2. Classification of congenital heart
diseases
Group I : Left to right shunts
Group II: Right to left shunts
Group III: Obstructive lesions
3. Left to right shunts
• Atrial Septal Defect
• Ventricular Septal Defect
• Patent Ductus Arteriosus
4. Right to Left Shunts
1) Tetralogy of Fallot
2) Tricuspid atresia
3) Ebstein’s anomaly
4) Transposition of Great Vessels
5) Truncus Arteriosus
6) Total Anomalous Pulmonary Venous
Return (TAPVR)
7. Cyanosis
• Bluish discoloration of
skin and mucous
membranes
• Noticeable when the
concentration of deoxy-
hemoglobin is at least
5g/dl
• (O2 sat < 85%)
8. Cyanosis:
peripheral vs central
peripheral
• Sluggish blow flow
in capillaries
• Involves extremities
(acrocyanosis)
• Spares trunk &
mucous membranes
central
• Abnl of lungs or
heart that interferes
w/ O2 transport
• Involves trunk &
mucous membranes
9. Evaluation of cyanosis: 100% O2
test
measure pAO2 in roomair and 100%
O2
Lung disease:
1. Room air pAO2 30
mmHg O2 sat=60%
2. 100% O2 pAO2 110
mmHg O2 sat=100%
Cardiac disease:
1. Room air pAO2 30
mmHg O2 sat=60%
2. 100% O2 pAO2 40
mmHg O2 sat=75%
pAO2 >100 mmHg suggests lung disease
Little or no change in pAO2 suggests cyanotic heart disease
11. Prostaglandin (PGE1)
• Stable derivative of endogenous compound
that maintains ductal patency in utero
• Prevents postnatal ductal closure, improves
pulmonary blood flow, improves oxygenation
• Stabilizes cyanotic neonate so that corrective
surgery can be performed “electively”
• PGE1 revolutionized the fields of pediatric
cardiology and cardiovascular surgery
12. The basis of congenital heart
disease is rooted in
an arrest of or deviation in
normal cardiac development
13. The 5 T’s of cyanotic heart
disease
• Tetralogy of Fallot
• TGA (d-transposition of the great arteries)
• Truncus arteriosus
• Total anomalous pulmonary venous return
• Tricuspid atresia / single ventricle
• Pulmonary atresia
• Ebstein’s malformation of tricuspid valve
16. Tetralogy of Fallot
• 6 % of all congenital heart
disease
• 1:3600 live births
• most common cause of
cyanosis in
infancy/childhood
• Severity of cyanosis
proportional to severity of
RVOT obstruction
RV
17. T of F occurs as a result of abnormal
cardiac septation
Normal developmentAbnormal development
18. Tetralogy of Fallot
• Anterior
deviation of the
outlet ventricular
septum is the
cause of all four
abnormalities
seen in tetralogy
of Fallot.
19. Tetralogy of Fallot - CXR
• Typical “boot-shaped”
heart secondary to RVH
and small main
pulmonary artery
segment
•Pulmonary vascular
markings are decreased
20. • Coeur en sabot
(boot-shaped heart)
secondary to uplifting
of the cardiac apex
from RVH
and the absence of a
normal main pulmonary
artery segment
21. Hypercyanotic “tet” spell
• Paroxysmal hypoxemia due to
acute change in balance
between PVR and SVR
• SVR causes an increase in
R L shunt, increasing
cyanosis
• SVR (hot bath, fever,
exercise)
• Agitation dynamic
subpulmonic obstruction
• Life-threatening if untreated
22. Management of “tet” spell:
goal is to SVR and PVR
1. Knee-chest position ( SVR
2. Supplemental O2
3. Fluid bolus i.v. ( SVR)
4. Morphine i.v. ( agitation,
dynamic RVOT obstruction
5. NaHCO3 to correct
metabolic acidosis ( PVR)
6. Phenylephrine to SVR
7. b-blocker to dynamic
RVOT obstruction
26. Definitive operation
• Closing the VSD
• Resecting infundibular
• 90% can return almost normal life after operation
• Complication:
– RBBB
– Residual VSD
– Residual Pulmonary stenosis
– Pulmonary regurgitation (pulmonary valve excised)
– Risk 5%
27. D-Transposition
of the Great
Arteries
• Ao is anterior, arises from
right ventricle
• PA posterior, arises from
left ventricle
• Systemic venous (blue)
blood returns to RV and
is ejected into aorta
• Pulm venous (red) blood
returns to LV and is
ejected into PA
RV LV
PAAo
28. RV
Ao
PA
RA LA
D-transposition of great arteries
• 5% of all congenital
heart disease
• Most common cause
of cyanosis in
neonate
• Male:female 2:1
29. d-TGA results from abnormal
formation of aortico-pulmonary
septum
LV
RV
LARA
truncus
30. TGA
• Survival is dependent on the presence of mixing
between the pulmonary and systemic circulation
• Atrial septal defect is essential for survival
• 50% of patients have a VSD
• Usually presents in the first day of life with
profound cyanosis
• More common in boys
31. D-transposition of great arteries
• Systemic and pulmonary
circulations are in
parallel, rather than in
series
• Mixing occurs at atrial
and ductal levels
• Severe, life-threatening
hypoxemia
32. • Narrow
mediastinum due to
anterior-posterior
orientation of great
arteries and small
thymus
• Cardiomegaly is
present w/ increased
pulmonary vascular
markings
d-TGA CXR: “egg on a string”
33. Initial management of d-TGA:
goal is to improve mixing
1. Start PGE1 to prevent ductal closure
2. Open atrial septum to improve mixing
at atrial level (Rashkind procedure).
35. Surgical management of d-TGA:
Arterial switch procedure
The arterial trunks are transected and
“switched” to restore “normal” anatomy
The coronary arteries are resected and re-implanted.
36. Truncus arteriosus
• Aorta, pulmonary
arteries, and coronary
arteries arise from single
vessel.
• Truncus sits over large
ventricular septal defect.
• Failure of septation of
embryonic truncus.
• Uncommon (1.4% of
CHD)
38. Truncus Arteriosus
• The presence of a
common trunk that
supply the systemic,
pulmonary and
coronary circulation
• Almost always
associated with VSD
40. Truncus Arteriosus
• Generally patients have increased
pulmonary blood flow
• Degree of cyanosis is mild and may not be
evident clinically until late stage with
pulmonary vascular disease
• Presenting feature is congestive heart
failure (tachypnia, hepatomegally)
CXR : Cardiomegaly , increased pulmonary circulation
41. Managment
• Acute management
– No O2 to minimize pulmonary blood flow
– Diuretics
– Afterload reduction to inhance systemic blood flow
•Surgical management: complete
repair with VSD closure and
conduit placement between the
right ventricle and pulmonary
arteries
•Long term problems :
–truncal valve dysfunction
–RV conduit obstruction
43. Total anomalous pulmonary venous
return (TAPVR)
• Failure of pulm veins
(PV) to fuse with
developing left atrium
• PV drainage occurs
thru embryological
remnants of systemic
veins
• Incidence: rare
44. Venous connections in TAPVR
Supracardiac:
Ascending vertical vein
most common
Cardiac:
RA or coronary sinus
Infracardiac:
Descending
vein to portal
system
supracardiac cardiac
infracardiac
45. Clinical manifestation of TAPVR
Obstructed
• Severe pulmonary
edema, cyanosis,
shock
• Surgical emergency
Unobstructed
• Mild to moderate
congestive heart
failure and cyanosis
• Surgery in first 6
mo.
48. Supracardiac TAPVC - CXR
“Snowman” appearance
secondary to dilated
vertical vein, innominate
vein and right superior
vena cava draining all
the pulmonary venous
blood
52. Tricuspid Atresia
• Complete absence of
communication
between the right
atrium and right
ventricle
• About 3 % of
congenital heart
disease
53. Tricuspid Atresia
• There is an obligate interatrial communication
• Usually associated with VSD
• The pulmonary blood flow is dependent on the
size of the VSD
• Pulmonary blood flow can be increased or
decreased causing variable presenting symptoms
• If there is no VSD ( also called Hypoplastic right
ventricle) the pulmonary blood flow is
dependent on the PDA
54. Tricuspid Atresia- presentation
• The presentation will depend on the
amount of pulmonary blood flow
– If the PBF is decreased, the main presenting
symptom is cyanosis
– If the PBF is increased, the presentation is
that of congestive heart failure
• CXR will also reflect the amount of
pulmonary blood flow
56. Surgical Management
Single ventricle paliation
• First stage : to establish a reliable source of
PBF
– Aorta to pulmonary artery shunt ( BT shunt)
– Pulmonary arterial banding in cases of increased
PBF
• Second stage: Glenn Anastomosis ( superior
vena cava to pulmonary artery
• Third stage : Fontan anastomosis ( Inferior
vena cava to pulmonary artery
57. Pulmonary atresia
• Atretic pulmonary
valve
• Pulmonary arteries
often normal in size
• hypoplastic RV,
RVH
• hypoplastic TV
58. • Ductal-dependent lesion
• Requires PGE1 to maintain oxygenation
• Therapy directed at opening atretic valve
in cath lab or surgery
• Prognosis depends upon size and
compliance of hypoplastic RV
Pulmonary atresia
59. Ebstein’s malformation of tricuspid
valve
• TV leaflets attach to RV wall, rather than TV annulus
• Tethered leaflets create 2 chambers w/in RV: large
“atrialized” RV, small noncompliant functional RV
• Severe tricuspid regurgitation
60. Ebstein’s malformation of TV
• Massive RA dilation due
to severe tricuspid
regurgitation
• R to L shunt at atrial
level causes cyanosis
• Degree of cyanosis
related to size and
compliance of functional
RV
• Cyanosis usually
decreases as PVR falls
shortly after birth
