Pulmonic stenosis
Pulmonic stenosis is obstruction to right ventricular outflow from a narrowed pulmonary valve, usually congenital. It causes a systolic ejection murmur with an ejection click and, when severe, right ventricular hypertrophy and failure; severe or symptomatic disease is treated effectively with balloon valvuloplasty.
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What this article covers
- Overview
Definition: pulmonic stenosis is obstruction to right ventricular outflow caused by narrowing at the pulmonary valve (or, less often, sub- or supravalvular), usually congenital.
- Epidemiology
A common congenital heart lesion, often isolated.
- Risk factors
Congenital valve malformation (often a domed or dysplastic valve).
- Pathophysiology
Fixed right-sided obstruction: valve narrowing raises right ventricular systolic pressure and the transvalvular gradient.
- Clinical features
Often asymptomatic with an incidental murmur.
- Differential diagnosis
Aortic stenosis: a systolic murmur radiating to the carotids, distinguished by a left-sided gradient at the aortic valve on echocardiography rather than right ventricular outflow obstruction (Aortic stenosis).
- Diagnosis
Establish and grade with echocardiography measuring the peak gradient and jet velocity: the diagnostic basis is echocardiography demonstrating a thickened or domed pulmonary valve with a raised…
- Management
First-line, relieve severe or symptomatic obstruction with balloon valvuloplasty: percutaneous balloon pulmonary valvuloplasty is the treatment of choice for typical domed valvular stenosis and gives durable relief.
- Complications
Right ventricular hypertrophy and failure.
- Prognosis
Excellent after relief of obstruction, with durable results for typical valves.
- Prevention
Timely valvuloplasty for severe disease (peak gradient over 64 mmHg) to prevent right ventricular damage.
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