Transposition of great_arteries

TRANSPOSITION OF GREAT
ARTERIES
Dr amarja sachin nagre
MD,DM,FCA
www.cardiacanaesthesia.in|DrAmarja

D-TGA first described by MATHEW BAILLIE
1797.
Farre coined the word TRANSPOSITION in
1814 (Trans : Across, ponere : to place)
Van Praagh & co-workers introduced terms
Concordant & Discordant 1971.
Clinical description-Fanconi in 1932 &
Taussig in 1938
HISTORY

DEFINITION
Defined as Congenital Cardiac Anomaly in
which Aorta arises from the morphologic R.V.
and pulmonary trunk from morphologic L.V.
(Atrio-ventricular concordance with
Ventriculo-arterial disconcordance)

•D Transposition - Classic complete TGA in
which Aorta is located anteriorly and to
the right of PA.
•L Transposition – When aorta is located to
the left of the PA.

EMBRYOLOGY
 Theory of CONAL INVERSION is most accepted
 Normally sub-aortic portion of conus is absorbed & sub-
pulmonic persists moves anteriorly to connect RV to PA.
 In TGA> sub-aortic persists which brings aorta anterior
to be connected to RV,at the same time sub-pulmonic is
absorbed –pulmonary valve remains posterior in
continuity to mitral valve.

INCIDENCE
5-8% of CONGENITAL HEART DISEASES.
1 in 2300 - 1 in 5100 LIVE BIRTHS
Male Preponderance ratio of 4:1
Association of Extracardiac anomalies <10%
Increased prevalence in Diabetic mother &
Prenatal sex hormone therapy.
More common in later pregnancies

PATHOPHYSIOLOGY

Simple TGA- when TGA pts have intact
ventricular septum & no other
significant associated lesions
Complex TGA- TGA with other associated
lesions like large VSD,
large PDA, LVOTO,hypoplasia
of RV,pulmonary or tricuspid
Artesia.

CO-EXISTING ANOMALIES
PFO/PDA - 50%.
VSD-40% - small, large & multiple.
VSD+LVOTO -5-10%.
LVOTO-5%.
Functional TV & MV anomalies 4%.
Bronchopulmonary collaterals 30%.
CoA, Arch interruption & hypoplasia 5%.

TGA – PHYSIOLOGICAL –CLINICAL
CLASSIFICATION
I. TGA – (IVS or Small VSD)
II. TGA – (VSD Large)
III. TGA (VSD & LVOTO), with Restricted
PBF
IV. TGA (VSD & PVOD), with Restricted
PBF www.cardiacanaesthesia.in|DrAmarja

INTERCIRCULATORY MIXING
ANATOMY PBF IC MIXING
TGV + IVS INCREASE LESS
TGV + IVS + ASD/ PDA INCREASE MORE
TGV + VSD INCREASE MORE
TGV + VSD + LVOTO DECREASE LESS
TGV + PVOD DECREASE LESS

LVOTO
• DYNAMIC – not at birth
-develops after few wks
-degree varies spontaneously
• Decrease PVR – decrease LV sys pres
- but RV pres high to
cause syst movement of septum into LV
• Systolic anterior motion of AML – venturi
effect

LVOTO
FIXED-
Fibrous ridge / membrane
Valvular Stenosis --
Annular Hyperplasia
Accessory mitral leaflet tissue
Leftward/posterior deviation of
infundibular septum

CORONARY PATTERN
 Important in planning arterial switch
 The morphologic RCA-concordant-morph RV
morphologic LCA-concordant-morph LV
 Left and post aortic sinus-face RVOT
right- does not
 Dual sinus origin-90%-lf sinus –LMCA-LAD/LCX
post sinus--RCA
Single sinus origin-both coronaries arise from
one facing sinuswww.cardiacanaesthesia.in|DrAmarja

CLINICAL FEATURES
HISTORY
 M:F:::4:1
 More common in multiple pregnancies
 Neonates- normal to large birth weights

MODES OF CLINICAL PRESENTATION
Predominantly Cyanosis (poor inter
circulatory mixing) -TGA with IVS
& no significant ASD.
CCF (good inter-circulatory mixing)-
TGA with large VSD.
Asymptomatic (balanced mixing)-
TGA with VSD or adequate sized
ASD.

 TGA/IVS – Cyanosis 1st day of life
 TGA/nonrestrictive VSD or large PDA –
Mild Cyanosis
CHF in 3 – 6 weeks.
 TGA/VSD/PVOD – Progressive Cyanosis.
 Infants may have necrotizing enterocolitis-
reduced mesenteric circulation.
 Cerebral infarct & brain abscess can occur.

SIGNS
 S1- Normal
 S2-loud & single
 A2- palpable at the left base.
 Pulmonary arterial impulse is absent.

Contd…
 TGA with LVOTO—Ejection systolic murmur
 VSD murmur is absent at birth, appears after
pulmonary vascular resistance falls. Later
murmur disappears when pulmonary vascular
resistance is high.
 Murmur of fixed LVOTO present at birth, best
heard at mid-left sternal border- sub pulmonary
obstruction- radiate upward & to right.
 Large PDA-systolic murmur (flow from aorta to
PA occurs in systole)www.cardiacanaesthesia.in|DrAmarja

DIFFERENTIAL DIAGNOSIS
• Pulm atresia with IVS
• TOF with absent pulm valve
• TOF with pulm atresia
• TAPVC
• Tricuspid atresia
• Truncus arteriosus

RADIOLOGICAL FEATURES
 Oval / egg shaped cardiac silhouette with
narrow superior mediastinum
{egg on string appearance}
 Mild cardiomegaly
 Increased pulmonary vascular markings.

ELECTROCARDIOGRAM
 RAD, RVH-TGA with IVS or restrictive
VSD
 Biventricular hypertrophy- Large VSD
with low pulmonary vascular résistance
with volume overload of LV.
 LAD –TGA with AV canal type VSD

ECHOCARDIOGRAPHY
• To demonstrate atrio-ventriculo , ventriculo-
arterial relation
Parasternal short axis- cup/ saucer appearance
TGA- double barrel gun
• Diagnose VSD, LVOTO
• Coronary anatomy –see for anterior ,posterior
double looping
- origin from single /2 ostia
• If 2 ostia and ant loop-LAD : RCA
2 ostia and post loop- LCX : RCA
1 ostium and 1 loop – all 3 : RCA
• If coronaries - intramural

Cardiac Catheterization
 Coronary Artery Anomaly.
 VSD,PAP, Pulm vascular resistance & aortic arch
abnormality.
 Therapeutic (Balloon atrial septostomy)
C T Angiography preferred.

Management of TGA
MEDICAL
SURGICAL

Palliative
 Rashkind balloon atrial septostomy.
 Blalock-Hanlon operation.
 Prostaglandin E1 -0.05-0.1mcg/kg/min

ATRIAL balloon septostomy
William Rashkind & William Miller 1966
Indications
• D-TGA
• TAPVC with restrictive ASD
• Tricuspid atresia with restrictive ASD
• Pulmonary atresia with intact IVS
• Mitral atresia
• Pulmonary HTN.

Technique
• Balloon is placed in LA – bobbing movement of balloon
over mitral valve
• Inflate till movement is lost

Technique
• Pull backward
• Interatrial septum gets displaced towards
IVC
• Primum septum ruptures
• Immediately move the catheter cranially
with deflation

success
• Inflated at lower volume -balloon should
easily pass
• HR & BP improve
• Decrease in cyanosis
• Loss gradient across atria
• ASD at least 5-6 mm

complications
• Atrial arrythmias
• Perforation of IVC ,pulmonary veins ,atria
• Air embolism
• Cardiac arrest
• Procedure failure. 12%
• Mortality 10-30%

Other methods
• Echoguided BAS
• Blade BAS
• Parks blade
• Amplatzer fenestrated device for ASD

SURGICAL MANAGEMENT
ATRIAL LEVEL
• SENNING OPERATION
• MUSTARD OPERATION
VENTRICULAR LEVEL
• RASTELLI OPERATION
GREAT ARTERY LEVEL
• ARTERIAL SWITCH OPERATION

Sennings operation
It uses atrial septal flap and
the RA free wall to redirect
the pulmonary and systemic
venous returns at the atrial
level.
First performed in 1958 by
Senning.

Mustard operation
Redirecting pulmonary and
systemic venous return at
the atrial level by using
either a pericardial or a
prosthetic baffle.
First done in 1964.

Complications of atrial switch
• SVC obstruction < 5%.
• IVC obstruction 1%
• Pulm Venous obstruction < 5%.
• Residual intra atrial baffle-shunt <
20%.
• Leaks 1-2%
• Arrythmias >50%, SVT.,sick sinus
syndrome
• RV depression.
• Sudden death.
• PVOD
• TV regurgitationwww.cardiacanaesthesia.in|DrAmarja

RV FAILURE
• 2-15%
• Echo 40% RV dysfunction
• Causes –
• Morphology of RV [RT ventriculotomy]
• Infundibulum - akinetic
• Myocardial ischaemia & hypokinetic segment
• Septum bulging to RV

Surgery at the ventricular level
Rastelli Procedure
 Done in patients of TGA with VSD-PS or
LVOTO.
 First performed in 1969.
 An intraventricular tunnel is created
between VSD and aortic valve, and a
conduit is placed between RV and PA
(homograft/heterograft).

RASTELLI OPERATION

Complications of Rastelli
• Mortality 20-30%
• two year survival 92%
• Conduit obstruction.
• Needs a re-operation as child grows.
• Intervention-relieve RVOTO/LVOTO
• Myocardial dysfunction

Great Artery level
ARTERIAL SWITCH OPERATION
• First done by Jatene in 1975.
• The coronary arteries are transplanted to
the PA, and the proximal great arteries are
connected to the distal end of the other
great artery.

challenge
• Functional adequacy of LV
• Dynamic LVOTO
• Delayed decrease of PA pressure

• One stage procedure (in first few weeks of
life)
• Two stage procedure -pulmonary artery
banding + ASO

Arterial Switch
Lecompte procedurewww.cardiacanaesthesia.in|DrAmarja

Arterial Switch

ARTERIAL SWITCH MORTALITY
• Simple TGA 5% to 15%
• Complex 10 % to 20%
• Survival rates (Kirklin & Barrat Boyes)
85% 5yrs & 81% 9 yrs

Complications of arterial switch
• Kinking & obstruction of coronary arteries- myocardial ishaemia
• Haemorrhage at suture lines
• May require graft
• Asymtomatic ischaemia
• Perfusion defects are common
• Low C.O. state

Supravalvular pulmonary stenosis
(5-30%)
Inadequate growth of PA
Stenosis at suture line
Tension on anastomosis site
Malaligned RVOT to MPA

Supravalvular aortic stenosis
• Seen in < 5%
• Coarctation of neoaorta
• Tubular hypoplasia / kinking of transverse
arch
• Enlargement of neoaorta to see in follow
up
• Neoaortic regurgitation 50 % but always
mild.

CPB in Switch
• CPB at 18-25 deg C
• High degree of myocardial & cerebral
ischaemia
• Necrotizing enterocolitis
• Renal failure.

Damus-Kaye-Stensel operation
• In patients with TGA – VSD – RVOTO –
Damus-Kaye-Stensel operation.
• MPA is transected near its bifurcation and
proximal MPA is anastomosed to
ascending aorta end to side & LV to aorta
continuity is established.
• RV-PA valved conduit.
• VSD is closed to direct the flow from LV to
native pulmonary ( neoaortic valve)

Contd…
• Suitable for pts abnormal coronary
anatomy
• Aortic regurgitation is common
• Suitable for taussig bing type

REV- Reparation a L’etage Ventriculaire Procedure
• Alternative to ASO – TGA/VSD
RASTELLI- TGA/VSD/LVOTO
• Adv- Coronary reimplantation
valved conduit not required
• Procedure - VSD patch for LV to aorta
continuity. PA transected, distal segment
reanastomosed to RV –RV to PA
continuity.

PLAN THE PROCEDURE
• TGA/IVS – ASO
-age< 4 wks – L V regresses
• TGA/VSD- ASO + VSD closure
-if coronaries unfavourable-
Rastelli-age-better conduit
placement
-if CCF – PA banding to restrict
PBF
• TGA/VSD/LVOTO-ASO not as LVOTO
-Rastelli donewww.cardiacanaesthesia.in|DrAmarja

CONTD…
TGA/VSD/PVOD- Surg may not be
appropriate if PVR is high

Anaesthetic Goals
• Maintain HR, Contractility,Preload  C.O.
• Maintain Ductal patency.
• Avoid increase in PVR
• Avoid decrease in SVR.
• Avoid hypoxaemia, hypercarbia,acidosis.

Pre-anaesthetic Considerations
In Pts with decreased PBF & poor mixing,
PVR decreased by 
• 1) Nitric oxide inhalation
• 2) Prostacyclin nebulization
• 3) IV Sildenafil
• 4) Ventilatory interventions.
-Fio2 - increase
-Pco2 – 25-35 mmHg
-pH – 7.5 -7.56

Pre-anaesthetic Considerations
• Pts with Hct > 65 % --- Maintain hydration.
• Chronic cyanotic pts. --- Coagulopathies.

Pre-anaesthetic Preparations
• PGE1 Infusion
• Ballon atrial Septostomy.
• Mechanical Ventilation
• Medications – Inotropes- Digoxin
Diuretics- Furosemide
Antibiotics
• ECMO

PGE1 (Alprostadil)
• Direct vasodilator - PG receptor
• Selectively dilates Ductus arteriosus
• Metabolised – lung enzymes so less
systemic vasodilatation
• Disadv– syst vasodilatation-hypotension
apnea
seizures
fever
expensive
platelet dysfunction

• INDICATION- cyanotic CHD-dec PBF
severe P’ HTN
• Dosage-- 0.05 to 0.1 to max 0.4 µg/kg/m
• PGI -2 - (EPOPROSTENOL)
- long term pulm HTN

NITRIC- OXIDE
• Vasoactive gas– L’ arginine in endothelial
cells
• M/A- diffuses to vascular smooth muscles
increases c GMP
decreases Ca++
• PVR– decreased
• SVR – no change
• OFFSET - ½ life – 6 sec
binds to heme –methHb

• ADV- Selective pulm vasodilator
No systemic actions
Improves V/Q
• DISADV- NO2 –P’ edema
methHbnemia
ciliary depletion
corrosive to metal

• USES- P’ HTN
ARDS
• Therapeutic conc- 0.05-80 ppm
• Onset- 1-2 min
• Available-prediluted in Nitrogen
• Not allowed to contact air,O2

Anaesthetic Management
• Monitoring - Blood Gas ,Electrolytes
ECG, pulse-oximeter
ETCO2,CVP
IBP,Temperature,TEE
• Induction - Opoids –
Hemodynamic stability
No myocardial depression
Blunt reactive P HTN

• Dosage – Fentanyl – 5-25 -100mcg/kg
Sufentanil-0.5-2.5-10mcg/kg
• Isoflurane/Sevoflurane
• Benzodiazepine – Midazolam
• Pancuronium -0.1mg/kg [Vagolytic]
• Ketamine +Glycopyrrolate
• Avoid injecting air alongwith

PostOperative Concern
• ASO
Bleeding
Myocardial ischemia.
Low C. O. state
• Rastelli procedure
RV dysfunction

THANK YOU

Transposition of great_arteries

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