Fact-sheet: Congénital pulmonary valve stenosis


Updated on 01/23/2026 at 10:43 AM

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Definition

Congenital stenosis of the right ventricular outflow tract associating pulmonary valve stenosis, which may be associated with subpulmonary stenosis or supravalvular stenosis.

Clinical features

Ranges from mild, asymptomatic stenosis to critical stenosis discovered in the neonatal period

Signs of right heart failure with cyanosis.

Exertional dyspnea and syncope.

6% are associated with a genetic form with dysplastic pulmonary valve leaflets

Possible associations with:

  • Double-outlet right ventricle
  • Transposition of great arteries
  • Ebstein malformation of the tricuspid valve

Ultrasound

First-line examination. 40% prenatal detection.

Isolated form: small central orifice with thin, dome-shaped pulmonary valve leaflets

Genetic form (6%): dysplastic pulmonary valve leaflets