eMedicine Specialties > Emergency Medicine > Cardiovascular

Pulmonic Valvular Stenosis: Differential Diagnoses & Workup

Author: David J Wallace, MD, MPH, Critical Care Medicine Fellow, University of Pittsburgh Medical Center
Coauthor(s): Mert Erogul, MD, Assistant Professor of Emergency Medicine, University Hospital of Brooklyn: Consulting Staff, Department of Emergency Medicine, Kings County Hospital Center; Kurt Pflieger, MD, FAAP, Active Staff, Department of Pediatrics, Lake Pointe Medical Center
Contributor Information and Disclosures

Updated: Jul 3, 2008

Differential Diagnoses

Anemia, Acute
Pediatrics, Tachycardia
Aortic Stenosis
Tetralogy of Fallot
Pediatrics, Pneumonia
Pediatrics, Reactive Airway Disease
Pediatrics, Respiratory Distress Syndrome

Other Problems to Be Considered

Complex congenital heart disease associated with findings of pulmonary stenosis
Infundibular/subinfundibular stenosis
Supravalvular pulmonary stenosis
Dysplastic pulmonic valve stenosis

Workup

Laboratory Studies

  • Laboratory evaluation usually is not helpful.
  • Oximetry provides information on possible right-to-left shunting in borderline cyanotic lesions or in patients with anemia but does not identify the cause of the shunt (pulmonary, interatrial, interventricular, great arterial).
  • Although arterial blood gases (ABG) analysis usually is not needed, one notable exception is the hyperoxia test in newborns with cyanosis of undetermined origin.
  • Administered 100% FIO2 generally does not increase the partial pressure of oxygen to levels much greater than 100 mm Hg in patients with a cyanotic congenital heart defect.

Imaging Studies

  • Chest radiography
    • Chest radiographs demonstrate a prominent main pulmonary artery segment but a normal heart size.
    • Pulmonary vascular markings are usually normal but may be decreased in severe PVS.
    • CHF presents as cardiomegaly with right ventricular and right atrial enlargement in severe valvular pulmonary stenosis with or without tricuspid insufficiency.
  • Echocardiography9,10,11
    • The transthoracic approach provides valuable information about the site of obstruction and other possible congenital abnormalities.
    • Valve surface area is not used to determine the severity of stenosis. Rather, the peak gradient across the pulmonic valve is used as an indicator of disease severity. Doppler studies can accurately determine velocity of flow across the valve. The gradient is calculated from 4 times the peak systolic velocity squared: Pressure gradient = 4 X velocity squared
    • Multiple views and measurements increase the accuracy of the predicted peak systolic pressure gradient.
    • A thickened pulmonic valve with restricted systolic motion (doming) in the parasternal short axis view is apparent.
    • Frequently, the main pulmonary artery is dilated distal to the stenotic orifice.
    • Most children with pulmonary stenosis do not require further evaluation beyond echocardiography.
  • Whole-heart MRI
    • A case report from 2007 described the identification of a patient with isolated subvalvular pulmonary stenosis using whole-heart MRI.12 This imaging tool is noninvasive and has the added benefit of creating a 3-dimensional representation of the heart and surrounding structures.
    • Other reports have described the use of whole-heart MRI as a presurgical adjunct.13

Other Tests

  • Electrocardiography
    • ECG reflects the degree of right ventricular involvement.
    • ECG confirms right axis deviation and right ventricular hypertrophy in moderate valvular pulmonary stenosis.
    • Degree of right ventricular hypertrophy correlates with the severity of PVS.
    • Right atrial hypertrophy and right ventricular hypertrophy with strain pattern are observed when pulmonary stenosis is severe. A tall R wave in V1 more than 10 mm suggests severe stenosis.
    • ECG confirms superior QRS axis (left axis deviation) with dysplastic pulmonary valve and Noonan syndrome.
    • In the Second Natural History Study of Congenital Heart Defects, the rates of arrhythmias in patients with PVS were higher than expected compared with historical controls. The prevalence of "serious arrhythmias" was lower in patients with PVS than either aortic stenosis or ventricular septal defect.14

Procedures

  • Cardiac catheterization
    • The technique for angioplasty was described in 1982.15
    • This procedure is not indicated for mild PVS but is essential in severe stenosis.
    • Catheterization assesses the morphology of the right ventricle, pulmonary outflow tract, degree of tricuspid regurgitant flow, and pulmonary arteries.
  • Patients with echocardiographic evidence of significant PVS (>50 mm Hg) should undergo diagnostic and therapeutic cardiac catheterization. Percutaneous balloon dilatation, stenting, and pulmonic valve replacement16 are increasingly being performed with high success rates.17
  • Patients with infundibular or supravalvular pulmonic stenosis, if severe, require operative and invasive surgical interventions.
  • A surgical approach is often preferred in patients with Noonan syndrome because of to the degree of immobility that is often present.18

More on Pulmonic Valvular Stenosis

Overview: Pulmonic Valvular Stenosis
Differential Diagnoses & Workup: Pulmonic Valvular Stenosis
Treatment & Medication: Pulmonic Valvular Stenosis
Follow-up: Pulmonic Valvular Stenosis
References
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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. Fyler DC. Pulmonary stenosis. In: Nadas Pediatric Cardiology. 1992:459-70.

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

  4. 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].

  5. 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].

  6. 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].

  7. 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].

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

  9. 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].

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

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

  12. 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].

  13. 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].

  14. 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].

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

  16. 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].

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

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

  19. 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].

  20. 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].

  21. 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].

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

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

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

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Further Reading

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Keywords

pulmonary valvular stenosis, pulmonary valve stenosis, PVS, pulmonary stenosis, valvular, subvalvular, supravalvular, lesions, right ventricular outflow obstruction, Laurence-Moon-Biedl syndrome, Noonan syndrome, trisomy 21, cardiac malformations

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Contributor Information and Disclosures

Author

David J Wallace, MD, MPH, Critical Care Medicine Fellow, University of Pittsburgh Medical Center
David J Wallace, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American College of Emergency Physicians, American Medical Association, Emergency Medicine Residents Association, Society for Academic Emergency Medicine, and Society of Critical Care Medicine
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.

Medical Editor

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 Health Sciences Center
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.

Pharmacy Editor

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

Managing Editor

David Eitel, MD, MBA, Associate Professor, Department of Emergency Medicine, York Hospital
David Eitel, MD, MBA is a member of the following medical societies: American College of Emergency Physicians, Society for Academic Emergency Medicine, and Society of Critical Care Medicine
Disclosure: Nothing to disclose.

CME Editor

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.

 
 
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