Congenital Heart Disease in the Emergency Department

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Pulmonary Stenosis (PS)

The pulmonary valve leaflets are partly fused together, giving a restrictive exit from the right ventricle (see figure).

pulmonary_stenosis

Clinical presentation

Symptoms
  • Most are asymptomatic
  • A small number of neonates with critical pulmonary stenosis have a duct-dependent pulmonary circulation and present in the first few days of life with cyanosis
Physical Signs
  • An ejection systolic murmur best heard at the upper left sternal edge; thrill may be present
  • An ejection click best heard at the upper left sternal edge
  • When severe there is a prominent right ventricular impulse (heave)

Investigations

Chest radiograph
  • Normal or post-stenotic dilatation of the pulmonary artery
ECG
  • Shows evidence of right ventricular hypertrophy (upright T wave in V1)

Management

When there is critical or severe valvular pulmonary stenosis, balloon valvuloplasty of the pulmonic valve is performed in the neonatal period. In cases of moderate pulmonary stenosis, the procedure is performed when the gradient across the pulmonary valve exceeds 50 mm Hg. Cases of mild pulmonary stenosis are usually followed conservatively.