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Natural history and treatment of pulmonic stenosis in adults

Karen Stout, MD
Section Editor
Heidi M Connolly, MD, FASE
Deputy Editor
Susan B Yeon, MD, JD, FACC


Nearly all cases of valvular pulmonic stenosis are congenital in origin, and most cases occur as an isolated lesion. Acquired cases of stenosis of the native pulmonary valve are encountered less commonly but may be caused by the carcinoid syndrome or rheumatic fever (in which case pulmonic stenosis is always associated with other valve abnormalities). There are increasing numbers of patients with stenosis of bioprosthetic or valved conduits used in repair of more complex congenital lesions affecting the pulmonary valve, such as tetralogy of Fallot, pulmonary atresia, or truncus arteriosus. (See "Clinical manifestations and diagnosis of pulmonic stenosis in adults", section on 'Introduction'.)

Congenital valvular pulmonic stenosis exists in three predominant forms [1]:

Most cases involve dome-type pulmonic valve stenosis, which is characterized by a narrow central opening with preserved valve motion. Three rudimentary raphes are generally present without clear-cut commissures. The pulmonary trunk may be dilated due to a medial abnormality. Calcification of the valve can be seen in older patients.

Approximately 20 percent of cases involve pulmonary valve dysplasia, with thickened valve leaflets and relative immobility of the valve (image 1). Pulmonary artery dilation is not commonly associated with dysplastic valves. Dysplastic pulmonary valves are a common component of Noonan syndrome, occurring in up to half of patients in some studies. (See "Clinical manifestations and diagnosis of pulmonic stenosis in adults", section on 'Introduction'.)

More complex congenital heart disease, such as cases of tetralogy of Fallot or transposition of the great arteries, may be associated with a unicuspid or bicuspid pulmonary valve, which may be obstructive. (See "Pathophysiology, clinical features, and diagnosis of tetralogy of Fallot", section on 'Right ventricular outflow obstruction'.)

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Literature review current through: Nov 2017. | This topic last updated: Mar 28, 2016.
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