Pulmonary stenosis is usually congenital, and associated with a number of syndromes:

• Noonan syndrome (valvular)
• Williams syndrome (supravalvular)
• Tetralogy of Fallot (valvular)

It is also associated with congenital rubella infection (valvular).

Pulmonary stenosis can be acquired, most often (but still very infrequently) due to carcinoid syndrome.

Pathophysiology:

• Main consequence - RV strain and increase in RV pressure = RV hypertrophy
• RVF In severe stenosis = raised JVP, peripheral oedema, ascites

PC

• Symptoms dont appear until adulthood:
  • syncope
  • angina dyspnoea

On examination:

• Mid-systolic, crescendo-decrescendo murmur heard best over pulmonary valve preceded by a systolic click