pulmonic valvular stenosis

Etiology

• may occur as an isolated lesion
• may occur in conjunction with ventricular septal defect
• Noonan's syndrome
• Williams-Beuren syndrome

Pathology

• valve is frequently pliable
• valve may be bicuspid
• thickened dysplastic valve occurs in Noonan's syndrome
• right ventricular hypertrophy may occur

Clinical manifestations

• often asymptomatic
• prominent a-wave in jugular venous pulse
• right ventricular heave
  • right ventricular parasternal lift
• pulmonic ejection click immediately after S1
  • diminishes with inspiration
  • earlier the click, the more severe the stenosis
  • click indicates valve is pliable & noncalcified
  • results from rapid opening of the pulmonary vavle leaflets
• systolic ejection murmur
  • mid- to late-peaking crescendo-decrescendo systolic murmur
    • heard best at the upper left sternal border (2nd left intercostal space)
  • radiation to the left clavicle
  • the longer the murmur & later peaking, the more severe the stenosis
• soft & late P2
  • with more severe stenosis, P2 becomes inaudible
  • S2 may become fixed with severe stenosis & inaudible P2
• systolic thrill due to high flow velocity across the pulmonary valve

Diagnostic procedures

• electrocardiogram
  • if right ventricular systolic pressure is < 60 mm Hg, electrocardiogram is normal
  • otherwise
    • right axis deviation
    • right ventricular hypertrophy
    • right atrial enlargement
• echocardiogram
  • right ventricular hypertrophy
  • increased right ventricular pressure
  • pressure gradient across pulmonic valve

Radiology

• chest X-ray
  • post-stenotic pulmonary dilatation
  • pulmonary oligemia only with severe stenosis
  • calcification of pulmonary valve (rare)
  • right atrial enlargement

Complications

• severe pulmonary regurgitation after pulmonary valvuloplasty

Differential diagnosis

• atrial septal defect
  • early-peaking systolic murmur
  • fixed splitting of S2
• aortic valvular stenosis
  • may be present in patients with a bicuspid aortic valve
  • crescendo-decrescendo systolic murmur heard best at the upper right sternal border
    • murmur becomes late peaking as disease progresses
• patent foramen ovale
  • generally normal cardiac examination
• hypertrophic obstructive cardiomyopathy
  • may cause a systolic murmur at the left sternal border
  • not associated with an ejection click
  • not associated with right ventricular heave

Management

• no treatment (surgery) unless
  • symptomatic
  • right ventricular pressure approaches 2/3 systemic pressure
  • small pulmonary valve annulus
  • pulmonary regurgitation > moderate
  • subvalvular or supravalvular pulmonary stenosis
  • cardiac surgery for another reason
• repair of severe pulmonary valvular stenosis regardless of symptoms in a patient with Noonan's syndrome
  • patients with dysplastic valve (Noonan's syndrome) should undergo pulmonary valve replacement
  • participation in sports not recommended in patients with severe pulmonary valvular stenosis
    • pulmonary valve replacement recommended
• percutaneous balloon valvuloplasty for pliable valve
  • not clear that this would correct pulmonary regurgitation
• surgical replacement for caclified valve
• lifelong cardiac survelliance if:
  • balloon valvuloplasty
  • pulmonary valve replacement
• prophylaxis for bacterial endocarditis not indicated

More general terms

• pulmonic valve disease

Additional terms

• pulmonic valve (valve of pulmonary trunk)