Pulmonic Stenosis Clinical Presentation

  • Author: Xiushui (Mike) Ren; Chief Editor: Richard A Lange, MD   more...
 
Updated: Jan 4, 2012
 

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 Pathophysiology.

  • Other forms of acquired pulmonic stenosis
    • 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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Contributor Information and Disclosures
Author

Xiushui (Mike) Ren  MD, Cardiovascular Physician, Department of Cardiology, Kaiser Medical Center; Associate Director of Research, Cardiovascular Diseases Fellowship, California Pacific Medical Center

Xiushui (Mike) Ren is a member of the following medical societies: Alpha Omega Alpha, American College of Cardiology, and American Society of Echocardiography

Disclosure: Nothing to disclose.

Coauthor(s)

Lauralyn B Cannistra, MD, FACC  Director of Echocardiography Lab and Cardiac Rehabilitation, Assistant Professor, Department of Medicine, Memorial Hospital of Rhode Island, Brown University School of Medicine

Lauralyn B Cannistra, MD, FACC is a member of the following medical societies: American College of Cardiology, American Heart Association, American Society of Anesthesiologists, American Society of Echocardiography, and Rhode Island Medical Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Park W Willis IV, MD  Sarah Graham Distinguished Professor of Medicine and Pediatrics, University of North Carolina at Chapel Hill School of Medicine

Park W Willis IV, MD is a member of the following medical societies: American Society of Echocardiography

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Marschall S Runge, MD, PhD  Charles and Anne Sanders Distinguished Professor of Medicine, Chairman, Department of Medicine, Vice Dean for Clinical Affairs, University of North Carolina at Chapel Hill School of Medicine

Marschall S Runge, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American College of Cardiology, American College of Physicians-American Society of Internal Medicine, American Federation for Clinical Research, American Federation for Medical Research, American Heart Association, American Physiological Society, American Society for Clinical Investigation, American Society for Investigative Pathology, Association of American Physicians, Association of Professors of Cardiology, Association of Professors of Medicine, Southern Society for Clinical Investigation, and Texas Medical Association

Disclosure: Pfizer Honoraria Speaking and teaching; Merck Honoraria Speaking and teaching; Orthoclinica Diagnostica Consulting fee Consulting

Amer Suleman, MD  Private Practice

Amer Suleman, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Institute of Stress, American Society of Hypertension, Federation of American Societies for Experimental Biology, Royal Society of Medicine, and Society of Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

Chief Editor

Richard A Lange, MD  Professor and Executive Vice Chairman, Department of Medicine, Director, Office of Educational Programs, University of Texas Health Science Center at San Antonio

Richard A Lange, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Cardiology, American Heart Association, and Association of Subspecialty Professors

Disclosure: Nothing to disclose.

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Pulmonic stenosis. Echocardiogram of a patient with severe pulmonic stenosis. This image shows a parasternal short axis view of the thickened pulmonary valve.
Pulmonic stenosis. Echocardiogram of a patient with severe pulmonic stenosis. This image shows a Doppler scan of the peak velocity (5.2 m/s) and gradients (peak 109 mm Hg, mean 65 mm Hg) across the valve.
Pulmonic stenosis. Echocardiogram of a patient with severe pulmonic stenosis. This image shows that moderately severe pulmonary insufficiency (orange color flow) is also present.
This video is an echocardiogram of a patient with severe pulmonic stenosis. The first segment shows the parasternal short axis view of the thickened pulmonary valve. The second segment shows the presence of moderate pulmonary insufficiency (orange color flow).AV=Aortic valve, PV=Pulmonary valve, PA=Pulmonary artery, PI=Pulmonary insufficiency
 
 
 
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