Pulmonary Valve Stenosis

For further information, see CMDT Part 10-04: Pulmonary Valve Stenosis

• Classic valve is smooth with doming and fusion of commissures

  • Pulmonary artery is often severely dilated due to an associated collagen defect

• Dysplastic valve occurs in Noonan syndrome

  • Pulmonary artery size is normal

• Increased resistance to RV outflow, increased RV pressure, decreased pulmonary blood flow

• Pulmonary stenosis is often congenital, associated with other cardiac lesions

• Without shunting, arterial saturation normal

• However, severe stenosis causes peripheral cyanosis mostly due to reduced RV compliance and opening of a patent foramen ovale

• Mild: asymptomatic

• Moderate to severe: dyspnea with exertion, syncope, chest pain, and eventually RV failure

• Palpable parasternal lift

• Loud, harsh systolic ejection murmur and thrill in second left interspace, radiating to left shoulder; murmur increases with inspiration

• P₂ delayed and soft or absent; ejection click decreases with inspiration

• ECG

  • Right axis deviation or RV hypertrophy

  • Peaked P waves

• Chest radiograph

  • Heart size normal

  • RV and RA prominent

  • Pulmonary artery dilatation

• Doppler echocardiography

  • Diagnostic

  • Can determine gradient across valve

    • Mild pulmonary stenosis: peak gradient < 30 mm Hg

    • Moderate pulmonary stenosis: peak gradient 30–60 mm Hg

    • Severe pulmonary stenosis: peak gradient > 60 mm Hg

  • Subvalvular obstruction may coexist

• Treatment indications

  • All symptomatic patients

  • Patients with a resting gradient > 60 mm Hg, regardless of symptoms

• Treatment of choice

  • Percutaneous balloon valvuloplasty for domed stenotic valve

  • Pulmonic valve replacement for dysplastic valve

• Surgical commissurotomy can also be done, or pulmonary valve replacement (with either a bioprosthetic valve or homograft) when pulmonic regurgitation is too severe or the valve is dysplastic

• Endocarditis prophylaxis is unnecessary for native valves even after valvuloplasty unless there has been prior pulmonary valve endocarditis (a very rare entity)

• RV outflow tract obstruction due to PA conduit stenosis or to homograft stenosis may be treated with a percutaneous pulmonary valve replacement (both the Medtronic Melody valve and the Edwards Sapien XT valve have been FDA approved)