Most common cyanotic congenital heart disorder + most common one that survives to adulthood
Tetralogy features:
- Ventricular septal defect (VSD)
- Pulmonary stenosis
- Right ventricular hypertrophy
- Overriding aorta (aorta more central in heart and accepts right sided blood)
Causes/Factors
- Congenital (both environmental and genetic factors)
- Severity of illness depends greatly on degree of pulmonary stenosis
Symptoms
- With closure of ductus arteriosus infants become more cyanotic due to decreasing flow of blood to the lungs and increasing right to left flow across VSD
- Difficulty in feeding/gaining weight
- Delayed growth
- Dyspnoea of exertion
Signs
- Children may squat during hypercyanotic spell as it increases systemic vascular resistance and temporary reversal of shunt
- Pulmonary murmur
Diagnostic Tests
- Echo demonstrates abnormal anatomy
- ECG can also show hypertrophy
- Cardiac CT shows clearer anatomy valuable for surgery
Management
- Oxygen
- Surgery to repair VSD and replace pulmonary valve
Complications/red Flags
- pulmonary regurgitation
- RV outflow tract obstruction