transected and suture ligated

RV-PA extra-cardiac valved conduit is placed

CONTD.. For success of this operation, the VSD must be both large

and free of obstruction from AV valve tissue, so that the neo

LVOT is patent

Surgical enlargement of small VSD can be done to

complete the Rastelli repair

Straddling of the tricuspid valve often precludes this type of

repair

Rastelli procedure

REV or Lecompte procedure VSD is closed routing Aorta to the LV after excising the

outlet septum

Pulmonary valve is closed through the RV or is transected

and ligated

PA’s are extensively mobilised

Lecompte manoeuvre is performed

PA’s brought down to anastomose to the ventriculotomy

posteriorly

Anteriorly augmented with a pericardial patch

REV

ADVANTAGES OF REV Avoides the use of an Extracardiac conduit

It involves the resection of the muscular outlet septum,

providing better alignment of aorta and LV

Rastelli operation is associated with more risk of

reintervention due to LVOT obstruction, and extracardiac

conduit problems

REV VS RASTELLISurgery for malposition of the great arteries:the REV procedur Duccio Di Carloadoi:10.1510/mmcts.2007

Nikaidoh Procedure, or Aortic Translocation

Unsuitable for the rastelli or REV procedure

1. Inlet or restrictive VSD

2. Straddling or overriding of the AV valves

3. Coronary artery crossing the right ventricular

outflow tract

Nikaidoh Procedure

Damus-stansel-Kaye procedure

It is for TGA and coronary artery patterns not suitable for

transfer .

The main pulmonary artery is transected and anastomosed in an

end-to-side fashion to the ascending aorta.

The coronary arteries are perfused in a retrograde fashion.

The native aortic valve may be left intact ,VSD is closed

RV to PA conduit is placed

Damus-stansel-Kaye procedure

Useful in patients who are undergoing staged conversion from

atrial baffel to systemic correction

In these patients dense adhesion prohibit coronary transfer and

ASO

Damus-stansel-Kaye procedure

TIMING OF SURGERY These surgeries can be performed in infants >6 months of

age

When cyanosis and symptoms are important before age 6

months

1. BT shunt, followed by a REV within 6 to 18 months

2. Primary REV

SURGICAL OPTIONS

Anatomy Surgical options Comments

TGA/IVS

Arterial switch (Jatene)

Neonatal period, usually within 2 wk of age

Physiologic repair

Senning or Mustard

Usually elective, neonatal-1 yr

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

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

PULMONARY VASCULAR DISEASE

When TGA occurs as an isolated lesion PVD occur in 10%

to 30% at 24 months

In patients with TGA and moderate or large VSD /PDA ,

PVD devlopes rapidly

At 6 months, 25% have developed severe pulmonary

vascular disease (≥grade 3), and 50% at 12 months

Palliative Surgery for Patients withSevere PAH

Palliative operations may be indicated when PVR> 10

woods

If the saturation in the PA is higher than the aorta, an atrial

redirection procedure, will improve streaming and improve

systemic oxygenation

When the ventricular septum is intact, a large VSD is

created in the apex of the ventricular septum

CONTD..

SaO2 in TGA depends on the relative proportions of

systemic venous and pulmonary venous blood reaching the

aorta, and on SvO2

After palliative switch repair, the effective systemic flow is

greatly increased

Decrease in the proportion of systemic venous blood

entering the aorta is also influenced by the rise in SVR that

follows the rise in SaO2

CONTD..

There is an absolute increase in SaO2 of approximately

20%

The only preoperative variable that correlates with

postoperative SaO2 is pulmonary AV difference

A higher AV difference is associated with a higher

postoperative SaO2

POST OPERATIVE SEQUELEWernovsky G et al Guidelines for the outpatient management of complex congenital heart disease. Congenit Heart Dis 2006

MYOCARDIAL ISCHEMIA

Obstructed coronary arteries are present in 5% to 7% of

survivors

Most common cause of morbidity and mortality following

ASO

Commonest in first 3 months after ASO

Coronary obstruction late after the ASO is uncommon

In a long-term study, freedom from coronary events was

88.1 %at 22 yearsKhairy P et al. Cardiovascular outcomes after the arterial switch operation for D-transposition of the great arteries.Circulation2013;127:331

NEOAORTIC ROOT DILATION AND AORTIC REGURGITATION Freedom from N-AR of grades IV, III, and II at 23 years

was 90.2%,70.9% and 20.3 %

Usually mildRISK FACTORS

Older age at time of ASO

Presence of VSD

Bicuspid pulmonic valve,

Previous PA banding

Higher neoaortic root/ascending aorta ratio

LVOTO

Taussig-Bing anomaly

Meshkishvili V. Fate of the aortic valve following the arterial switch operation. Card Surg 2010;25:730

CONTD.. Severe AR requiring intervention is less then 2% in long

term follow up

McMahon CJ et al. Risk factors for neo-aortic root enlargement and aortic regurgitation following

arterial switch operation. Pediatr Cardiol 2004;25:329-35.

Right Ventricular Outflow Tract Obstruction

RVOTO has occurred with sufficient severity to require

reintervention in about 10% of patients

Peak incidence about 6 months after the ASO

In one analysis, freedom from reintervention for RVOTO

was 94% at 1 year, and 79% at 5 years

Swartz et al.Decreased incidence of supravalvar PS after ASO . Circulation 2012;126(11

Suppl 1):S118–22

RVOT OBSTRUCTION The obstruction can occur at multiple levels

Diffuse hypoplasia of the pulmonary trunk commonly

results from inadequate mobilisation of the pulmonary

arteries

FREEDOM FROM SURGERY FOR RVOTO From Norwood WI Congenital Heart Surgeons Society: personal communication; 1992

REOPERATION STUDY The ASO reoperation study revealed that pulmonary artery

reconstruction was required earlier than neoaortic

intervention ( 6.8 years vs. 13.8 years, p < 0.001)

Raju V et al. Reoperation after arterial switch: a 27-year experience. Ann Thorac Surg

2013;95:2105–12.

ARRYTHMIAS

Chronotropic impairment found consistently post-ASO

Associated with residual hemodynamic lesions or CAD

Late post-operative atrial flutter or fibrillation, is

associated with RVOT obstruction

Khairy P et al. Cardiovascular outcomes after the arterial switch operation for D-transposition of the great arteries. Circulation 2013;127:331–9.

SUDDEN CARDIAC DEATH. Most deaths occur 1 to 5 years after the ASO

Probably related to exercise-induced external compression

of unusually distributed coronary arteries

Incidence is 0.3% to 0.8%

VF and late SCD are usually associated with myocardial

ischemia or infarction

Khairy P et al. Cardiovascular outcomes after the arterial switch operation for D-transposition of the

great arteries. Circulation 2013;127:331–9

High-risk patients History of atypical, intramural, or problematic coronary

transfer require screening prior to engaging in high-level

physical activity

Neurodevelopmental Status Significant hypoxemia, acidosis ,long CPB, and low

cardiac output are correlated with abnormal ND andbehavioral testing

In a study behavior, speech, and language delays at 4 and 8years, with significant deficits in visual-spatial and -memory skills

Bellinger DC et al. Neurodevelopmental status at eight years in children with dextro-transposition of the great arteries: The Boston Circulatory Arrest Trial. J Thorac CardiovascSurg 2003;126:1385–96

Neuro developmental status at 4 years (n=74)

Neuro developmental sequelae patients controls P value

WPPS IQ 101.9 108.6 .0007

Speech problem worse better .002

Language expression problem worse better .001

Language comprehension

problemworse better .033

inattentive worse better .033

Karl JTCVS 2004 ,127.1,213 1988-1994

Risk factors for neurodevelopmental outcomes

Pre op factors Severe pre op acidosis & sepsis <.04

Peri op Duration of circulatory arrest .03

JTCVS 2002 124 448

LIFESTYLE CHOICES, CHOLESTEROL, HTN, ANDEXERCISE

Neonatal coronary manipulation, potential endothelial

stress, and ongoing aortic root pathology may increase the

CAD risk

These individuals have limited aerobic capacity on exercise

testing

Atypical coronary anatomy, pulmonary artery stenosis, are

associated with decreased aerobic capacity

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