Pulmonic stenosis is narrowing of the pulmonary valve, usually present from birth, which makes the right ventricle work harder to push blood into the lungs. It can occur alone or as part of syndromes such as Noonan syndrome, and mild cases often never need treatment.
Key symptoms
- Mild cases: no symptoms
- Shortness of breath and fatigue on exertion
- Chest pain or fainting with severe narrowing
- Cyanosis in severe cases in newborns
What you find on exam
- Systolic ejection murmur at the left upper sternal border
- Ejection click that gets softer with inspiration
- Widely split S2 with a soft pulmonic component
- Right ventricular heave and prominent a waves in the neck
Tests
- Echocardiogram with Doppler: valve gradient and right ventricle size
- ECG: right axis deviation and right ventricular hypertrophy
- Chest X-ray: dilated pulmonary artery beyond the narrowing
- Cardiac MRI or catheterization when needed
Treatment basics
- Observation for mild stenosis
- Balloon valvuloplasty for moderate to severe stenosis or symptoms
- Surgical repair or valve replacement if the balloon fails or the valve is dysplastic
- Alprostadil to keep the ductus open in critical stenosis in newborns
Red flags
- Fainting or chest pain on exertion
- Cyanosis in a newborn
- Signs of right heart failure: leg edema, enlarged liver
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Sources: NCCPA PANCE Content Blueprint; NBOME COMLEX-USA clinical presentations; AAMC Core Entrustable Professional Activities (Core EPAs). Written by Making Synapses. Making Synapses is not affiliated with these organizations.