Aortic and pulmonary stenosis

Question 1

What is aortic stenosis?

Answer 1

Where the aortic valve leaflets are partly fused together, giving restrictive exit from the left ventricle.

There may be one to three leaflets

Question 2

What are the clinical features of aortic stenosis?

(signs (5) and symptoms)

Answer 2

Symptoms:

• Asymptomatic except in severe stenosis
• Severe stenosis:
  
  • reduced exercise tolerance
  • Chest pain or syncope on exertion

Physical signs:

• Small volume and slow rising pulse
• Carotid thrill (always)
• Ejection systolic murmer at ULSE radiating to nect
• Delayed and soft aortic second sound
• Apical ejection click

Question 3

What investigations should you do for suspected AS?

What would they show?

Answer 3

Chest x-ray:

• Normal or prominent left ventricle with post-stenotic dilatation of ascending aorta

ECG:

• May show left ventricular hypertrophy (deep s wave in V2 and tall R wave in V6)
• (down going t wave suggests left ventricular strain and severe aortic stenosis)

Question 4

How should you manage AS?

Answer 4

• Regular clinical and echo assessment in order to assess when to intervene
• Children with symptoms on exercise (severe AS) will undergo balloon valvotomy
• May require aortic valve replacement if severe

Balloon valvotomy generally safe in older children but more dangerous & difficult in neonates

Question 5

What is pulmonary stenosis?

Answer 5

• Where the pulmonary valve leaflets are partial fused together giving resticted exit from the right ventricle

Question 6

Signs and symptoms for PS?

Answer 6

Symptoms:

• Most asymptomatic
• Small number of neonates with critical PS have a duct dependent pulmonary circulation and present in first few days of life with cyanosis

Signs:

• An ejection systolic murmer best heard at the ULSE
• Thrill may be present
• Ejection click may be heard at ULSE
• When very severe ventricular heave can be felt (prominent right ventricular impulse)

Question 7

What investigations are required for PS?

What would they should?

Answer 7

Chest radiograph:

• Normal or post-stenotic dilation of the pulmonary artery

ECG:

• Evidence of right ventricular hypertrophy (upright T wave in V1)

Question 8

How should PS be managed?

Answer 8

• Most children asymptomatic
• Intervention required when pressure gradient across pulmonary valve become markedly increased (>64mmHg):
  
  • Trans-catheter ballon dilatation is the treatment of choice in most children