1) Congenital heart defects are the most common birth defects, affecting 1 in 125 live births. They range from simple shunt lesions to complex defects involving multiple structures. 2) The anesthetic goals vary depending on the type of shunt (left-to-right vs right-to-left) and aim to balance systemic and pulmonary vascular resistances. 3) Preoperative evaluation and optimization is important. Regional techniques may be used when hemodynamically appropriate but general anesthesia allows better control of ventilation and hemodynamics for high risk surgery.

1. SPEAKER: DR V.SRAVANI
MODERATOR: DR. ANNAPURNA SHARMA
PROFESSOR
DEPARTMENT OF ANAESTHESIOLOGY, MIMS

4. SHUNT CLOSURE
Foramen ovale Functional closure immediately after
birth.
Anatomical closure with in 10 days.
Ductus arteriosus Functional closure with in 24hrs after
birth.
Anatomical closure with in 3months
after birth.
Ductus venosus Immediately after birth.

5. COMMONEST BIRTH DEFECT
1 IN 125 LIVE BIRTHS
30% OF CHILDREN HAVE EXTRA CARDIAC ANOMALIES
▪ Tracheoesophageal fistula
▪ Cleft lip and palate
▪ Anorectal anomalies
▪ Skeletal anomalies

6.  Types of congenital heart disease
 Physiology of different lesions
 Preoperative assessment
 Anesthetic management

7. Simple left to right shunt lesions
 Atrial septal defect (ASD)
 Ventricular septal defect (VSD)
 Atrioventricular septal defect
 Patent ductus arteriosus (PDA)
Simple right to left shunt lesions
 Tetrology of Fallot (TOF)
 Pulmonary atresia
 Tricuspid atresia
 Ebstein’s anomaly
Complex shunts
a. Transposition of the great
arteries
b. Truncus arteriosus
c. Total anomalous pulmonary
venous drainage
d. Hypoplastic left heart syndrome
Obstructive lesions
a. Coarctation of the aorta
b. Aortic stenosis
c. Pulmonary stenosis

9.  Truncus arteriosus
 TGA
 TAPVR
 HLHS

10. Shunting A) R → L shunt
L → R shunt
B) Simple shunts
Complex shunt
Pulmonary blood flow A) Increased
B) Decreased

11. ✓ Intra cardiac connections between the chambers of the
heart or extra cardiac connections between a systemic and
pulmonary artery.
✓ The direction of blood flow through a shunt is dependent
on:
➢ the size of the shunt orifice
➢ the pressure difference between the chambers
➢ the relative resistances on either side of the shunt.

12. PVR >SVR R → L shunt (reducing PBF)
Mixing of venous blood into systemic blood
Hypoxaemia, Cyanosis
Increased CO & Hb concentration
Volume and pressure load on heart

13. L →R shunt
Pulmonary blood flow
Structural alteration in
pulmonary vasculature
PAH

14. PBF Increase in airway
resistance and decrease in
pulmonary complaince
Structural alterations
in pulmonary
vasculature
Increased
PAH

15. PBF Significant abnormalities in
pulmonary arterial tree
Increased PVR
Polycythaemia
Increased blood flow
Alveolar
hyperventilation
Aorto-pulmonary
collaterals

16. COMPLEX SHUNTS OR MIXING LESIONS
✓ In these defects, the mixing between the pulmonary and the
systemic circulation is so large that the systemic and pulmonary
artery oxygen saturations approach each other.
✓ The pulmonary to systemic flow ratio (Qp/Qs ratio) is independent of
shunt size and totally dependent on vascular resistance or outflow
obstruction.

17. II. In critical neonatal right-sided heart
obstructive lesions include
a) right ventricular dysfunction
b) decreased PBF
c) systemic hypoxemia
d) PDA – dependent PBF..
OBSTRUCTIVE LESIONS
✓ left and right ventricular outflow tract obstructions which produce
a pressure overload on the corresponding ventricle

19. CARDIAC SEQUELAE
 Pulmonary hypertension
 Ventricular dysfunction
 Dysrhythmias and
conduction defects
 Residual shunts
 Valvular lesions-regurgitation
or stenosis
 Hypertension
 Aneurysms
NON-CARDIAC SEQUELAE
 Secondary erythrocytosis
 Cholelithiasis
 Nephrolithiasis
 Developmental abnormalities
 Seizure disorders from previous
thromboembolic events or
cerebrovascular accidents
 Restrictive and obstructive lung
disease

20. ▪ Should be thoroughly familiar with the child’s cardiac anatomy and
pathophysiology.
▪ Valuable information can be obtained by discussion with the child’s
cardiologist.
▪ A history of either exercise intolerance in the older child or feeding
intolerance in the infant are reliable symptoms of cardiopulmonary
decompensation.
▪ The presence of even mild upper respiratory infection is a
contraindication to elective surgery

21. ▪ History of cyanotic spells and triggering events, episodes of
unconsciousness or convulsions
▪ Any additional systemic disease and noncardiac anomalies
Preoperative fasting guidelines:
▪ The avoidance of preoperative dehydration is important in
children with cyanotic congenital heart disease (especially
when Hb > 18g/dl). Fasting guidelines will be dictated by the
age of the child and the nature of the surgery

22.  Regional may produce unacceptable decreases in SVR and
could exacerbate Right to left shunt.
 General anaesthesia allows for optimal control of ventilation
and may be preferable in patients with high risk surgery.

23.  advantages of preoperative sedation easy separation from parents, less
crying, decreased oxygen consumption and decreased levels of
intraoperative anesthetic requirements.
 The disadvantage of sedative premedication is respiratory depression with
desaturation .
 administered in a controlled environment with monitoring of vitals.
 midazolam orally or nasally.
 Oral and intranasal ketamine
 Cardiac premedication like antiarrhythmics, beta blockers and diuretics
should be continued preoperatively

24.  History of endocarditis
 Prosthetic heart valve (prosthetic material used for valve repair)
 Status post heart transplant with valvulopathy
 Congenital heart disease-associated conditions:
 Unrepaired cyanotic congenital heart lesion (including palliative shunts and
conduits)
 Completely repaired congenital heart lesion, during the first 6 months after
the procedure (if prosthetic materials or device were used)
 Repaired congenital heart lesion with a residual defect (at or adjacent to the
site of a prosthetic patch or prosthetic device)

25.  Inhalational induction is generally well tolerated by children with minor cardiac
defects.
 Sevoflurane provides better cardiovascular stability compared to halothane and has
less myocardial depressant and arrhythmogenic properties.
 Intravenous induction is usually the preferred method in severe cardiovascular
limitation.
 well compensated chd thiopental or propofol in conjunction with an opioid well.
 Etomidate may have less hemodynamic side effects and can be used for children
with limited reserve. Ketamine as sole induction agent may be advantageous when
preservation of heart rate, blood pressure and ejection fraction seems to be
important, that is, in cyanosed children or those with congestive cardiac failure

26.  Pancuronium – tachycardia & hypertension
This effect is desirable to support CO in infants with CHF
where SV is relatively fixed
 Atracurium , vecuronium → bradycardia

27. THE ANESTHETIC GOALS AND CONCERNS
 In children with left to right shunt:
avoid any fall in PVR or increase in SVR that would increase the
shunt flow and precipitate congestive heart failure and pulmonary
edema.
▪ In children with TOF, any fall in SVR or anything which precipitates
infundibular spasm should be avoided.
▪ In right to left shunts, any rise in PVR or fall in SVR increases the R – L
shunt and worsens hypoxemia and should be avoided

28. L – R SHUNTS
 Decrease SVR
 Increase PVR
R – L SHUNTS
 Increase SVR
 Decrease PVR
 Adequate hydration

29. Factors that decrease PVR
 High FiO2
 Hypocapnea
 Respiratory alkalosis
 Low hematocrit
Factors that increase SVR
 Light planes of anesthesia
 Vasoconstrictors
Factors that increase PVR
 Hypoxia
 Hypercapnia
 Acidosis
 High airway pressures ,PEEP
 High hematocrit
 Inadequate anesthesia
 Hypothermia
Factors that decrease SVR
▪ Anesthetic agents which
cause hypotension
▪ Hypovolemia

30. PVR
R→L shunt
Arterial desaturation &
increased stress
on RV RVF
PVR
L→R shunt
Increased PBF &
Decreased systemic flow
Hypotension & acidosis

31.  Nitroglycerine
 Sodium nitroprusside
 Milrinone
 Sildenafil
 Prostaglandins
 Levosimendan
 Adenosine infusion (50mcg/kg/min)
 Bosentan
 BNP
 NO
 Avoid Hypoxia, Hypercarbia, Acidosis, Hypothermia,
Hyper inflation, sympathetic stimulation
 Pain management & careful airway manipulation

32.  Phenylephrine
 Norepinephrine
 Manual external compression of abdominal aorta / axillary
artery

33.  Principle 1: The Presence or Absence of Cyanosis
 Principle 2: The Presence or Absence of Intracardiac or
Extracardiac Shunts.
 principle 3: The Presence of Pulmonary Hypertension
 Principle 4: The Presence of Ventricular Dysfunction

34. ➢ Adequate pain relief
▪ Pain increases SVR and PVR
▪ Pain worsens infundibular spasm of TOF
➢ Extubate only when fully awake
▪ No respiratory depression.
➢ Use regional analgesia wherever applicable
▪ Caution in cyanotics (coagulopathy)
▪ Can decrease SpO2 in R → L shunts
▪ Hazardous in left sided obstructive lesions

35.  NPO
 IE prophylaxis
 Inhalational or IV, but slow and
cautious induction
 Monitoring
 Avoid air bubbles and N2O
 Avoid decrease in PVR and
increase in SVR
 Extubate when awake

36. a. With PDA ligation ,the aortic leak is closed
Most danger complication – profound haemorrhage due to
rupture of ligation

37.  Blalock-Tausing shunt (subclavian artery to pulmonary artery)
 Waterson shunt (ascending aorta to right pulmonary artery,
direct)
 Central shunt ( ascending aorta to main pulmonary artery tube
graft)
 The anaesthesia consideration for the defects with RL shunts
will apply

38.  3 types:
Ostium secundum- deficiency in septum primum
Ostium primum- deficiency inendocardial cushion
Sinus venous defect- at cavo-atrial junction
 Pathophysiology :
RV is thin walled &
more compliant
L→R shunt
PBF

39.  Closure of defect :
Percutaneous transcatheter techniques
Direct surgical repair with suture /patch
 Anaesthesia: lower doses of opiods (fentanyl-5mcg/kg) an
inhalational based anaesthetic technique (or) propofol
infusion
 Extubated immediately orwith in 2-3hrs
 Post-op period:
Supraventricular arrhythmias
Atrial flutter
Atrial fibrillation
Nodal rhythm

40.  Deficiency in ventricular septum
 Pathophysiology:
a) Small defect
b) Moderate defect
c) Large defect
When PVR>>SVR shunt may
become R→L (EISENMENGER
SYNDROME)

41.  Anaesthesia:
NPO
IE prophylaxis
Inhalational or IV, but slow and cautious induction
Avoid air bubbles and N2O
Avoid decrease in PVR and increase in SVR
Extubate when awake
 Post-op period:
Prolonged ventilation
Inotropic support & measures to decrease PVR
Intraop & postop pacemaker support

42. 1) VSD
2) RV outflow obstruction
3) Overriding of aorta
4) Hypertrophy of RV

43.  Surgical procedures: patch closure of VSD through a right
ventriculotomy & outflow enlarged by pericardial or synthetic
patch
 Anaesthesia:
Adequate premedication reduce the chances of hypercyanotic
spells
Goals: to maintain SVR
minimize PVR
provide mild myocardial depression

44.  An opiod based anaesthetic technique with midazolam or
inhalational agent is generally employed
 Glycopyrrolate , ketamine , vecuronium, Sevoflurane
 Post op: RV function may be suboptimal
RBBB/ complete heart block
Junctional ectopic tachycardia

45.  All the pulmonary venous
blood enters a systemic
venous structure due to
anatomical abnormality of
pulmonary veins

46.  Anaesthesia :
Avoidance of myocardial depressants, inotropic support
for RV function and maneuvers for decreasing PVR
 Postop:
Inotropic support
Prolonged ventilation

47.  Aorta arises from RV and PA originates from LV
 The systemic venous blood returns to RA & RV and again
pumped into aorta
 The oxygenated plumonary venous blood returns to LA &
LV and back to PA
 A parallel R & L circulations across the atria or ventricular
septum or through the ductus arteriosus is required for
survival therefore preoperative management of these
neonates includes infusion of PGE1 that helps to maintain
patency of PDA
 In addition , balloon atrial septostomy ( echo guided /
fluroscopy guided) can also be performed

48.  Six repairs:
a. atrial switch (mustard & senning) operation
b. arterial switch: procedure of choice for TGA
c. rastelli repair
▪ ANAESTHESIA:
Neonates can be intubated awake and oxygenated with
100% oxygen IV line is secured, arterial, central venous
lines are secured Low dose fentanyl, thiopentone,
atracurium, isoflurane
▪ Post operative period:
Inotropic support
Mechanical ventilation for prolonged duration

49.  Imperforate tricuspid valve with hypoplasia of RV for the survival of
the infant, an ASD/PFD is essential to allow the circulation of
systemic venous blood from the RA to the left side
 Due to shunting of systemic venous bl0od into the LA, hypoxaemia
and cyanosis are present
 Sx: Fontan procedure
 ANAESTHESIA: aimed at minimizing the PVR and optimising CO so
that blood flow through the lungs is improved patient should be
extubated as early as possible ,provided haemodynamics are stable