Pulmonic Stenosis Clinical Presentation

Updated: Dec 22, 2014
  • Author: Xiushui (Mike) Ren, MD; Chief Editor: Richard A Lange, MD, MBA  more...
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Presentation

History

Most children and adults with mild-to-moderately severe pulmonic stenosis (PS) are asymptomatic. Those with severe PS may experience exertional dyspnea and fatigue.

In extremely rare cases, patients present with exertional angina, syncope, or sudden death.

Peripheral edema and other typical symptoms occur with right heart failure.

Cyanosis is present in those with significant right-to-left shunt via a patent foramen ovale, atrial septal defect, or ventricular septal defect.

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Physical

A precordial heave or a palpable impulse from the RV along the left parasternal border may suggest severe PS. In the left upper sternal border, a systolic thrill may be palpable at the level of the second intercostal space.

In valvular PS, auscultation reveals a normal S1 and a widely split S2, with a soft and delayed P2. Valvular PS typically causes a systolic crescendo-decrescendo ejection murmur in the left upper sternal border that increases with inspiration and radiates diffusely.

In patients with pliable valve leaflets, a systolic ejection click may precede the murmur, distinguished from aortic ejection sounds by its increased intensity on expiration and softening on inspiration. As the severity of PS increases, the ejection murmur increases in intensity, its duration prolongs, and its peak becomes more delayed. No ejection click is heard when dysplasia or severe leaflet thickening immobilizes the valve leaflets, or if the stenosis is above or below the pulmonic valve.

The murmur of PPS may be continuous, softer, and higher pitched.

Mild-to-moderately severe desaturation or frank cyanosis may be noted with right-to-left shunting through a patent foramen ovale, atrial septal defect, or ventricular septal defect.

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Causes

See also Pathophysiology.

Other forms of acquired pulmonic stenosis include the following:

  • PS is a rare manifestation of rheumatic heart disease, and it follows involvement of the mitral and aortic valves.
  • Carcinoid may result in development of myxomatous plaques in the RV outflow tract, with distortion and constriction of the pulmonic ring, as well as fusion or destruction of pulmonary valve leaflets, resulting in both stenosis and regurgitation.
  • Rarely, cardiac tumors can grow on or into the RV outflow tract and cause flow obstruction.
  • Sinus of Valsalva aneurysms and aortic graft aneurysms are extracardiac entities that can cause PS by external compression.
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