Pulmonic Valvular Stenosis Clinical Presentation

  • Author: Allysia M Guy, MD; Chief Editor: Robert E O'Connor, MD, MPH   more...
 
Updated: Aug 16, 2010
 

History

Adults presenting with symptoms of progressive outflow tract obstruction due to pulmonic valvular disease were most likely born with some congenital malformation that was not diagnosed as a child.[4]

Signs of pulmonic valvular stenosis include the following:

  • History of a heart murmur since birth
  • Cyanosis
  • Dyspnea
  • Fatigue
  • Dizziness or syncope, occasionally
  • Chest pain
  • Mental retardation/developmental disorders
Next

Physical

Physical examination findings correlate with the severity of right ventricular outflow obstruction.

  • The first heart sound is normal and followed by a systolic ejection click. The systolic ejection click is variable with respiration and louder on expiration. It is loudest over the left upper sternal border.[1, 6] The murmur of pulmonic stenosis is of the systolic ejection type and is best heard at the second left intercostal space.[11]
    • Patients with dysplastic valves may not have a systolic ejection click. If the valve is pliant, a systolic ejection click is often heard.
    • The second heart sound is split. This is due to delayed closing of the pulmonic valve at the end of systole. The pulmonic component of the second heart sound may be diminished in intensity.
  • Systolic ejection murmur (crescendo-decrescendo), grade 2-5/6, is audible at the left upper sternal border, transmitting into the back and posterior lung fields. The murmur is heard best in the first to third intercostal spaces.[3, 6] The murmur usually does not radiate to the left sternal boarder.
  • Severity of valvular disease is related directly to the intensity and duration of the murmur. When severe, murmur extends into diastole (beyond the second heart sound).
  • Severe pulmonic valvular stenosis is associated with tricuspid insufficiency and may be associated with elevated central venous pressure, hepatosplenomegaly, a pulsatile liver, jugular venous pulsations, and hepatojugular reflux. Hepatosplenomegaly may develop in cases of CHF.
  • Significant pulmonic stenosis is characterized by a prominent jugular venous a wave and a right ventricular lift
  • Myocardial infarction of hypertrophied right ventricle may occur.[16]
  • Cyanosis may occur with right-to-left shunting at the atrial level as with a patent foramen ovale or septal defect.[11]
Previous
Next

Causes

Pulmonic valvular stenosis primarily results from a maldevelopment of the pulmonic valve tissue and the distal portion of the bulbus cordis. One maldevelopment is characterized by fusion of leaflet commissures, resulting in a domed appearance to the valve. Other etiologies result in dysplastic valves, which do not open and close normally.[16]

  • Coexisting cardiac malformations (eg, ventricular septal defect, atrial septal defect, patent ductus arteriosus) may complicate the anatomy, physiology, and clinical picture.
  • Aberrant flow patterns in utero may be associated, in part, with maldevelopment of the pulmonary valve.
  • Rubella embryopathy may cause pulmonic valvular stenosis.
  • Family history is a mild risk factor.[17]
  • Cases have been reported in the setting of congenital syndromes such as Mayer-Rokitansky-Kuster-Hauser syndrome.[18]
Previous
Proceed to Differential Diagnoses
 
 
Contributor Information and Disclosures
Author

Allysia M Guy, MD  Staff Physician, Department of Emergency Medicine, State University of New York Downstate Medical Center

Disclosure: Nothing to disclose.

Coauthor(s)

Mert Erogul, MD  Assistant Professor of Emergency Medicine, University Hospital of Brooklyn: Consulting Staff, Department of Emergency Medicine, Kings County Hospital Center

Mert Erogul, MD is a member of the following medical societies: American College of Emergency Physicians, American Medical Association, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Kurt Pflieger, MD, FAAP  Active Staff, Department of Pediatrics, Lake Pointe Medical Center

Kurt Pflieger, MD, FAAP is a member of the following medical societies: American Academy of Pediatrics, American College of Emergency Physicians, American Heart Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Peter MC DeBlieux, MD  Professor of Clinical Medicine and Pediatrics, Section of Pulmonary and Critical Care Medicine, Program Director, Department of Emergency Medicine, Louisiana State University School of Medicine in New Orleans

Peter MC DeBlieux, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Emergency Medicine, American College of Emergency Physicians, American Medical Association, Radiological Society of North America, and Society of Critical Care Medicine

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

David Eitel, MD, MBA  Associate Professor, Department of Emergency Medicine, York Hospital; Physician Advisor for Case Management, Wellspan Health System, York

David Eitel, MD, MBA is a member of the following medical societies: American College of Emergency Physicians, American Society of Pediatric Nephrology, Society for Academic Emergency Medicine, and Society of Critical Care Medicine

Disclosure: Nothing to disclose.

John D Halamka, MD, MS  Associate Professor of Medicine, Harvard Medical School, Beth Israel Deaconess Medical Center; Chief Information Officer, CareGroup Healthcare System and Harvard Medical School; Attending Physician, Division of Emergency Medicine, Beth Israel Deaconess Medical Center

John D Halamka, MD, MS is a member of the following medical societies: American College of Emergency Physicians, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Chief Editor

Robert E O'Connor, MD, MPH  Professor and Chair, Department of Emergency Medicine, University of Virginia Health System

Robert E O'Connor, MD, MPH is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians, American College of Physician Executives, American Heart Association, American Medical Association, Medical Society of Delaware, National Association of EMS Physicians, Society for Academic Emergency Medicine, and Wilderness Medical Society

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author, David J Wallace, MD, MPH, to the development and writing of this article.

References

  1. Blount SG, Komesu S, McCord MC. Asymptomatic isolated valvular pulmonary stenosis; diagnosis by clinical methods. N Engl J Med. Jan 1 1953;248(1):5-11. [Medline].

  2. Perloff JK, Child JS, Aboulhosn J. Pulmonic valvular disease. In: Congenital Heart Disease in Adults. 3rd ed. Saunders; 2008.

  3. Fyler DC. Pulmonary stenosis. In: Nadas Pediatric Cardiology. 1992:459-70.

  4. Inglessis I, Landzberg MJ. Interventional catheterization in adult congenital heart disease. Circulation. Mar 27 2007;115(12):1622-33. [Medline].

  5. Armstrong EJ, Bischoff J. Heart valve development: endothelial cell signaling and differentiation. Circ Res. Sep 3 2004;95(5):459-70. [Medline]. [Full Text].

  6. Libby P, Bonow RO, Mann DL, Zipes DP. Braunwald's Heart Disease; A Textbook of Cardiovascular Medicine. 8th ed. Saunders Inc; 2007.

  7. Hatemi AC, Gursoy M, Tongut A, Bicakhan B, Guzeltas A, Cetin G. Pulmonary stenosis as a predisposing factor for infective endocarditis in a patient with Noonan syndrome. Tex Heart Inst J. 2010;37(1):99-101. [Medline].

  8. Diller GP, van Eijl S, Okonko DO, Howard LS, Ali O, Thum T. Circulating endothelial progenitor cells in patients with Eisenmenger syndrome and idiopathic pulmonary arterial hypertension. Circulation. Jun 10 2008;117(23):3020-30. [Medline].

  9. Nishimura RA, Gersony et al. Second Natural History Study of Congenital Heart Defects. Circulation. Feb 1993;87:89-137.

  10. Jorge AA, Malaquias AC, Arnhold IJ, Mendonca BB. Noonan syndrome and related disorders: a review of clinical features and mutations in genes of the RAS/MAPK pathway. Horm Res. 2009;71(4):185-93. [Medline].

  11. Crawford. Pulmonic stenosis. In: Cardiology. 3rd ed. Mosby Inc; 2009.

  12. Nishimura RA, Pieroni DR, Bierman FZ, et al. Second natural history study of congenital heart defects. Pulmonary stenosis: echocardiography. Circulation. Feb 1993;87(2 Suppl):I73-9. [Medline].

  13. Driscoll DJ, Wolfe RR, Gersony WM, et al. Cardiorespiratory responses to exercise of patients with aortic stenosis, pulmonary stenosis, and ventricular septal defect. Circulation. Feb 1993;87(2 Suppl):I102-13. [Medline].

  14. Ardura J, Gonzalez C, Andres J. Does mild pulmonary stenosis progress during childhood? A study of its natural course. Clin Cardiol. Sep 2004;27(9):519-22. [Medline].

  15. Hameed AB, Goodwin TM, Elkayam U. Effect of pulmonary stenosis on pregnancy outcomes--a case-control study. Am Heart J. Nov 2007;154(5):852-4. [Medline].

  16. Moore K, Persaud T. The Developing Human. In: Clinically Oriented Embryology. 1998.

  17. Driscoll DJ, Michels VV, Gersony WM, et al. Occurrence risk for congenital heart defects in relatives of patients with aortic stenosis, pulmonary stenosis, or ventricular septal defect. Circulation. Feb 1993;87(2 Suppl):I114-20. [Medline].

  18. Kula S, Saygili A, Tunaoglu FS, Olguntürk R. Mayer-Rokitansky-Küster-Hauser syndrome associated with pulmonary stenosis. Acta Paediatr. Apr 2004;93(4):570-2. [Medline].

  19. Wolfe RR, Driscoll DJ, Gersony WM, et al. Arrhythmias in patients with valvar aortic stenosis, valvar pulmonary stenosis, and ventricular septal defect. Results of 24-hour ECG monitoring. Circulation. Feb 1993;87(2 Suppl):I89-101. [Medline].

  20. Silverman NH. Right heart obstructive lesions. In: Pediatric Echocardiography. 1993:327.

  21. Snider AR, Serwer GA. Abnormalities to right ventricular outflow. In: Echocardiography in Pediatric Heart Disease. 1990:231-41.

  22. Seibt C, Flender B, Gutberlet M. Comprehensive non-invasive pre-surgical magnetic resonance imaging in a patient with LEOPARD's syndrome cardiomyopathy. Eur Heart J. Jun 2006;27(12):1407. [Medline].

  23. Bettencourt N, Rocha J, Carvalho M, Leite D, Toschke AM, Melica B, et al. Multislice computed tomography in the exclusion of coronary artery disease in patients with presurgical valve disease. Circ Cardiovasc Imaging. Jul 2009;2(4):306-13. [Medline].

  24. Sato Y, Komatsu S, Matsuo S, et al. Isolated subvalvular pulmonary stenosis: depiction at whole heart magnetic resonance imaging. Int J Cardiovasc Imaging. Feb 2007;23(1):49-52. [Medline].

  25. Kan JS, White RI Jr, Mitchell SE, Gardner TJ. Percutaneous balloon valvuloplasty: a new method for treating congenital pulmonary-valve stenosis. N Engl J Med. Aug 26 1982;307(9):540-2. [Medline].

  26. Bonhoeffer P, Boudjemline Y, Qureshi SA, et al. Percutaneous insertion of the pulmonary valve. J Am Coll Cardiol. May 15 2002;39(10):1664-9. [Medline].

  27. Block PC, Bonhoeffer P. Percutaneous approaches to valvular heart disease. Curr Cardiol Rep. Mar 2005;7(2):108-13. [Medline].

  28. Castenada AR, Jonas RA, Meyer JE. Surgery for infants with congenital heart defects. In: Cardiac Surgery. 1993:1013-35.

  29. Gersony WM, Hayes CJ, Driscoll DJ, et al. Bacterial endocarditis in patients with aortic stenosis, pulmonary stenosis, or ventricular septal defect. Circulation. Feb 1993;87(2 Suppl):I121-6. [Medline].

  30. [Guideline] Wilson W, Taubert KA, Gewitz M, et al. Prevention of infective endocarditis: guidelines from the American Heart Association: a guideline from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. Circulation. Oct 9 2007;116(15):1736-54. [Medline].

  31. Fawzy ME, Hassan W, Fadel BM, 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. Mar 2007;153(3):433-8. [Medline].

  32. Almeda FQ, Kavinsky CJ, Pophal SG, Klein LW. Pulmonic valvular stenosis in adults: diagnosis and treatment. Catheter Cardiovasc Interv. Dec 2003;60(4):546-57. [Medline].

  33. Park MK. Pulmonary stenosis. In: Pediatric Cardiology for Practitioners. 145-7.

  34. Rocchini AP, Emmanouilides GC. Pulmonary stenosis. In: Heart Disease in Infants, Children and Adolescent: Including the Fetus and Young Adult. 930-62.

  35. Sarkozy A, Digilio MC, Dallapiccola B. Leopard syndrome. Orphanet J Rare Dis. May 27 2008;3:13. [Medline]. [Full Text].

 
Previous
Next
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
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.