Tetralogy Of Fallot: Definition
Congenital cardiac malformation characterised by:Right ventricular out flow tract obstruction
Right ventricular hypertrophy
VSD
Overriding aorta
TOF: Pathophysiology
RVOTO + VSD creates ↓ in pulmonary blood flowRVOTO is progressive and dynamic
Pulmonary oligaemia leads to systemic hypoxemia and invites polycythaemia
RV hypertrophies in response to RVOTO
Polycythaemia increases blood viscosity and promotes thromboses in various vascular beds
TOF: Clinical Features
Cyanosis is present by 6wks to 6mths of life.Dyspnoea on exertion
Squatting
Hypoxic spells
Growth retardation
RV predominance on palpation
Systolic thrill / murmur LSE
Cyanosis and clubbing
TOF: Diagnosis
HistoryPhysical examination
CXR
ECG
ECHO
FBC + haematocrit
TOF: Treatment
Hypercyanotic attacksKnee-chest position
Oxygen inhalation ( limited benefit )
Morphine sulphate 0.1- 0.2mg/kg
Phenylephrine 0.02mg/kg IV to increase systemic vascular resistance
Palliative treatment
Systemic pulmonary shunt e.g modified BT shunt.
Definitive treatment
Total intracardiac correction under cardiopulmonary bypass.
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