Pulmonary Stenosis

Question 1

Aetiology

Answer 1

• uncommon
• 5% of all congenital heart disease
• usually valvular level but can be sub or supra-valvular
• virtually all congenital in origin and may be due to maternal rubella
• unlikely but can be related to rheumatic fever, in combination with other valves
• rarely carcinoid syndrome

Question 2

Symptoms

Answer 2

• frequently none. Mild to moderate cases are very stable and rarely require treatment

In significant disease can cause:

• asthenia
• syncope
• right sided heart failure

Question 3

Signs

Answer 3

• ejection systolic murmur loudest p area and during inspiration
• softening or delay of P2 sound
• palpable thrill over p area
  Palpable ejection click over p area
• pronounced a wave on severe disease due to RV hypertrophy
• RV heave in same situation and RV gallop rhythm

Question 4

Differential diagnosis

Answer 4

Aortic stenosis

• in mild disease difficult to tell the two apart. But being louder in pulmonary area and during inspiration good sign.
• if more severe then presence of right-sided signs with absence of arterial pulse character changes of AS allow distinction. No carotid radiation.

Question 5

Associated conditions

Answer 5

Look for signs of other congenital heart problems, particularly fallots tetralogy

Question 6

Investigations

Answer 6

• Echo

- CMR if associated anatomical lesions, particularly supra-valvular lesions (also images pulmonary artery anatomy well

Question 7

Management

Answer 7

• If seen in adulthood will be very mild, requiring no intervention, or already had corrective procedure
• balloon valvuloplasty of RVOT in infants as palliative procedure
• surgical reconstruction of RVOT of obstruction is severe
• shunts from vena cava or RA are used
• percutaneous PV replacement is done (preceded TAVI)