eMedicine Specialties > Cardiology > Valvular Heart Disease

Pulmonic Stenosis: Differential Diagnoses & Workup

Author: Xiushui (Mike) Ren, MD, Clinical Cardiology Fellow, Division of Cardiology, Kanbar Cardiac Center, California Pacific Medical Center
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
Contributor Information and Disclosures

Updated: Jul 15, 2009

Differential Diagnoses

Benign Cardiac Tumors
Cardiac Neoplasms, Primary
Cardiac Sarcoma
Sinus of Valsalva Aneurysm

Other Problems to Be Considered

Congenital heart abnormality (see Causes)
Rheumatic valvular heart disease
Carcinoid heart disease

Workup

Imaging Studies

  • Plain chest radiographs
    • A characteristic radiographic finding, even with mild valvular pulmonic stenosis (PS), is prominence of the main, right, or left pulmonary arteries caused by poststenotic dilatation.
    • The intrapulmonary vasculature usually appears normal, even in severe PS.
    • In critical PS, the pulmonary vasculature may appear decreased if significant right-to-left shunting occurs through a patent foramen ovale or atrial septal defect or if severe unilateral pulmonary artery branch obstruction is present.
    • The overall heart size usually is normal unless RV failure or tricuspid regurgitation develops.
    • A prominent right heart border suggesting right atrial enlargement may be present in as many as 50% of affected individuals.
  • Echocardiography
    • Echocardiography provides a definitive confirmation of the diagnosis of PS. Both 2-dimensional and Doppler techniques should be used to comprehensively evaluate the pulmonic valve.
    • Using 2-dimensional imaging, thickening of the valves, characteristic doming of nondysplastic valves, and right ventricular (RV) hypertrophy can be noted readily (see image below). RV size and systolic function, right atrial (RA) size, and pulmonary artery dimensions can be quantified in most patients.

    • Pulmonic stenosis. Echocardiogram of a patient wi...

      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 wi...

      Pulmonic stenosis. Echocardiogram of a patient with severe pulmonic stenosis. This image shows a parasternal short axis view of the thickened pulmonary valve.

    • Color Doppler aids in both defining high velocity jets and localizing their origin. Pulsed waved Doppler (placed just proximal to the site of obstruction) and continuous wave Doppler are used to measure jet velocity, which can be converted to pressure gradient using the modified Bernoulli equation. Normally, no systolic gradient is present across the pulmonic valve. With PS, however, the RV systolic pressure increases and a pressure gradient occurs between the RV and pulmonary artery. Doppler studies of the stenotic valve can determine the severity of the gradient (see image below).

    • Pulmonic stenosis. Echocardiogram of a patient wi...

      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 wi...

      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.


      • The pulmonary valve area of a healthy adult is 2.0 cm2/m2 of body surface area.
      • Mild valvular PS is defined by a valve area larger than 1 cm2 and a transvalvular pressure gradient of less than 50 mm Hg.
      • Moderately severe PS occurs if the valve area is 0.5-1.0 cm2, with a transvalvular pressure gradient between 50 and 75 mm Hg.
      • Severe PS is defined by a valve area smaller than 0.5 cm2 and a transvalvular pressure gradient greater than 75 mm Hg.
    • RV hypertrophy with asymmetric septal hypertrophy may be present. In addition, restrictive physiology can be demonstrated by Doppler interrogations of tricuspid inflow, hepatic vein flow, and Doppler tissue imaging. A restrictive RV pattern is associated with worse RV systolic function and worse exercise tolerance.
    • Doppler evidence of right-sided pressures approaching or exceeding systemic pressures or a 2-dimensional echocardiogram demonstrating paradoxical septal motion during systole with reversal of the usual right convex curvature of the interventricular septum is an indication for therapeutic intervention (see image and video below).

    • Pulmonic stenosis. Echocardiogram of a patient wi...

      Pulmonic stenosis. Echocardiogram of a patient with severe pulmonic stenosis. This image shows that moderately severe pulmonary insufficiency (orange color flow) is also present.

      Pulmonic stenosis. Echocardiogram of a patient wi...

      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 feature requires the newest version of Flash. You can download it here.

      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

      This video is an echocardiogram of a patient with...

      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

  • Cardiac catheterization and pulmonary angiography
    • Cardiac catheterization generally is not needed to verify the findings of noninvasive tests, except when a significant discrepancy is noted between clinical findings and echocardiographic findings. When performed, cardiac catheterization can diagnose PS by a pressure gradient from the pulmonary artery to the RV on "pull-back" of the pulmonary artery catheter. Cardiac catheterization may be useful in assessing the presence of concomitant congenital abnormalities.
    • In the case of isolated PPS, pulmonary angiography may be needed to establish the diagnosis.

Other Tests

  • Electrocardiogram
    • The degree of (right) ventricular hypertrophy on the ECG is largely correlated directly with the severity of PS.
    • With mild PS, 50% of patients have a normal ECG tracing or only mild right-axis deviation.
    • With moderately severe PS, right-axis deviation and increased R-wave amplitude in V1 are seen.
    • Severe PS is associated with extreme right-axis deviation, a dominant R wave in AVR, and a prominent R wave (>20 mm) in V1.

More on Pulmonic Stenosis

Overview: Pulmonic Stenosis
Differential Diagnoses & Workup: Pulmonic Stenosis
Treatment & Medication: Pulmonic Stenosis
Follow-up: Pulmonic Stenosis
Multimedia: Pulmonic Stenosis
References

References

  1. Hameed AB, Goodwin TM, Elkayam U. Effect of pulmonary stenosis on pregnancy outcomes--a case-control study. Am Heart J. November 2007;154:852. [Medline][Full Text].

  2. Brickner ME, Hillis LD, Lange RA. Congenital heart disease in adults. First of two parts. N Engl J Med. Jan 27 2000;342(4):256-63. [Medline].

  3. Brickner ME, Hillis LD, Lange RA. Congenital heart disease in adults. Second of two parts. N Engl J Med. Feb 3 2000;342(5):334-42. [Medline].

  4. Cabrera, A, Martinez, P, Rumoroso, J. R. Double-chambered right ventricle. Eur Heart J. 1995;16:682-6. [Full Text].

  5. Chen CR, Cheng TO, Huang T, et al. Percutaneous balloon valvuloplasty for pulmonic stenosis in adolescents and adults. N Engl J Med. Jul 4 1996;335(1):21-5. [Medline].

  6. Perloff JK, Child JS, eds. Congenital Heart Disease in Adults. Philadelphia: WB Saunders; 1998.

  7. Connelly MS, Webb GD, Somerville J, et al. Canadian Consensus Conference on Adult Congenital Heart Disease 1996. Can J Cardiol. Mar 1998;14(3):395-452. [Medline].

  8. Dajani AS, Taubert KA, Wilson W, et al. Prevention of bacterial endocarditis. Recommendations by the American Heart Association. Circulation. Jul 1 1997;96(1):358-66. [Medline].

  9. Earing MG, Connolly HM, Dearani JA, et al. Long-term follow-up of patients after surgical treatment for isolated pulmonary valve stenosis. Mayo Clin Proc. Jul 2005;80(7):871-6. [Medline].

  10. Fawzy ME, Hassan W, Fadel BM, Sergani H, El Shaer F, El Widaa H, et al. Long-term results (up to 17 years) of pulmonary balloon valvuloplasty in adults and its effects on concomitant severe infundibular stenosis and tricuspid regurgitation. Am Heart J. March 2007;153:433-8. [Medline][Full Text].

  11. Lam YY, Kaya MG, Goktekin O, Gatzoulis MA, Li W, Henein MY. Restrictive right ventricular physiology: its presence and symptomatic contribution in patients with pulmonary valvular stenosis. J Am Coll Cardiol. October 2007;50:1491-7. [Medline][Full Text].

  12. Lip GY, Singh SP, de Giovanni J. Percutaneous balloon valvuloplasty for congenital pulmonary valve stenosis in adults. Clin Cardiol. Nov 1999;22(11):733-7. [Medline].

  13. Mack G, Silberbach M. Aortic and pulmonary stenosis. Pediatr Rev. Mar 2000;21(3):79-85. [Medline].

  14. McCrindle BW. Independent predictors of long-term results after balloon pulmonary valvuloplasty. Valvuloplasty and Angioplasty of Congenital Anomalies (VACA) Registry Investigators. Circulation. Apr 1994;89(4):1751-9. [Medline].

  15. Mitropoulos FA, Laks H, Kapadia N, Gurvitz M, Levi D, Williams R, et al. Intraoperative pulmonary artery stenting: an alternative technique for the management of pulmonary artery stenosis. Ann Thorac Surg. October 2007;84:1338-41. [Medline][Full Text].

  16. Noonan JA. Hypertelorism with Turner phenotype. A new syndrome with associated congenital heart disease. Am J Dis Child. Oct 1968;116(4):373-80. [Medline].

  17. Silvilairat S, Cabalka AK, Cetta F, Hagler DJ, O'Leary PW. Outpatient echocardiographic assessment of complex pulmonary outflow stenosis: Doppler mean gradient is superior to the maximum instantaneous gradient. J Am Soc Echocardiogr. Nov 2005;18(11):1143-8. [Medline].

  18. Task Force on Practice Guidelines. ACC/AHA guidelines for the management of patients with valvular heart disease. A report of the American College of Cardiology/American Heart Association. Task Force on Practice Guidelines (Committee on Management of Patients with Valvular Heart Disease. J Am Coll Cardiol. Nov 1998;32(5):1486-588. [Medline].

  19. Teerlink JR, Foster E. Valvular heart disease in pregnancy. A contemporary perspective. Cardiol Clin. Aug 1998;16(3):573-98, x. [Medline].

  20. Therrien J, Gatzoulis M, Graham T, et al. Canadian Cardiovascular Society Consensus Conference 2001 update: Recommendations for the Management of Adults with Congenital Heart Disease--Part II. Can J Cardiol. Oct 2001;17(10):1029-50. [Medline].

Further Reading

Keywords

pulmonic stenosis, pulmonary valve stenosis, PS, peripheral pulmonic stenosis, PPS, congenital heart anomalies, valvular PS, atrial septal defect, ASD, ventricular septal defect, VSD, patent ductus arteriosus, congenital heart disease, congenital heart defect

Contributor Information and Disclosures

Author

Xiushui (Mike) Ren, MD, Clinical Cardiology Fellow, Division of Cardiology, Kanbar Cardiac Center, California Pacific Medical Center
Xiushui (Mike) Ren, MD 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.

Medical Editor

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.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

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

CME Editor

Amer Suleman, MD, Consultant in Electrophysiology and Cardiovascular Medicine, Department of Internal Medicine, Division of Cardiology, Medical City Dallas Hospital
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, 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.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.