pediatric anethesia consideration for congenital heart disease pateints

1. ANESTHESIA FOR CHD
BIRHANU Y.(BSC,
MSC IN ANESTHESIA)

2. Outlines
Introduction to CHD
Classification of CHD
Acyanotic CHD
Cyanotic CHD

3. Introduction
CHD is defined as structural or functional
heart disease that is present at birth
It occurs in 0.5–0.8% of live births
The incidence is higher in abortus and still
births
 3-4/100 still born
10-25/100 abortuses

4. Etiology
Specific aetiology only known in 10%
8% genetic
2-4% environmental or adverse maternal
conditions (rubella, foetal-alcohol syndrome,
maternal DM)
90% Multifactorial inheritance
A small percentage is related to
chromosomal abnormalities
– Trisomy 21(50%) and Turner syndrome(40%)

5. The incidence & types of
CHD
Condition Incidence
VSD /Ventricular septal defect 32%
PDA/ Patent arterial duct 12%
PS /Pulmonary stenosis 8%
CoA /Coarctation of the aorta 6%
ASD /Atrial septal defect 6%
TOF /Tetralogy of Fallot 6%
AS/ Aortic stenosis 5%
TGA /Transposition of the great
arteries
5%
AVSD/ Atrioventricular septal
defects
2%

6. Recognizing Cardiac Disease in
Children
Recognition of the presence of heart disease
prior to surgery:
❖To treat cardiac failure prior to surgery
(VSD, AVSD)
❖To minimise the risk of air emboli through
abnormal shunts (TOF)
❖To ensure antibiotic prophylaxis is given
to children at risk of endocarditis

7. Recognizing Cardiac Disease in
Children
Children rarely present with the symptoms
classically associated with heart disease in
adults (chest pain, shortness of breath,
swollen ankles)
Rather they present with a variety of
symptoms such as failure to thrive, frequent
chest infections, or unexplained ‘funny
turns’
CXR,ECG, pulse oximetry, Cardiac
catheterization

8. Physiologic approach to
congenital heart disease
The defects are more complex, but can
be understood within limited physiologic
spectrum
Four categories
❖Shunt lesions
❖Mixing lesions
❖Flow obstructions
❖Regurgitate lesions

9. Classification of Congenital Heart
Defects
Physiologic
Classification
Pulmonary Blood Flow Comments
Left-to-right
shunts
VSD ↑
Volume-overloaded
ventricle
ASD Development of CHF
PDA
Right-to-left
shunts
Tetralogy of Fallot ↓
Pressure-overloaded
ventricle
Pulmonary
atresia/VSD
Cyanotic

10. Classification of Congenital Heart
Defects
Physiologic
Classification
Pulmonary Blood Flow Comments
Mixing lesions
Transposition/VSD
Generally ↓ but
variable
Variable pressure
versus volume loaded
Obstructive lesions
Critical aortic stenosis
Pressure-overloaded
ventricle
Critical pulmonic
stenosis
Ductal dependence
Coarctation of the
aorta
Mitral stenosis
Regurgitant lesions
Ebstein's anomaly
Volume-overloaded
ventricle

11. Classification
CHD
Acyanotic
Left-to-right
shunt
Outflow
obstruction
Cyanotic
11
 Ventricular Septal
Defect (VSD)
 Persistent Ductus
Arteriosus (PDA)
 Atrial Septal Defect
(ASD)
 Pulmonary
Stenosis
 Aortic Stenosis
 Coarctation of
aorta
 Tetralogy of Fallot
 transposition of
the great arteries

12. Clues for Evaluation of an Infant with
suspected CHD
1. On History and Physical Examination
(color)
• Acyanotic
• Cyanotic
2. Chest roentgenogram
• Normal
• Increased/Plethora
pulmonary blood flow
• Decreased/Oligemia

13. Clues for Evaluation of an Infant with
suspected CHD
3.Electrocardiogram
- Right
- Left
hypertrophy
- Biventricular
Final diagnosis - Precordial examination
- Echocardiography

14. Acyanotic
Left to Right Shunt

15. Acyanotic CHD
 Atrial Septal Defect
 Ventricular Septal Defect
 Persistent Ductus Arteriosus
 Pulmonary stenosis
 Aortic Stenosis
 Coarctation of the Aorta

16. Acyanotic (Left to Right
Shunt)
It occur when the PVR is lower than
the SVR
BF is preferentially directed toward the
lungs, resulting in increased PBF
In patients with large left-to-right shunts
and low PVR, a substantial increase in
PBF can occur

17. Atrial Septal Defect
Due to failure of septal growth or
excessive reabsorption of tissue
Defect occur in any portion of the atrium
- Ostium secundum (at fossa ovalis) (80%)
- Ostium primum (ECD) (lower atrial
septum)
- Sinus venosus (upper atrial septum)
Left to right shunt
- Transatrial in OS & SV
- Transatrial & transventricular in OP

18. Pathophysiology
 Shunting across an atrial septal defect is left to
right
 The degree of this shunting is dependent on;
- The size of the defect
- The relative vascular resistance in the
pulmonary and systemic circulations.
 Resistance in the pulmonary vascular bed is
commonly normal in children with ASD, and
increase in volume load is usually well tolerated
18

19. Pathophysiology
 However, altered ventricular compliance
with age can result in an increased left-to-
right shunt contributing to symptoms
 The chronic significant left-to-right shunt can
alter the pulmonary vascular resistance
leading to pulmonary arterial hypertension,
even reversal of shunt and Eisenmenger
syndrome 19

20. Clinical Manifestation
 Initially no symptoms, no physical finding.
 May remain undetected for years
 Large ASD, ratio more than 1.5,causes
dyspnea on exertion, supraventricular
arrhythmias, RHF and recurrent pulmonary
infections
 A systolic ejection murmur is present in 2nd
left intercostal space
 Mid-diastolic murmur at tricuspid area
20

21.  Diagnosis
 Clinical
 CXR - Right. V & A
enlargement
- Large pulm.
artery
- ↑ed pulm.
vascularity
 ECG - volume
overload,
- right axis
deviation
- minor right
ventricular conduction
delay
 Echocardiography
Documents type, size and
direction of shunt
 Prognosis - Well
tolerated
 Complications –
- pulm. Hypertension,
Eismenger syndrome
 Treatment
 Surgery
- For all symptomatic
ASD
- Rt. Cardiac Chamber
enlargement with /out
symptoms

23. Ventricular Septal Defect
 There is a defect anywhere in the ventricular
septum, usually perimembranous (adjacent to
the tricuspid valve) or muscular (completely
surrounded by muscle)
 The amount of flow crossing a VSD depends on
the size of defect and the pulmonary vascular
resistance

24. Ventricular Septal Defect
 At birth, the pulmonary vascular resistance is
normally elevated, thus, even large VSDs are
not symptomatic at birth
 Over the first 6-8 weeks of life, pulmonary
vascular resistance normally decreases more
blood flows through the lung and into the left
atrium
 However, in VSD, the amount of shunt
increases, and symptoms may start to develop
 The size of the VSD affects the clinical
presentation

25. Ventricular Septal Defect
 When blood passes through the VSD from the
left ventricle to the right ventricle  a larger
volume of blood handled by the right side of the
heart  extra blood then passes through the
pulmonary artery into the lungs  pulmonary
hypertension and pulmonary congestion 
pulmonary arteries become thickened and
obstructed due to increased pressure
 If VSD is not repaired, and lung disease begins
to occur  pressure in the right side of the heart
will eventually exceed pressure in the left  R to
L shunt  cyanosis (Eisenmenger complex)

26. Small defects with trivial Lt to Rt Shunt
- Most common
- Asymptomatic
- Loud, harsh holosystolic M at LLSB
 Large defects
- Excessive pulmonary blood flow
- Pulmonary hypertension
- Dyspnea, feeding difficulties, poor growth,
perspiration, recurrent plum. infection, heart
failure
- Less harsh but more blowing holosystolic murmur
- Accentuated 2nd heart sound
Clinical Manifestation

27. Diagnosis
- Clinical
- CXR - Cardiomegaly, Pulmonary edema
- Plethoric lung
- ECG - Biventricular hypertrophy by 2 months
of age and signs of pulmonary HPT right
ventricular enlargement and hypertrophy
- Echocardiography
Prognosis
- 30-50% small defects close by 2 yr of age
- Rarely moderate to large defects close

28.  Complications
- Infective endocarditis
- Recurrent lung infection
- Heart failure
- Pulmonary HTN
- Acquired pulmonary stenosis
- aortic valve regurgitation
 Treatment
- Small defects - reassurance
- Prophylaxis against IE
- Large defects - medical treatment
control of CHF,
promoting normal growth
prevent IE, prevent
development of p. HTN)
- Surgical repair between 6-12m

30.  Functional closure soon after birth
 The ductus arteriosus does not close after
delivery
 Aortic end of the ductus distal to the origin of left
subclavian artery and the other end at bifurcation
of pulmonary artery
 Male to female ratio 1:2
 Pathology – Deficiency of mucoid endothelial
Patent Ductus Arteriosus

31. Patent Ductus Arteriosus
 The ductus arteriosus allows blood to flow from
the pulmonary artery to the aorta during fetal life.
This changes to the opposite after birth.
 In term infants, it normally closes shortly after
birth.
 Failure of the normal closure of it by a month
post term is due to a defect in the constrictor
mechanism of the duct.
 In preterm infants, the PDA is not from CHD
but due to prematurity

32.  Pathophysiology
Lt to Rt shunt - size
- ratio of pulm. to systemic
resistance
Clinical Manifestation
Asymptomatic in small ductus
Wide pulse pressure
Bounding pulse
Continuous or machinery large
M at 2nd Left ICS

33. Diagnosis
- Clinical
- Chest X-ray
- ECG
- Echocardiography
Prognosis
- Small PDA -
normal life
- Large PDA - CHF
Complications
- Infective
Endocarditis
- CHF
- Pulmonary HTN
Treatment - Medical
- Surgical
closure

35. Pulmonary stenosis
 Narrowing of the
pulmonary valve
opening that increases
resistance to blood flow
from the right ventricle
to the pulmonary
arteries
35

36. Site: Valvular (most),
supravalvular, or
subvalvular
 The leaflets that are
partially fused together
 Three leaflets, but thick
and partly or completely
stuck together
Narrowing of the
valve
36

37. Cont.
37
Pulmonary valve is
mildly to moderately
narrowed
The right ventricle
pump harder and at a
higher pressure to
propel blood through
the valve
Right
ventricular
hypertrophy

38. severe stenosis in a neonate
Right ventricle cannot eject sufficient
volume of blood flow into the
pulmonary artery
Right ventricular pressure becomes
extremely high
Right-to-left shunt
cyanosis
Lead to right-to-left shunting through
a patent foramen ovale/atrial septal
defect
38

39. Clinical features
 Severity depend on degree of stenosis
 Most asymptomatic (mild)
 Moderate – Severe :
 Exertional dyspnoea, easily fatigability, rapid
breathing, shortness of breath, chest pain
(angina), cyanosis
 may develop as the child gets older.
39

40. Clinical features
Physical sign: heart murmur
– Sys ejection murmur best heard at 2nd IS
(P2) which radiates to the back
– Thrill may present
– In severe: impulse at the left sternal
border(RVH)
– Often associated with click sound
40

41. Diagnosis
- Clinical
- CXR - Rt vent. hypertrophy
- reduced pulm. blood flow
- ECG - RVH
- Echocardiography
Prognosis - good in mild to moderate
Complications - CHF in severe Ps
- rarely IE
Treatment - ballon valvoplasty
- surgery

43. Aortic stenosis
 A narrowing of the
valve that opens to
allow blood to flow
from the left
ventricle into the
aorta and then to
the body 43

44. Aortic stenosis
 Valvular, subvalvular
or supravulvalar – 5%
 Failure of :
 development of the
three leaflets
 Resorption of tissue
around the valve
44

45. Pathophysiology
Narrowed aortic valve
The LV must pump under very high
pressures
Left ventricular
hypertrophy
• Mild stenosis: usually well
tolerated, with minimal hypertrophy
and normal LV function
• Severe hypertrophy and valvar
obstruction: myocardial ischemia &
limited CO, reduced coronary perfusion,
with increased myocardial oxygen
consumption
• Fibrosis may occur in areas of the
myocardium damaged by ischemia

46. Clinical features
Depend on degree of stenosis
Mild to moderate : asymptomatic
Severe:
 Easy fatigability, exertional chest pain,
syncope
 In infant with severe stenosis can survive only
if:
-PDA permits flow to the aorta and coronary arteries
46

47. Physical signs
Small volume
Sys ejection murmur at Rt 2nd IS and
radiating to neck
Apical ejection click
Thrill at RUS border/suprasternal
notch/carotid
47

48. Diagnosis
- Clinical
- CXR - LVH, post stenotic dilation of
ascending or aortic knob
- ECG - LVH
- Echocardiography
Prognosis is good for mild to moderate
Treatment
- Balloon valvoplasty
- Surgical

52. Coarctation of the Aorta
 Narrowing of the aorta
 Can occur anywhere, but is most likely to
happen in the segment just after the aortic arch
 This narrowing restricts the amount of blood to
the lower part of the body
 Occurs in about 8-11 % of all children with CHD

53. Coarctation of the Aorta
EFFECTS:
 The left ventricle has to work harder to try to
move blood through the narrowing in the aorta
 left-sided heart failure
 BP is higher above the narrowing, and lower
below the narrowing
 Older children may have headaches from too
much pressure in the vessels in the head, or
cramps in the legs or abdomen from too little
blood flow in that region.
 The walls of the arteries may become weakened

54. Pathophysiology
54
Afterload on the left
ventricle (LV), which
results in increased wall
stress
LV hypertrophy
• Acute increased in afterload lead to rapid
development of CHF and shock
•These children may be asymptomatic
until hypertension is detected or another
complication develops

55.  Hypertension → mechanical obstruction
 Differential cyanosis → pink upper extr.
→ cyanosed lower
extr.
 Classic signs
- Disparity in pulse & BP
- Radio-femoral delay
- Systolic M at LMSB & inter-scapular
area
Clinical Manifestation

56. The aorta
narrows
Reduces blood
flow to the lower
half of the body
The BP is lower
than normal in the
legs and tends to
be higher than
normal in the
arms
56

57. Diagnosis
- Clinical
- CXR - cardiomegaly & pulm. congestion
- Notching of ribs
- ECG - LVH
- Echocardiography
Complications - CVA
- Aneurism
- stroke
Treatment
- Medical - IV PGE1 in neonatal age
- Surgery

59. Anesthetic Considerations for
Acyanotic Defects
GOAL: Decrease shunt & maintain adequate
oxygenation and perfusion
PreOp:
 How big is the shunt? (echo)
 Baseline cardiorespiratory status. Exercise
tolerance, Baseline VS, including SpO2

60. Anesthetic Considerations for
Acyanotic Defects
Induction:
 Potent intravenous and inhalational agents will
decrease SVR
 An inhalation induction is generally tolerable, if
necessary (i.e., peds).
 So VAA (dec. SVR) and PPV (Inc. PVR) are well
tolerated
 Patients with severe pulmonary HTN or RV
failure should have an IV induction
Theoretically
 left-to-right shunt may speed inhalation induction

61. Anesthetic Considerations for
Acyanotic Defects
IntraOp:
 Avoid acute & long-term increases in SVR or
decreases in PVR (worsens the left-to-right
shunt).
 Hypoxemia ,Acidosis
 IV bolus meperidine may increase PA pressures.
PostOp:
Drugs to decrease pulmonary HTN:
• Inhaled nitric oxide, prostacyclin, prostaglandin
I2,NTG,Nitroprusside

63. Cyanotic
Right to Left Shunt

64. Cyanotic
 Tetralogy of Fallot
1. Rt ventricular outflow obst.
2. Ventricular septal defect
3. Overriding aorta
4. Right ventricular hypertrophy
 Pulmonary Atresia
 Tricuspid atresia
 Transposition of GA
 Truncus arteriosus
 Single Ventricle

65. Cyanotic
 R - L shunts cause hypoxia and central cyanosis
 Venous blood is shunted from the R to the L side
of the heart w/o passing through the lungs to be
oxygenated
 Unoxygenated blood circulates in arteries 
cyanosis
What increases right-to-left shunt?
 Decrease in SVR and Increase in PVR
 Atresia: absence or closure of a natural
passage of the body

66.  A complex condition of several congenital defects
that occur due to abnormal devt. of the fetal heart
during the first 8 weeks of pregnancy
 Consists:
1. Rt ventricular outflow obst.
2. Ventricular septal defect
3. Overriding aorta = The aorta sits above both the left
and right ventricles over the VSD, rather than just over
the left ventricle. As a result, oxygen poor blood from
the right ventricle can flow directly into the aorta
instead of into the pulmonary artery to the lungs
4. Right ventricular hypertrophy = The muscle of the
right ventricle is thicker than usual because of having
Tetralogy of Fallot

67. EFFECTS:
 If the right ventricle obstruction is severe, or if
the pressure in the lungs is high  a large
amount of oxygen-poor (blue) blood passes
through the VSD, mixes with the oxygen-rich
(red) blood in the left ventricle, and is pumped
to the body  cyanosis
 The more blood that goes through the VSD,
the less blood that goes through the
pulmonary artery to the lungs  
oxygenated blood to the left side of the heart.
Tetralogy of Fallot

69. - Outflow obstruction
- Normal or small pulmonary valve annulus
- Rarely pulmonary atresia
- VSD - Non-restrictive, located just below
aortic valve
- Aortic arch is right side in 20%
- Right ventricular output shunts to the aorta
Pathophysiology

70. - Rarely pink TOF - in the absence of obstruction
- Cyanosis
- Clubbing
- Paroxysmal hypercyanotic attacks
 occur during 1st 2 years
- Systolic ejection M
- Delayed growth & development
- Single 2nd heart sound
Clinical Manifestation

71. Diagnosis
CXR - A boot or wooden shoe
- decreased pulm. vascularity
- Right side aortic arch in 20%
ECG
Echocardiography
Complication
- Cerebral thrombosis - in < 2 years
- Infective endocarditis
- Polycythemia
- CHF in pink TOF

72. Treatment
Severe outflow obstruction
- Medical Px - PGE1 infusion
- Prevent dehydration
- Oral propranolol can help
- Surgery- Total correction

75.  With VSD - Extreme form of TOF
 Without VSD - No egress of blood from Rt
vent.
- Shunt through foramen ovale to Lt
atrium
Left ventricle
systemic circulation
Aorta
pulmonic
circulation(PDA)
- Hypoplastic right ventricle (PDA)
Pulmonary Atresia

76. Clinical Manifestation
- Cyanosis at birth
- Respiratory distress
- Single 2nd heart sound
- No murmur
Diagnosis
- CXR
- ECG
- Echocardiography
Treatment - PGE1
- Surgery

78. No outlet from Right atrium to right vent.
Systemic venous return
Rt atrium
Lt atrium
Left ventricule
systemic Pulmonic
(VSD, PDA)
Tricuspid atresia

79. Clinical Manifestation
- Cyanosis at birth
- Polycythemia
- Easily fatiguability
- Exertional dyspnea
Diagnosis
- EXR -Pulm. Under
circulation
- ECG -Lt axis
deviation & Lt vent.
hypertrophy
- Echocardiography
Treatment
- PGE1
- Surgery

81. TGA
- Systemic venous return to Rt atrium
Normal
- Pulmonary venous return to Lt atrium
- Aorta arise from left Vent.
- Pulmonary artery arise from Rt ven.
Normal
- Aorta arises from Right ventricle
- Pulm. artery arises from Lt vent. Pathology
Transposition of GA

82.  Systemic & Pulmonary Circulations Consists of two
parallel circuits
 Survival is with associated - patent foramen ovale
or
- VSD or
- PDA
Clinical Manifestations
- Tachypnea & cyanosis at birth
- Rarely congestive heart failure
Transposition of GA

83. Diagnosis
- Clinical
- CXR - Cardiomegaly
- Narrow mediastinum
- Increased pulmonary blood flow
- ECG
- Echocardiography
Treatment
- PGE1 - emergency
- Surgery

85.  Single arterial trunk for both pulmonary &
systemic circulation
Clinical Manifestation
- Cyanosis
- CHF at 2-3rd m
- Systalic ejection m
Treatment - surgery
Truncus arteriosus

87.  No inter-ventricular septum
Single Ventricle

88. Persistent fetal circulation

89. Anesthetic Considerations for
Cyanotic Defects
 PreOp:
Avoid preoperative dehydration (esp. with TOF)
 Preop admission for overnight hydration may be
necessary.
 Induction:
 Maintain SVR>PVR to reduce right-to-left shunt
Ketamine may maintain SVR
An inhalation induction is generally tolerable.
 Theoretically
 Right-to-left shunt may dilute the inhaled anesthetic
agent in the LV, decreasing the amount of IA

90. Anesthetic Considerations for
Cyanotic Defects
IntraOp:
Maintain SVR , a decrease in SVR and/or
an increase in PVR worsens shunt and
hypoxia
Avoid excessive positive airway pressure
and excessive PEEP in patients with
decreased pulmonary flow (TOF, pulmonary
stenosis), further decrease flow

91. Anesthetic Considerations for
Cyanotic Defects
PostOp:
 Adequate analgesia without sedation-
induced hypercapnia
 Pain yields sympathetic stimulations which
PVR
 Over-sedation yields hypercapnia which 
PVR

93. THANK YOU!!!