Congenital pulmonary valvar stenosis
ORPHA:3189· ICD-10 Q22.1
Definition
A rare congenital heart malformation characterized by an obstruction to flow through the pulmonary valve with a clinical presentation that may vary from critical stenosis presenting in the neonatal period to asymptomatic mild stenosis. The obstruction at the valvular level can be associated with obstruction at the subpulmonary, or supravalvar levels (valvar, subpulmonary, supravalvar pulmonary stenosis (PS).
- Prevalence
- 1-5 / 10 000
- Inheritance
- Multigenic/multifactorial, Not applicable
- Age of onset
- Childhood, Infancy, Neonatal