eMedicine Specialties > Emergency Medicine > Cardiovascular

Pulmonic Valvular Stenosis: Follow-up

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

Follow-up

Further Inpatient Care

  • Patients with mild PVS rarely require treatment.
  • Intervention with either balloon angioplasty or valve repair is indicated for patients with peak valve gradients more then 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.
  • Patients with severe or symptomatic infundibular or supravalvular pulmonary stenosis require surgical intervention.
  • Critical PVS 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 PVS with inadequate right ventricle requires a shunt (usually modified Blalock-Taussig or central shunt) after the ductus is kept patent pharmacologically with prostaglandin E1.
    • 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.
    • Frequently, the main and branch pulmonary arteries require augmentation.

Further Outpatient Care

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

Transfer

  • Patients with symptomatic PVS should be transferred to a tertiary care center offering pediatric cardiology and pediatric cardiothoracic surgery.

Complications

  • One complication of acute palliation or relief of severe PVS involves hypercontractile, residual, obstructing muscular hypertrophy in the infundibulum.
    • Infundibular obstruction after valvular stenosis repair by surgery or valvuloplasty has led to the designation of suicide right ventricle.
    • This complication is more frequent in older patients with long-standing pulmonary stenosis.
  • Other complications include the following:
    • Late atrial arrhythmias
    • Persistent repolarization abnormalities
    • Iatrogenic injury from balloon angioplasty or stent delivery

Prognosis

  • Mild PVS usually does not progress. However, patients with moderately severe to severe disease tend to progress.
  • After relief of the stenosis, it does not usually recur, and right ventricular hypertrophy frequently regresses.
  • Following balloon or surgical valvulotomy, outcome generally is excellent.
  • 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.21

Patient Education

  • Patients and parents of those with mild PVS should be reassured that this condition is not related to, or associated with, coronary artery disease, dysrhythmia, or sudden death. Patients are no more at risk for disastrous health consequences than the general population.
  • Insurability may become a factor in obtaining further care.
  • If the patient is asymptomatic and acyanotic and has mild PVS evinced by initial Doppler echocardiography, an annual screening examination and ECG would be prudent follow-up.
  • If no significant change in the condition appears for a few years after the initial evaluation, the patient can be discharged reasonably for follow-up care over extended periods of 3-5 years.

Miscellaneous

Medicolegal Pitfalls

  • Failure to diagnose a more serious congenital heart defect, such as tetralogy of Fallot (tet), could yield disastrous consequences.
  • Acyanotic patients with tet and mild right ventricular outflow tract obstruction may have a similar presentation and physical examination findings.
  • Tet is a lesion that is surgically correctable; the repair can be performed safely even in the neonatal period.
  • Echocardiography can reliably confirm the precise diagnosis and differentiate between PVS and tet.
  • Echocardiography should not be withheld if any suspicion exists of a more complex anatomy.
 


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

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  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].

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  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].

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  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].

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

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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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