Pulmonary Stenosis (PS)

Pulmonary stenosis at the valvar level accounts for 8-10% of all congenital heart defects.  Presentation depends on the severity of the stenosis and the presence of an ASD.

Patients present as infants with a harsh systolic ejection murmur.  CXR may show a prominent pulmonary artery shadow secondary to post-stenotic dilation.  Echocardiography is used to establish the diagnosis and severity, and associated abnormalities.  Cardiac catheterization may be performed for balloon valvotomy.

Treatment is indicated if symptoms are present or the transvalvar pressure gradient is significant.  Catheterization with balloon valvotomy is the preferred initial treatment, and may be attempted in cases of recurrent stenosis.  Most patients will have pulmonary insufficiency after valvotomy, but it is usually well tolerated.  Open surgical pulmonary valvotomy is indicated in cases of failed balloon valvotomy.  A trans-annular patch can be placed to enlarge a small annulus.  Long-term outcomes are excellent.