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Вроджений стеноз легеневого клапана

ORPHA:3189· ICD-10 Q22.1· Congenital pulmonary valvar stenosis

Визначення(English summary)

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).

Поширеність
1-5 / 10 000
Успадкування
Multigenic/multifactorial, Not applicable
Вік початку
Childhood, Infancy, Neonatal