PROVe: Pulmonary stenosis, Right ventricular hypertrophy, Overriding aorta, VSD
Expansion
PROVe:
- P - Pulmonary stenosis (right ventricular outflow tract obstruction)
- R - Right ventricular hypertrophy
- O - Overriding aorta
- V - Ventricular septal defect
The pulmonary stenosis is the determinant: the tighter it is, the more blood is shunted right to left through the VSD, and the more cyanosed the child.
Presentation: usually at 1 to 2 months, with cyanosis, a harsh ejection systolic murmur at the left sternal edge from the pulmonary stenosis (not the VSD), and later clubbing and failure to thrive.
Tet spells are acute episodes of deep cyanosis and irritability, precipitated by crying, feeding or waking, caused by infundibular spasm. Older children squat, which raises systemic vascular resistance and reduces the right to left shunt. Acute management is knees to chest, oxygen, morphine, fluids and beta blockade.
Chest radiograph shows a boot shaped heart (coeur en sabot) from the upturned apex, with a concave pulmonary bay and reduced pulmonary vascular markings.
It arises from anterosuperior displacement of the infundibular septum, and is associated with 22q11 deletion (DiGeorge) and Down syndrome.