Stenosis of the pulmonary artery is a condition where the pulmonary artery is subject to an abnormal constriction .Peripheral pulmonary artery stenosis may occur as an isolated event or in association with Alagille syndrome, Berardinelli-Seip congenital lipodystrophy type1, Costello syndrome, Keutel syndrome,  nasodigitoacoustic syndrome (Keipert syndrome), Noonan syndrome  or Williams syndrome. It should not be confused with a pulmonary valve stenosis, which is in the heart, but can have similar hemodynamic effects. Both stenosis of the pulmonary artery and pulmonary valve stenosis are causes of pulmonic stenosis. The presentation of such a case may be very misleading. The case that we present here is about a 37 year old Carribean female who presented with progressive increase in SOB.

Keywords: Pulmonary Stenosis; Pulmonary Hypertension; Cardiovascular; Hypertension; Angiography; Congenital Heart Disease; Chest Pain.