Congenital Heart

Disease

DR. ARVIND BISHNOI

DEPARTMENT OF CVTS, KMC MANIPAL
CLASSIFICATION OF CONG HEART DISEASE
TETROLOGY OF FALLOT
ÉTIENNE-LOUIS ARTHUR FALLOT!

  a French physician, 1888 Fallot

accurately described in detail the
four anatomical characteristics of
tetralogy of Fallot. …’la maladie
bleue

 First anatomic description…Danish

anatomist Niels Stensen, in 1672...
PATHOPHYSIOLOGY
TOF

4 component!

Imagine this is a HEART!

TOF

1) Vetricular Septal Defect

TOF

1) Vetricular Septal Defect

2) Pulmonic Stenosis
TOF

1) Vetricular Septal Defect

2) Pulmonic Stenosis
3) Overriding of dextroposed aorta
TOF

1) Vetricular Septal Defect

2) Pulmonic Stenosis
3) Overriding of dextroposed aorta
4) Right Ventricular hypertrophy
Hence,

 Severity of cyanosis is directly proportional to the severity of pulmonic

stenosis
Clinical Picture

 Symptomatic any time after birth

 Paroxysmal attacks of dyspnea
 Anoxic spells
 Predominantly after waking up
 Child cry
 Dyspnea
 cyanosis
 Loss of consciousness
 Convulsion
 Frequency varies from
once a few days to many
attack everyday
 Dyspnea on exertion
 Exercise intolerance
 Sitting posture – squatting
 Compensatory mechanism
 Squatting increases the peripheral vascular resistance,
 which diminishes the right-to-left shunt
 increases pulmonary blood flow
Cyanosis & Clubbing
Course and Complication

1) Each anoxic spell is potentially fatal

2) Polycytemia
Cerebrovascular thrombosis
3) Anoxic infarction of CNS
Neurological complication
LUNG is a very good filter.
1) Bypassing it may not be a good idea!
2) TOF, venous blood from gut, peripheral system by pass the lung and re-enter
circulation
CNS complications

 Paradoxic embolus
 Cerebral thrombosis
 Cerebral abcess
 Seizures
 Hypoxic damage
 Endocarditis & vegetations
INVESTIGATIONS
 ECHO is diagnostic and demonstrates that aorta is not
continuous with ventricular septum
 Surgical line
 Palliative operations
- recurrent spells,
children who cannot tolerate total correction,
infants with early symptoms
Helen Taussig
Alfred Blalock, Helen Taussig,
Vivian Thomas
shunt

Types :
1. Blalock–Taussig shunt subclavian artery &
pulmonary artery anastomosis
2. Modified Blalock – Taussig shunt Gore-Tex
interposition
3. Waterson shunt Ascending aorta to right
pulmonary artery
4. Potts-Smith shunt Descending aorta to left
pulmonary artery
5. Glenn’s shunt Superior vena cava to right
pulmonary artery
TOF correction
 Rastelli procedure
outcome

 Immediately cyanosis is cured

 Survival 98%
 Good effort tolerance
 Normal life expectancy
 Re intervention for pulmonary valve in 12% of patients