ANAESTHESIA FOR TGA

DrZuraini2020

TGA (cyanotic): 2nd most common congenital cardiac lesion; is a/w the lowest risk of coexisting noncardiac
malformations or genetic syndromes

Hallmark: Ventriculoarterial (VA) discordance

2 main categories:

A. Physiologically uncorrected (d-TGA or TGA): concordant AV, discordant VA – most

common; outcome is very good
1. Simple (75%) : isolated VA discordance with either PFO or PDA, or both
2. Complex :
a) VSD
b) LVOTO (1/3 of pts in TGA-VSD. Rare in TGA-IVS) LVOTO limits pulmonary blood
flow, therefore exacerbates the cyanosis in TGA.
c) DORV and Taussig-Bing (DORV with sub-pulmonary VSD in simple term)
d) Rare : TGA + VSD + coarctation of aorta or TGA + VSD + interrupted Ao arch

Taussig-Bing Heart : Ao originates from RV, PA arises from above the non-restrictive VSD;
frequently a/w subaortic RVOTO bcoz of hypertrophic infundibular septum & Ao arch
obstruction. LV oxygenated blood is preferentially streamed into the PA, therefore
stimulating TGA physiology.

B. Congenitally corrected (cc-TGA or l-TGA): discordant AV & VA

1. Incomplete TGA
2. Complete TGA (d-TGA, TGA)
a) TGA-IVS 70%) } usually neonates >> to prevent involution of LV
b) TGA-VSD 25% } neonates >>to avoid pulmonary overcirculation
c) TGA-VSD-LVOTO. (5-10%) Rastelli/Nikaidoh >> infants >> to provide definitive pulmonary
blood flow
Mixing may occur via secundum ASD, PFO, VSD or PDA. Degree of cyanosis depends on the amount
of mixing btw the parallel circuits.

A successful primary ASO repair is usually first 2-3 weeks of life when there is adequate LV mass to
support the circulation (ie. to prevent involution of LV)

Risk factors for preop mortality:

1. Restrictive PFO or ASD

2. Persistent PHT
3. Complex coronary anomalies / intramural coronary artery
4. Concomitant LV obstruction at conal septum or Ao arch (in VSD)
5. Prem / low birth wt
6. Late Px

• Cyanosis : absence of adequate inter-circulatory mixing

• CHF : large amount of mixing, increased pulmonary blood flow

Pre-Op Assessment

1. Associated cardiac anomalies

2. Adequacy of inter-circulatory mixing (eg if atrial communication - ? restrictive or non-restrictive)
3. BAS?
4. Prostaglandin dependant?
5. Ventricular size & function, LV mass
6. Electrolyte abnormalities
7. Coronary anatomy
8. Presence of coarctation/IAA (reverse cyanosis is indication of significant PHT or of Ao Arch obstruction,
coarctation or IAA)
9. Presence of arrhythmias, inotropic support, cyanosis, CHF and pulmonary overcirculation

Blood & blood products should b available. Chronic cyanosis triggers ↑ erythropoiesis, ↑ circulating blood
volume, vasodilation to ↑ O2 delivery to the tissues & compensate for chronic hypoxaemia. Polycythaemia
results in ↑ blood viscosity, ↑ both PVR & SVR. ↑ PVR is >> than SVR, further ↓ PBF in children who already hv
compromised blood flow.

Adequate hydration (IV) sh b ensured to avoid cerebral, pulmonary & renal thrombosis.

Intra-Op MX

Anaesthetic goal :

#$
1. Maintenance of ductal patency with PGE1 infusion (0.05 – 1.0 %$ /min ) in ductal dependant
pts (usually TGA-IVS). Continue until CPB to assure adequate intercirculatory mixing.
2. Maintenance of CO by maintaining HR, contractility & preload. ↓ CO will ↓ systemic arterial
saturation. (↓ HR → ↓ CO)
 3. The PVR needs to remain lower than SVR to ensure effective blood flow to the lungs. ( If PVR is ↑
>> decrease PBF reduces inter-circulatory mixing, ↓ systemic perfusion. Simple 1st line measures:
ventilatory control :
i. Increase FiO2
ii. Hyperventilation (to achieve moderate metabolic alkalosis). PCO2 25-35 mmHg (3-5 kPa), PH
7.50 -7.56
iii. Avoid acidemia, hypothermia, hypoxaemia

4. SVR should be kept high in relation to PVR to avoid increased recirculation of systemic
venous blood & further worsen cyanosis/sats.
5. In TGA-VSD with symptoms of LV overload & CHF, ventilatory interventions to reduce PVR
only produce a small improvement in sats at the expense of systemic perfusion, therefore
unnecessary.

Induction – intravenous (most). Inhalational >> slower & extremely prone to anaesthetic-induced
myocardial depression.

Pre- & post-ductal placement of pulse oximeters.

IV access is difficult :
• IM ketamine 2-3 mg/kg + IM glycopyrrolate 10 𝜇g/kg

More rapid intubating conditions required & no IV access:

• Ketamine 4-5 mg/kg + glycopyrrolate 10 𝜇g/kg + (succinylcholine 4-5 mg/kg or rocuronium 1.0 mg/kg)
mixed in the same syringe & give IM

Maintenance of anesthesia with hi dose opioid.

* Hi dose opioid (25-100 𝜇g/kg) is particularly useful in neonates & infants (provide hemodynamic stability, do
not depress the myocardium, blunt reactive pulmonary hypertension). Low-to-moderate doses (5-25 𝜇g/kg) in
combo with inhalational or benzo (midaz 0.025 – 0.05 mg/kg) can also be used. Some use 2-15 𝜇𝑔/𝑘𝑔 fentanyl.
*Small quantity of inhalational ag may be used. Neonates → limited contractile reserve in the immature
myocardium → poor toleration of myocardial depressive & systemic vasodilatory effects of inhalational agents.
Synergistic vasodilatory effects of BDZ & opioids.
.
If the neonate was breathing RA pre-op, then the need for supplemental O2 pre-CPB shud b minimal.
SpO2 near 75% are common , typically well-tolerated if adequate systemic perfusion is present. If
desats beyond this occur, the usual causes of desats sh b rapidly assessed. If desats is persistent →
increase FiO2 ; rapid institution of bypass.

If DHCA is planned, the head is also surrounded with ice.

PVR can be reduced:

• iNO
• Sildenafil
• Moderate hyperventilation (to achieve moderate metabolic alkalosis) with high FiO2
(reduce PVR, increase pulmonary blood flow, mixing & arterial sats)
 • Avoid hypoxia, hypercarbia, metabolic acidosis, hypothermia, hi PEEP,
hypoglycaemia, sympathetic stimulation 2° light plane of anaesthesia, pain.
• PGEi continued

SVR should b kept high in relation to PVR (to avoid increased recirculation of systemic venous blood,
& further decrease in arterial sats.)

Strategies to maintain PVR (low PVR ensures effective blood flow to lungs, thus intercirculatory
mixing)
1. Use of opioid & sevoflurane in induction
2. Low VT (6ml/kg)
3. Hi RR
4. optimal PEEP (3cmH2O)
5. adequate depth of anaesthesia
6. use of milrinone

Preserving RV function during pre-bypass by maintaining adequate preload, afterload and

monitoring RAP regularly). Periop use of milrinone.

Prevention of neurological risk a/w circ arrest: adequate perfusion pressure, glucose (avoid hypo- &
hyper-glycaemia)

Ultrafiltration is useful.

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POST-BYPASS

Platelets & cryoprecipitate are needed

Maintenance of hi perfusion pressures on CPB after AoXC removal can promote distal migration &
dissipation of coronary air emboli.

Separation from Bypass – Anticipate pulmonary hypertension!

1. Milrinone (LD 50 mcg/kg, 0.25-1.0 mcg/kg/min)
2. Adrenaline and/or Dopamine
3. Calcium (neonates are sensitive to disturbance of ionized Ca levels, following citrated blood transfusion. It is
frequently necessary to administer additional Ca while infusing blood/products)
4. GTN 1mcg/kg/min before cpb weaning to prevent surgical inflammation mediated coronary artery
spasm, cause vasodilatation of reimplanted coronary arteries; help control preload

Hemodynamic goals post CPB:

1. Adequate CO (by maintaining HR, contractility, preload, afterload) while maintaining the lowest possible LAP.
Systemic arterial desaturation could occur by ↓ CO.
2. BP shud b kept appropriate
3. Avoid Pulmonary Hypertension
 4. Careful fluid boluses – LV is noncompliant, LAP rise quickly, re-implanted coronaries can get stretched >>
coronary ischaemia & cardiac distension. Undesired hyper-volaemia with ↑ LAP can be corrected rapidly by
withdrawing blood (3-5 ml) from arterial or central catheter
5. LAP~ 6 mmHg with SBP 55-75 mmHg (avoid LA distension → after repair, PA is anterior to Ao and close to
origins of coronaries. LA distension → PA distension → compress the coronaries)
6. PPHTN ~ iNO / ecmo;

LV function is the primary concern after separation from CPB. LV is now ejecting thru the aorta instead of
PA, which cause an abrupt increase in afterload on LV.

LV distension & LA hypertension : particularly if there is MR

• Myocardial ischaemia (coz further LV dilatation, further ↑ LA & PA pressures, continuing compromise of coronary
blood flow)
• Afterload mismatch
• Excessive volume infusion → can cause systemic HTN, LA dilation, pulmonary edema, suture line bleeding, MR

LA line may be beneficial post repair in whom LV dysfunction is a concern (eg. TGA-IVS, long bypass time)

If repair is good → unusual to require significant inotropic support or pacing or experience ECG
abnormalities, dysrhythmias or AV block.( If need for AV pacing, occurrence of dysrhythmias, need for unexpected
degrees of vasoactive support (eg adrenaline) should lead to a search for technical problem)

SR is most common. Ischaemia-induced arrhythmias from coronary air or coronary artery stenosis, AVN injury
or any other causes can occasionally be seen. 1/4 pts with TGA-VSD-LVOTO (Rastelli) >> JET, SVT, AV block.

Echo/TEE & invasive pressures to assess repair & guide hemodynamics.

( Echo : deairing, wall motion, assessment presence of flow in proximal portions of reimplanted coronaries;
MR used as surrogates of coronary patency, pulmonary or systemic venous obstruction, atrial baffle leaks)

Signs of coronary ischaemia (typically ECG abnormalities : S-T/T wave changes, various dysryhtmias & forms of AV
block) should prompt the surgeon to carefully examine the coronary buttons & anastomosis. Also
ensure all intracardiac shunts are closed.

When the cardiorespiratory status is tenuous, the chest is left open, and rarely ECMO is required

Post CPB myocardial dysfunction:

1. Air in the coronaries
2. Inadequate coronary transfer (coronary stenosis, stretching, spasm or compression)
3. Poor myocardial protection
4. Deconditioned LV

Concern for Coronary anastomosis problems: (hence prompt echo)

1. Newly onset arrhythmias
2. ST segment abnormalities
3. MR
4. Worsening or refractory cardiogenic shock
Complications of ASO
1. Bleeding
2. Hypothermia
3. RV dysfunction, LV dysfunction & failure
4. Myocardial ischaemia
5. Residual structural defects
6. Dysrythmias (SVT); Sinus node dysfunction
7. PHT
8. obstruction to either pulmonary or systemic venous return

*Untrained LV*

Pt with inadequate LV mass for ASO (later age or hv other concurrent medical problems eg sepsis):

1. Preliminary PA banding to train the LV to tolerate higher pressures & increase LV mass, then ASO a
short time later. In young pts, LV training is rapid (<1 week). During that time, often requires
significant inotropic support & ICU care ( as LV training places an acute & significant increase in
afterload on ventricle). The LV & end organs must be fully recovered from low output state prior to
definitive ASO.
2. Perform ASO & provide L heart support postop (LVAD or ECMO)

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RASTELLI PROCEDURE

To correct certain combinations of :

1. d-TGA, or overriding aorta, or DORV and

2. VSD and
3. RVOTO
a. pulmonary atresia; or
b. pulmonary stenosis; or
c. subpulmonary stenosis.
4. TGA-VSD-LVOTO
{Procedure of choice for TGA-VSD and Pulmonary Atresia or significant PS.}

Typically performed between one and two years of age. Since d-TGA, overriding aorta, and DORV are cyanotic
heart defects, the child is palliated with a Blalock-Taussig shunt in the meantime.
Method: A Gore-Tex patch is used to direct oxygenated blood from the left ventricle to the aorta, while at the
same time closing the VSD. The pulmonary valve is surgically closed and an artificial conduit and valve are
constructed from the right ventricle to the pulmonary bifurcation, allowing oxygen depleted blood to travel to
the lungs for reoxygenation.

Result : LV to Ao continuity, RV to PA continuity w bypass of subpulmonic & pulmonic stenosis.

Incidence of arrhythmias is high after procedure – RBBB, AV block, even fatal ventricular dysrhythmias.