Congenital heart diseases

(CYANOTIC)
By Vignaa
Supervisor: Dr. Sum
Embryology of the heart
 Lesions with right to left shunts
(Cyanotic)
A) Normal or decreased pulmonary blood flow
- Tetralogy of Fallot
- Tricuspid atresia

B) Increased pulmonary blood flow

- Complete transposition of great arteries
- Truncus arteriosus
- TAPVR (Total anomalous pulmonary venous
return)
 Complete transposition of great
arteries (D-transposition)
• Aorta arises from the morphologic right ventricle
and lies anterior to the pulmonary artery, which
originates from morphologic left ventricle

• Not compatible with life UNLESS there is a

simultaneous presence of an interarterial
communication ( foramen ovale or ASD)

• Common in male babies (esp in diabetic mothers)

• Associated with VSD and PDA

• S1: normal, S2: single aortic component heard,

associated with holosystolic murmur- VSD, continuous
machine like murmur- PDA

• ECG: right ventricular hypertrophy

• Treatment:
• A) Supportive: PGE1 infusion to keep PDA open,
balloon atrial septostomy to keep ASD open
• B) Surgery: Jatene procedure/ Mustard or senning
procedure
 TAPVR
• Pulmonary veins usually drain into left atrium but in
these patients, pulmonary veins may connect to
systemic veins within the thorax (supradiaphragmatic)
or portal vein in the abdomen(infradiaphragmatic)
draining oxygenated blood into right atrium
• Associated anomalies:
• - common atrium
• - single ventricle
• - PDA
• - Truncus arteriosus
• Signs and symptoms: cyanosis, continuous
murmur along LSE, loud P2 (development of
pulmonary hypertension), intensity of the
murmur decreases as the pulmonary
hypertension progresses

• Natural history of TAPVR

- 50% infants dies by 6 months
- 80% die in 1 year
Type Description Abnormality
1 Supracardiac PV joins SVC
2 Cardiac PV joins RA
3 Infracardiac PV joins IVC or
below
4 Mixed Rare, multiple
connections
Merry Christmas!
 Tetralogy of Fallot
• Most common congenital heart disease

• 4 components
- Large VSD
- Pulmonary stenosis
- Overriding aorta
- Right ventricular hypertrophy
• Severity depends on the pulmonary outflow
obstruction

• Clinical features: S1 normal, single and loud

S2, ESM

• ECG: right axis deviation, large R wave in V1

• Hypercyanotic spells (Tet spells)

• Complications:
- Infective endocarditis
- Embolization and cerebral abscess
- Secondary polycythemia

• Treatment
- Squating/ knee to chest position
- IV PGE1 infusion
- Oxygen
- Morphine
- Beta blocker ( Propanolol)
- Noradrenaline in resistant cases
- Surgery- Blalock- Taussig shunt, Pott’s procedure
 Truncus Arteriosus
• Uncommon congenital abnormality

• Single vessel forming the outflow tract for both

ventricles

• It is due to failure of development of aortico-

pulmonary septum

• Always associated with large VSD

• Three types (Collette-Edward classification)

• Associated with Di-George syndrome

• Usually death follows within 5 weeks

• Only 15% survives till 1 year, severe PPHN

develops after 1 year of life

• Ideal age for corrective surgery is 3-6 months old

 Tricuspid atresia
• Tricuspid valve is absent ,the floor of RA is intact

• Usually accompanied by VSD

• Blood flows from RA LA LV RV

pulmonary artery

• Clinical features: prominent JVP, cyanosis, systolic

murmur (VSD)
• Management
- No way to replace a tricuspid valve

- Treatment aim is to ensure adequate blood

flow through the heart and into the lungs

- Requires multiple surgery ( Atrial septostomy,

shunting, Fontan procedure)
• Thank you.