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ESSENTIALS OF DIAGNOSIS

  • Asymptomatic unless lesion is at least moderately severe.

  • Severe cases may present with right-sided heart failure.

  • 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 greater than 64 mm Hg or a mean of 35 mm Hg by echocardiography/Doppler should undergo intervention regardless of symptoms. Otherwise, operate for symptoms or evidence for right ventricular (RV) dysfunction.

  • A dysplastic pulmonary valve usually requires surgical treatment, while a domed pulmonary valve stenosis usually can be treated with balloon valvuloplasty.

  • RV outflow tract obstruction due to conduit stenosis, homograft stenosis and some prior bioprosthetic valve dysfunction may be treated with a percutaneous pulmonary valve replacement.

GENERAL CONSIDERATIONS

Stenosis of the pulmonary valve or RV infundibulum increases the resistance to RV outflow, raises the RV pressure, and limits pulmonary blood flow (eFigure 10–14). Pulmonic stenosis is often congenital and associated with other cardiac lesions. Pulmonary blood flow preferentially goes to the left lung in valvular pulmonic stenosis. Most patients with valvular pulmonic stenosis have a domed valve, although some patients have a dysplastic valve, especially those with Noonan syndrome. The phenotype of Noonan syndrome includes short stature, web neck, dental malocclusion, antimongoloid slanting of the eyes, mental retardation, and hypogonadism. Unlike those with a domed valve, patients with a dysplastic valve do not have a dilated main PA or commissural fusion. In the absence of associated shunts, arterial saturation is normal. Peripheral pulmonic stenosis can accompany valvular pulmonic stenosis and may be part of a variety of clinical syndromes, including the congenital rubella syndrome. Patients who have had the Ross procedure for aortic valve disease (transfer of the pulmonary valve to the aortic position with a homograft pulmonary valve placed in the pulmonary position) may experience noncongenital postoperative pulmonic valvular or main pulmonary artery (PA) stenosis 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 or when there is degeneration of a bioprosthetic replacement pulmonary valve.

eFigure 10–14.

Pulmonary stenosis. (Used with permission of Mayo Foundation for Medical Education and Research, all rights reserved.)

CLINICAL FINDINGS

A. Symptoms and Signs

Mild cases of pulmonic stenosis are asymptomatic; moderate to severe pulmonic stenosis may cause symptoms of dyspnea on exertion, syncope, chest pain, and eventually RV failure.

On examination, there is often a palpable parasternal lift due to right ventricular hypertrophy (RVH) and the pulmonary outflow tract may be palpable ...

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