Paediatrics: Pulmonary stenosis

Pulmonary stenosis

This is a common form of CHD due to the following:

• Isolated thickened deformed pulmonary valves (usually).
• Isolated infundibular stenosis; supravalvular pulmonary stenosis.
• Branch pulmonary artery stenosis.

Pulmonary valve stenosis is seen in Noonan syndrome.

Clinical features

• Asymptomatic (mild to moderate stenosis).
• Poor exercise tolerance (severe stenosis).
• Right ventricular failure/cyanosis (critical stenosis).

Prognosis

Mild to moderate stenosis is compatible with normal activities, but patients require monitoring because worsening obstruction and significant pressure gradients may develop, which will predispose them to heart failure when very severe.

Treatment 

Severe pulmonary valve stenosis requires treating by transvenous catheter balloon dilatation.