Peripheral Pulmonary Stenosis (PPS)

Peripheral pulmonary stenosis (PPS) represents a narrowing  in one or multiple regions of the branches of the pulmonary arteries. PPS can cause obstruction at the level of the main pulmonary artery, at its bifurcation, or at the more distal branches. It may be associated with other congenital cardiac anomalies such as valvar PS, ASD, VSD or PDA or tetralogy of Fallot (TOF). PPS is associated with various inherited and acquired conditions including Rubella, Alagille, Noonan, Ehlers-Danlos, or Williams syndromes.  It may also be seen in newborns within the first few months of life which typically represents a mild variant which typically resolves within the first 6 months of life.