Sections View Full Chapter Figures Tables Videos Full Chapter Figures Tables Videos Supplementary Content + Download Section PDF Listen ++ For further information, see CMDT Part 10-04: Pulmonary Valve Stenosis + Key Features Download Section PDF Listen +++ ++ 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 Download Section PDF Listen +++ ++ 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 Download Section PDF Listen +++ ++ 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 Download Section PDF Listen +++ ++ 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)