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Key Features

  • 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

Clinical Findings

  • 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

Diagnosis

  • 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

  • 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)

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