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For further information, see CMDT Part 10-04: Pulmonary Valve Stenosis

Key Features

Essentials of Diagnosis

  • 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

General Considerations

  • 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

    • May be seen in patients who have had the Ross procedure for aortic valve disease

    • Occurs due to an immune response in the homograft

  • RV outflow obstructions can also occur when there is

    • A conduit from the RV to the pulmonary artery that becomes stenotic from degenerative changes over time

    • Degeneration of a bioprosthetic replacement pulmonary valve

Clinical Findings

  • 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

Diagnosis

Imaging

  • Radiography

    • Heart size may be normal

    • A prominent RV and RA or gross cardiac enlargement may be present, depending on the severity

  • Echocardiography/Doppler

    • Diagnostic

    • Can provide evidence for a doming valve versus a dysplastic valve

    • Can estimate the gradient across the valve

    • Peak gradients

      • Mild pulmonic stenosis: < 36 mm Hg

      • Moderate pulmonic stenosis: 36–64 mm Hg

      • Severe pulmonic stenosis: > 64 mm Hg (or mean ...

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