What’s wrong?

• Narrowing or obstruction between the RV and the pulmonary circulation
• Frequently associated with other abnormalities
• Four types:
  
  
  • Valvular (Valve leaflets are thickened or narrowed)
  • Supravalvular (PA just above the pulmonary valve is narrowed)
  • Subvalvular also known as infundibular (Muscle under the valve area is thickened, narrowing the outflow trace from RV)
  • Branch peripheral (Right or left PA is narrowed or both may be narrowed)
  
  
• Presents in varying degrees, classified according to how much obstruction to blood flow is present. Critical pulmonary stenosis, severe, moderate, mild.

Corrective Procedures?

1. For Critical Stenosis
   
   Require immediate intervention after birth
   
   
   • Balloon pulmonary valvuloplasty
   • Open pulmonary valve or Blalock Taussig shunt
   
   
2. For Severe Stenosis
   
   
   • To open the valve leaflet – usually Ballon Valvuloplasty
   • If unsuccessful, open pulmonary valvuloplasty (surgery)


3. For Mild-moderate Stenosis
   
   
   • No intervention required

What are the possible complications after the procedures?

1. Non-surgical Method (Balloon Valvulplasty)
   
   
   • Too wide opening may result in severe leak from the valve
   
   
2. Surgical Method
   
   
   • Too wide opening may result in severe leak - patient tend to tolerate for a few years before definite intervention is required. Pulmonary valve replacement using homograft (human) valve or bioprosthetic valve.