Right ventricular outflow tract obstruction pulmonary supravalvular stenosis

Right ventricular outflow tract obstruction Microchapters
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Anatomy of Pulmonary Valve
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Pulmonary valve stenosis
Pulmonary subvalvular stenosis
Pulmonary supravalvular stenosis
Pulmonary atresia
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]; Associate Editor-in-Chief: Keri Shafer, M.D. [3]

Overview

Supra valvular pulmonary artery stenosis is defined as stenosis of the pulmonary artery above the level of the valve area.

Anatomy

1. Also called peripheral pulmonary stenosis.

2. May have one or many stenoses of the PA or its branches.

3. Infrequently occurs as an isolated lesion, more commonly occurs as part of Tetralogy of Fallot.

Diagnosis

Physical Examination

1. The murmur is most prominent at the upper left sternal border, axillae, and the posterior lung fields.

ACC / AHA Guidelines - Recommendations for evaluation of patients with supravalvular, branch, and peripheral Pulmonary Stenosis (DO NOT EDIT)

Class I

"1.Patients with suspected supravalvular, branch, or peripheral PS should have baseline imaging with echocardiography- Doppler plus 1 of the following: MRI angiography, CT angiography, or contrast angiography.(Level of Evidence: B) "