History
Most children and adults with mild-to-moderate pulmonic stenosis (pulmonary stenosis) (PS) are asymptomatic. Those with severe PS may experience exertional dyspnea and fatigue. They may also report symptoms of right heart failure (peripheral edema, fatigue, dyspnea). In rare cases, patients can present with exertional angina, syncope, or sudden death.
Physical Examination
In patients with pulmonic stenosis (pulmonary stenosis) (PS), the jugular venous waveform will reveal a prominent a wave due to forceful right atrial contraction in the setting of a stiff, noncompliant right ventricle.
A precordial heave or a palpable impulse from the right ventricle along the left parasternal border may suggest severe PS. In the left upper sternal border, a systolic thrill may be palpable at the level of the second intercostal space.
In valvular PS, auscultation reveals a normal S1 and a widely split S2, with a soft and delayed P2. Valvular PS typically causes a midsystolic murmur in the left upper sternal border that increases with inspiration and radiates diffusely.
In patients with pliable valve leaflets, a systolic ejection click typically precedes the murmur, distinguished from aortic ejection sounds by its increased intensity on expiration and softening on inspiration. As the severity of PS increases, the ejection murmur increases in intensity, its duration prolongs, and its peak becomes more delayed. No ejection click is heard when dysplasia or severe leaflet thickening immobilizes the valve leaflets or if the stenosis is above or below the pulmonic valve.
The murmur of PPS may be continuous, softer, and higher pitched.
Mild-to-moderate desaturation or frank cyanosis may be noted on pulse oximetry if there is right-to-left shunting through a patent foramen ovale, atrial septal defect, or ventricular septal defect.
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Pulmonic Stenosis. Echocardiogram of a patient with severe pulmonic stenosis. This image shows a parasternal short-axis view of the thickened pulmonary valve.
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Pulmonic Stenosis. Echocardiogram of a patient with severe pulmonic stenosis. This image shows a Doppler scan of the peak velocity (5.2 m/s) and gradients (peak 109 mmHg, mean 65 mmHg) across the valve.
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Pulmonic Stenosis. Echocardiogram of a patient with severe pulmonic stenosis. This image shows moderately severe pulmonary insufficiency (orange color flow) is also present.
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Pulmonic Stenosis. This is an echocardiogram of a patient with severe pulmonic stenosis. The first segment shows the parasternal short-axis view of the thickened pulmonary valve. The second segment shows the presence of moderate pulmonary insufficiency (orange color flow). AV = aortic valve; PA = pulmonary artery; PI = pulmonary insufficiency; PV = pulmonary valve.
