1. Congenital cyanotic heart disease refers to heart defects present at birth that result in low oxygen levels in the blood. They are classified as those with increased or decreased blood flow to the lungs. 2. Common types with increased pulmonary blood flow include persistent truncus arteriosus and transposition of the great arteries (TGA). Common types with decreased pulmonary blood flow include tricuspid atresia and pulmonary atresia with intact ventricular septum. 3. TGA accounts for 5-7% of congenital heart defects. It involves the aorta originating from the right ventricle and the pulmonary artery originating from the left ventricle. It requires mixing of blood between the pulmonary and systemic

1. CONGENITAL CYANOTIC HEART
DISEASE
DR.D.Rasikapriya

2. OBJECTIVES
• Introduction
• Clasification
• Common cyanotic heart disease

3. INDRODUCTION
• Most manifest during neonatal period and
requiring a correct diagnosis for appropriate
management.
• With increased pulmonary blood flow
• With decreased pulmonary blood flow

4. With increased pulmonary flow
• Persistent truncus arteriosus
• TGA
• TAPVD
• Univentricular heart
• Common atrium
• TOF with pulmonary atresia with increased
collateral blood flow

5. With decreased pulmonary flow
• Tricuspid atresia
• Pulmonary atresia with intact ventricular
septum
• Ebstein’s anomaly
• TOF
• Critical pulmonary stenosis

6. TRANSPOSITION OF GREAT ARTERIES
• Aorta originating from
the
right ventricle, and
pulmonary artery
originating from the left
ventricle
Accounts for 5-7% of all
congenital heart
disease

8. CONTD..
• In TGA aorta is anterior
• Survival is dependent on the presence of mixing
between the pulmonary and systemic circulation
ASD is essential for survival
50% of patients have a VSD
• More common in boys
• Presents with cyanosis and tachypnea within first
few hours or days of life
• Severity inversely proportional to presence of
shunts

9. TGA variants
TGA with intact Interventricular septum
• TGA with VSD
• TGA with VSD and LVOT obstruction
(Pulmonary stenosis)

10. Exam :
• cyanosis in an otherwise healthy looking baby
• Loud S2 ( aorta is anterior )
CXR :
• Egg on side
• Narrow mediastinum

11. ECHO

12. TREATMENT
PGE1 (start with 0.05-0.1mcg/kg/min,once the
desired effect is achieved the dose is gradually
reduced to 0.01mics/kg/min)) to maintain PDA
• Rashkind balloon atrial septostomy in severely
hypoxic or acidotic infants
• Arterial switch procedure (Jatene) performed as
early as first 2 weeks of life
• Rastelli operation another alternative
• Previously, atrial switch operations were done by
creating atrial baffles

13. TOTAL ANOMALOUS PULMONARY VENOUS
CONNECTION
• 2-3% of CHD
• Male (4:1 ratio)
• PATHOLOGY:
NO direct communication exists between the
pulmonary veins/LA.
Drain into systemic venous tributaries/RA.
• 4 TYPES :
Depending on drainage site of pulmonary veins.
1.Supracardiac
2.Cardiac
3.Infracardiac
4. Mixed

15. CONTENTS NON OBSTRUCTIVE OBSTRUCTIVE
HISTORY CHF with growth retardation
Frequent pulmonary infection
History of mild cyanosis
Marked cyanosis
Respiratory distress in
neonatal period with
failure.
Cyanosis worsen with
feeding.
PHYSICAL
EXAMINATION
Undernourished
Mildly cyanotic
Precordial bulge and hyperactive
RV impulse.
Quadruple rhythm.
S2- wide split , fixed
Systolic murmur-2 to 3(upper left
sternal border)
Mid diastolic rumble (lower LSB)
Cyanosis
Tachypnea
S2- loud, single, gallop
Murmur –absent
Pulmonary crackles and
hepatomegaly.
ECG RVH RVH- Tall R waves in right
precordical leads.
x ray Cardiomaegaly –RA/RV with
increased pulmonary markings
SNOWMAN or FIGURE OF 8
Heart size – normal
Pulmonary edema

16. SNOWMAN APPERANCE

17. • ECHO- RVH and patent foramen ovale with
R L shunt.
MANAGEMENT:
CHF- inotopic support and diuretic.
Obstructive – initial stabilization by intubation
and ventilation. PGE 1 infusion and emergency
surgical correction-anastomosis of common
pulmonary vein with LA.
Non- obstructive- control of CCF, stablization of
patient and elective surgery.

18. TRICUSPID ATRESIA
• 1.4%
• Congenital absence or agenesis of
morphologic tricuspid valve.
• Type 1- normally related great arteries.
• Type 2-D-transpostion of the great arteries.
• Type3-Malpostion of great arteries other than
D-transposition.
• Type 4-Persistent truncus arterious.

20. CONTENTS INFANT WITH
PULMONARY OLIGEMIA
INFANT WITH
PULMONARY
PLETHORA
SYMPTOMS Cyanosis within first few
days of life.
Severe pulmonary
oligemia.
Hyperapnea and acidosis
if pulmonary blood flow
is markedly diminished.
Signs of heart failure
within first few days
or weeks of life.
Minimal cyanotic,
dyspnoea, fatigue,
difficulty to feed &
perspiration.
Recurrent respiratory
tract infection & FTT.
PHYSICAL
FINDINGS
Central cyanosis,
clubbing, tachypnoea,
hyperapnoea, prominent
‘a’ wave in JVP. Single S2.
Tachypnoea,
tachycardia,
prominent ‘a’ wave in
JVP presystolic
hepatic pulsations.
Signs of congestive
heart failure present.

21. Contd..

22. MANAGEMENT
• Medical management:
Maintenance of neutral thermal environment,
normal acid-base status, normoglycemia and
normocalcemia by apropriate monitoring and
correction, if needed.
• Corrective operation:
Font & Reutzer procedure.

23. Surgical
• 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)

24. TRUNCUS ARTERIOSUS
• The presence of a common trunk that supply
the systemic, pulmonary and coronary
circulation
• Almost always associated with VSD
• 1.2-2.5% of all congenital heart disease

25. Contd..
• There are different anatomical tupes of
truncus arteriosus

26. Contd..
• Presenting feature is congestive heart failure
(tachypneoa, hepatomegaly)
• Exam is significant for
Single and loud S2
– Ejection click of the abnormal truncal valve
– Systolic murmur of truncal valve stenosis if present
– Diastolic murmur of truncal valve insufficiency
– Gallop
• CXR : Cardiomegaly , increased pulmonary circulation

27. • Diuretics – After load
reduction to enhance
systemic blood flow
• Surgical
management:
complete repair with
VSD closure and
conduit placement
between the right
ventricle and
pulmonary arteries

28. EBSTEIN’S ANOMALY
• Ebstein Anomaly of the tricuspid valve - Down
ward displacement of the tricuspid valve -
Right ventricle with two parts - atrialized -
normal ventricular myocardium - Abnormal
tricuspid valve - Huge Rt atrium - Tricuspid
regurgitation - Compromised Rt ventricular
function 65

30. • Clinical Manifestations –
• Easly fatiguability
- Cyanosis
- - Dysrhythmia
- - Rt to Lt shunt through formen ovale
- Holosystolic M at tricuspid area
- Heart failure
Diagnosis: -
CXR - box shaped heart –
ECG - Right BBB - Superior axis deviation
Treatment :- PGE1 - Surgery

31. BOX SHAPE HEART

32. SINGLE VENTRICLE

33. HYPOPLASTIC LEFT HEART SYNDROM

34. THANK YOU