Severe cases may present with right-sided heart failure.
P2 delayed and soft or absent.
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 gradients greater than 60 mm Hg or a mean of 40 mm Hg by echocardiography/Doppler should undergo intervention regard less of symptoms. Otherwise, operate for symptoms.
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