FFinalistSouthampton · 2027
Child health
AKT · Child health/Congenital heartlow yield

Pulmonary stenosis

Obstruction of right ventricular outflow at the pulmonary valve (acyanotic, or duct-dependent if critical)

Overview

Narrowing of the pulmonary valve/right ventricular outflow tract, obstructing flow to the lungs. Mild-moderate is acyanotic with an ejection systolic murmur; CRITICAL pulmonary stenosis in the neonate is duct-dependent (needs prostaglandin). A feature of Tetralogy of Fallot and Noonan syndrome. Treated with balloon valvuloplasty.

Recognise

  • Ejection systolic murmur at the UPPER LEFT sternal edge (pulmonary area), radiating to the back; ejection click; widely split S2
  • Critical neonatal PS: cyanosis/collapse (duct-dependent pulmonary circulation)
  • Associations: Noonan syndrome (dysplastic pulmonary valve), Tetralogy of Fallot, congenital rubella

Red flags

  • Critical neonatal PS → prostaglandin (duct-dependent) + intervention
  • Right heart failure in severe stenosis

Differentials & how to tell them apart

ASDfixed split S2; PS murmur varies with respiration
Tetralogy of FallotPS + VSD + overriding aorta + RVH (cyanotic)
Innocent murmurno click/RVH, normal echo

Investigations

Echocardiography (valve, gradient, RV); ECG (right ventricular hypertrophy in severe disease).

Management

Balloon valvuloplasty (prostaglandin first if critical neonatal/duct-dependent)

  1. 1Confirm severity on echo. Significant stenosis: balloon valvuloplasty. Critical neonatal PS: prostaglandin to maintain duct-dependent pulmonary flow, then intervention.Gate: Critical neonatal pulmonary stenosis is DUCT-DEPENDENT (pulmonary circulation) → prostaglandin to keep the duct open; think Noonan syndrome (dysplastic valve)
  2. 2Balloon valvuloplasty/surgery; manage associated syndromes; follow-up for restenosis/regurgitation.
Balloon valvuloplasty — first-line intervention for significant stenosis
Prostaglandin (critical neonatal PS) — keep the duct open while arranging intervention

Key points

Ejection systolic murmur (upper left sternal edge) + click + RVH = pulmonary stenosis. Critical neonatal PS is duct-dependent → prostaglandin. Think Noonan; it's also part of Fallot.

Monitor & prognosis

Gradient, RV function.

Good with valvuloplasty.

Source: RCPCH; paediatric cardiology