What is the recommended treatment for severe pulmonary stenosis?

Treatment for Severe Pulmonary Stenosis

For adults with severe pulmonary stenosis, balloon valvuloplasty is the recommended first-line treatment, regardless of symptom status. 1

Definition of Severe Pulmonary Stenosis

Severe pulmonary stenosis is defined as:

• Peak gradient ≥64 mm Hg (peak velocity >4 m/s)
• Mean gradient >35 mm Hg 1

Treatment Algorithm

For Symptomatic Patients with Severe PS:

1. First-line: Balloon Valvuloplasty

   • Class I recommendation (Level of Evidence: B-NR) for patients with:
     • Heart failure symptoms
     • Cyanosis from interatrial right-to-left communication
     • Exercise intolerance 1

2. Second-line: Surgical Repair

   • Class I recommendation (Level of Evidence: B-NR) for patients who:
     • Are ineligible for balloon valvuloplasty
     • Have failed balloon valvuloplasty 1
   • Surgical indications include:
     • Marked dysplasia of the pulmonary valve
     • Significant hypoplasia of the annulus 1

For Asymptomatic Patients with Severe PS:

• Intervention is reasonable (Class IIa recommendation, Level of Evidence: C-EO) 1
• Benefits include:
  • Reduction of RV pressure
  • Prevention of potential sequelae including RV dysfunction
  • Avoidance of progressive TR or right-to-left shunting 1

Technical Considerations for Balloon Valvuloplasty

• Recommended balloon/annulus ratio: 1.2 to 1.25 2
• Balloon valvuloplasty results in:
  • Immediate reduction of gradient
  • Increased jet width
  • Improved motion of pulmonary valve leaflets 2
• Long-term outcomes show:
  • Excellent results up to 17 years post-procedure
  • Minimal restenosis rates
  • Event-free rates in mid-80s and mid-70s at 10 and 15 years respectively 3, 2

Management of Concomitant Conditions

• Infundibular Stenosis: Often regresses after successful balloon valvuloplasty 3
• Tricuspid Regurgitation: Significant TR typically regresses or disappears after successful balloon valvuloplasty 3

Post-Procedure Monitoring

Regular follow-up is essential to monitor:

• Residual or recurrent stenosis
• Development of pulmonary regurgitation
• RV size and function
• Exercise capacity 1

Potential Complications and Pitfalls

1. Restenosis:

   • Occurs in approximately 10% of cases
   • Risk factors: balloon/annulus ratio <1.2, immediate post-valvuloplasty gradient ≥30 mm Hg 2
   • Management: Redilatation with larger balloons is effective 2

2. Pulmonary Regurgitation:

   • Common long-term sequela
   • Requires lifelong monitoring 2
   • May eventually require pulmonary valve replacement if severe with RV dilation 1

3. New Infundibular Stenosis:

   • May develop immediately after balloon valvuloplasty
   • Usually resolves over time 4

Balloon pulmonary valvuloplasty should be considered the treatment of choice for adult patients with severe valvular pulmonary stenosis, even in the presence of severe infundibular stenosis or severe tricuspid regurgitation, as these conditions typically improve following successful relief of the valvular obstruction 3.