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Essentials of Diagnosis
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High-pitched systolic ejection murmur maximal in the second left interspace with radiation to the left shoulder
P2 delayed and soft or absent
Pulmonary ejection click often present and decreases with inspiration—the only right heart sound that decreases with inspiration; all other right heart sounds increase
Echocardiography/Doppler is diagnostic
Patients with peak pulmonic valve gradient > 64 mm Hg or a mean of 35 mm Hg by echocardiography/Doppler should undergo intervention regardless of symptoms; otherwise, percutaneous or surgical intervention may be indicated for symptoms or evidence of right ventricular (RV) dysfunction
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General Considerations
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Often congenital and associated with other cardiac lesions
In valvular pulmonic stenosis, pulmonary blood flow preferentially goes to the left lung
Most patients with valvular pulmonic stenosis have a domed valve, although some patients have a dysplastic valve
Peripheral pulmonic stenosis
Can accompany valvular pulmonic stenosis
May be part of a variety of clinical syndromes, including the congenital rubella syndrome
Noncongenital postoperative pulmonic valvular or main pulmonary artery (PA) stenosis
RV outflow obstructions can also occur when there is
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Mild cases of pulmonic stenosis are asymptomatic
Moderate to severe pulmonic stenosis may cause
Dyspnea on exertion
Syncope
Chest pain
RV failure
In mild to moderate pulmonic stenosis
A loud ejection click can be heard to precede the murmur
This sound decreases with inspiration as the increased RV filling from inspiration prematurely opens the valve during atrial systole when inspiratory increased blood flow to the right heart occurs
In severe pulmonic stenosis
The second sound is obscured by the murmur
Pulmonary component of S2 may be diminished, delayed, or absent
A right-sided S4 and a prominent a wave in the venous pulse are present when there is RV diastolic dysfunction or a c-v wave may be observed in the jugular venous pressure if tricuspid regurgitation is present
Stenosis of the pulmonary valve or RV infundibulum increases the resistance to RV outflow, raises the RV pressure, and limits pulmonary blood flow
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Radiography
Echocardiography/Doppler