2. INTRODUCTION:
Tricuspid atresia :
complete agenesis of
tricuspid valve without
any direct communication
b/w RA & RV
3% of CHD
3rd most common cause
of cyanotic CHD
An ASD or VSD is always
present
Extra cardiac anomalies
present in 20%
3. ETIOLOGY:
Almost always congenital Characterized by:
Small RV
Large LV
Small VSD &PDA
Diminished pulmonary
circulation
cyanosis
4. PATHOPYSIOLOGY:
Due to tricuspid agenesis, the blood cannot flow
from the RA to the RV.Blood ultimately cannot enter
the lungs.
Instead, the blood passes through ASD between
the RA &LA. In the LA, it mixes with oxygenated
blood returning from the lungs. This mix of
oxygenated and de-oxygenated blood is then
pumped out into the body. This causes oxygen level
in the blood to be lower than normal.
The lungs receive blood either through a VSD
between the right and left ventricles, or through
ductus arteriosus that connects the pulmonary
artery to the aorta.
5.
6. MANAGEMENT:
Primarily surgical
Either an operation to increase pulmonary blood
flow (shunt)
Or to decrease pulmonary blood flow (pulmonary
artery band)
Fontan’s procedure
7. ANESTHETIC GOALS:
Anesthetic management is very challenging
Profound cyanosis may impair non invasive
monitoring of oxygen saturation
Nitrous oxide or sevoflorane used for inhalational
induction followed by a change to narcotics based
anesthetic once intravenous access established
MAP must be kept up to maintain shunt patency
Careful rehydration necessary despite fluid deficit
preoperatively
Maintain the hemostasis of PVR to SVR ratio &
ventricular performance
9. Early tracheal extubation & spontaneous ventilation
are desirable
Positive inotropic drugs (dopamine) with or without
vasodilators (nitroprusside)are often required to
optimize cardiac output & maintain low PVR
Monitoring the CVP to assess the intravascular fluid
volume & to detect sudden impairment of LV
function & increased PVR
Peak & mean airway pressure must be maintained.
10. TRANSPOSITION OF GREAT ARTERIES:
5% of CHD
10% of all neonatal cyanotic CHD
Failure of truncus arteriosus to go spiral so that
aorta arises from the anterior portion of RV & the
pulmonary artery from LV
11.
12. PATHOPHYSIOLOGY:
Due to complete seperation of pulmonary &
systemic circulation , systemic venous blood
traverses RA,RV, aorta & systemic circulation
Pulmonary venous blood traverses LA,LV,
pulmonary arteries & lungs
Circulation is parallel instead of normal in series
circulation
Survival is posible only if there is communication
between the 2 circulations in the form of a
VSD,ASD or PDA
13. SIGNS & SYMPTOMS:
Cyanosis (in the 1st week of life)
Tachypnea
Respiratory distress
Non specific systolic ejection murmur
Congestive heart failure (tachypnea, tachycardia,
sweating & poor feeding)
Right axis deviation &RV hypertrophy on ECG
Cardiac shadow “egg shaped with a narrow stalk”
on CXR
14. TREATMENT:
Immediate management involves creating
intracardiac mixing or increasing the degree of
mixing
Infusions of PG E1 to maintain patency of DA
Ballon atrial septostomy
Oxygen administration to decrease PVR & increase
pulmonary blood flow
Diuretics & digoxin for CHF
Mustard’s/ senning’soperation(venous switch)
Arterial switch procedure
15. ANESTHETIC MANAGEMENT:
Doses & rates of IV administered drugs have to
decreases(distribution of drugs with minimal dilution
to heart & brain)
Onset of inhaled anesthesia delayed(small amount
reaches systemic circ.)
Induction & maintenance are accomplished with
ketamine combined with muscle relaxants to
facilitate tracheal intubation
Ketamine can be supplemented with opiods or
benzodiazepines for maintenance
Nitrous oxide-limited application
16. Potential cardio depressant effects of volatile
anesthetics– not used
Selection of muscle relaxant to avoid histamine-
induced changes in systemic BP----- Pancuronium
used(inc. HR & systemic BP)
Dehydration must be avoided during perioperative
period(inc. hematocrit--- inc incidence of cerebral
venous thrombosis)
Prophylaxis for thrombosis & dysarhythmias