Pulmonic Valvular Stenosis Follow-up

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

Further Inpatient Care

  • Patients with mild pulmonic valvular stenosis rarely require treatment.
  • Intervention with either balloon angioplasty or valve repair is indicated for patients with peak valve gradients more than 50 mm Hg or for patients with angina, syncope, exertional dyspnea, or presyncope. Corrective options include open heart surgery, balloon angioplasty, percutaneous stenting, percutaneous valve replacement, or percutaneous conduit placement.[6]
  • Patients with severe or symptomatic infundibular or supravalvular pulmonary stenosis require surgical intervention.[6]
  • Critical pulmonic valvular stenosis may present with near pulmonary atresia (a cyanotic lesion) with a small and often inadequate right ventricle. These patients survive because of a patent ductus arteriosus. Pulmonary valve atresia or critical pulmonic valvular stenosis with inadequate right ventricle requires a shunt (usually modified Blalock-Taussig or central shunt) after the ductus is kept patent pharmacologically with prostaglandin E1.[6] Definitive repair may not be possible if the right ventricle is hypoplastic, requiring a single ventricular palliation, such as the Fontan procedure, or a variation, such as a direct right atrial appendage to main pulmonary artery anastomosis.[2] Frequently, the main and branch pulmonary arteries require augmentation.
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Further Outpatient Care

  • Patients should maintain normal physical activity.
  • Most patients with murmurs are given prophylaxis against infective subacute bacterial endocarditis (SBE).[30] Opinions differ about the need for SBE prophylaxis recommendations for patients with pulmonic valvular stenosis because of the extremely low incidence of endocarditis in this relatively large subpopulation.[30]
  • For patients older than 6 months with a gradient less than 40 mm Hg at the time of diagnosis, follow-up care can safely be performed at intervals of 2 years or more.
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Transfer

  • Patients with symptomatic pulmonic valvular stenosis should be transferred to a tertiary care center offering pediatric cardiology and pediatric cardiothoracic surgery.
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Complications

Complications of pulmonic valvular stenosis may include the following:

  • Sustained infundibular obstruction after valvular stenosis repair by surgery or valvuloplasty[4]
  • Late atrial arrhythmias[6]
  • Persistent repolarization abnormalities
  • Iatrogenic injury from balloon angioplasty or stent delivery
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Prognosis

  • Mild pulmonic valvular stenosis has a good overall prognosis. Life expectancy approaches that of someone without valvular disease.[9]
  • Patients with moderately severe to severe stenosis have clinically progressing disease. The survival rate for severe stenosis is 96%; however, mean follow-up over a period of 33 years suggest that 53% of patients had required further intervention. Forty percent may have atrial or ventricular arrhythmias.[6, 11]
  • Following balloon or surgical valvulotomy, outcome generally is excellent. After interventions to relieve the stenosis, stenosis usually does not recur and right ventricular hypertrophy often regresses.[6]
  • A 2007 study presented long-term follow-up data on 90 adult patients who had pulmonary balloon valvuloplasty. In this cohort, outcome data were excellent; this study supports the use of balloon angioplasty in these patients, even if there is an associated tricuspid regurgitant lesion or infundibular stenosis.[31]
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Patient Education

  • Patients and parents of those with mild pulmonic valvular stenosis should be reassured that this condition is not related to, or associated with, coronary artery disease, or sudden death. There is a mild association with benign dysrhythmia.[4, 9]
  • The disease is progressive, and isolated mild pulmonic valvular stenosis may not cause symptoms until age 40 years or older.[6]
  • If the patient is asymptomatic with mild pulmonic valvular stenosis, annual screening examination and ECG should be scheduled. Follow up every 3-5 years may be initiated if subsequent evaluations show no change.[6]
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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].

 
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