CHU Pontoise, France

About 10 % of CHD  (isolated = 0.8 % for L. Allan)

Sex ratio M/F = 1

Two varieties :

• Valvular 90 %
• Infundibular 10 %

Etiology: Possible genetic etiology with a recurrent rate of 2-4 %

Diagnosis:

• Small or enlarged (less frequent) pulmonary artery
• Thickening and restrictive motion of the pulmonary valve
• Thickened interventricular septum
• Dilated right ventricle (may be inapparent)
• Turbulent flow at the pulmonary valve
• Accelerated post stenotic flow
• Pulmonary regurgitation (forward flow) if valvular dysplasia
• Tricuspid regurgitation (more severe types)
• Reversed flow in the ductus arteriosus

Case Report:

Enlarged main pulmonay artery

Post-stenotic jet flow

Thickened and stenotic pulmonary valve

Turbulent forward flow in the pulmonary valve

Turbulent flow at the level of the stenotic pulmonary valve

Forward flow in the pulmonary valve

Associated Anomalies:

• Mainly with atypical types of pulmonary stenosis.
• Patent foramen ovale
• Ostium secundum
• Supravalvular aortic stenosis
• Total abnormal venous return

Syndromes::

• Noonan syndrome (Autosomal Dominant)
• Williams syndrome (del 7q 11.2)
• Alagille syndrome (Autosomal Dominant)
• Congenital rubella

Differential Diagnosis: Hypertrophic myocardiopathy of the right ventricle

Prognosis: depends on the severity and gestational age at diagnosis

• TOP may be consider in critical cases (22 %)
• In continuing pregnancy, serial follow-up is advisable.
• Cardiologic evaluation for prognosis: delivery in a tertiary center with a pediatric cardiac surgery unit.