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Pulmonic Stenosis Follow-up

  • Author: Xiushui (Mike) Ren, MD; Chief Editor: Richard A Lange, MD, MBA  more...
 
Updated: Dec 22, 2014
 

Deterrence/Prevention

Infective endocarditis prophylaxis: The American Heart Association (AHA) Guidelines on Prevention of Bacterial Endocarditis considers all forms of isolated pulmonic stenosis (PS) to be in the moderate-risk category, and any PS associated with complex congenital heart disease to be in the high-risk category. Therefore, antibiotic prophylaxis is recommended for all forms of PS.

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Prognosis

Except for critically severe stenosis in neonates, survival is the rule for individuals with congenital PS.[6] The long-term course of individuals with mild PS is indistinguishable from that of the unaffected population. Mild PS does not tend to progress in severity; rather, pulmonic valve orifice size usually increases with body growth.

Severe PS may result in outflow obstruction that progresses over a period of years despite body growth (60% of patients require intervention within 10 y of diagnosis). With appropriate intervention, those with moderately severe PS have an excellent prognosis.

The functional effect of PS may change during an individual's lifetime such that symptoms or limitations occurring in childhood may resolve by adulthood.

Available data support relieving moderately severe and severe PS in childhood, with follow-up care through adolescence and into adulthood. When PS is corrected during childhood, the life expectancy of the affected individual matches that of the unaffected age- and sex-matched cohort. The more severe and protracted the course of PS, the less optimal the outcome of intervention, including death due to RV failure in the most severe cases.

Balloon valvuloplasty is preferred, provided the valve is compliant and mobile. Those with severe valvular fibrocalcific thickening are more likely to require a surgical approach. The recurrence rate of PS in patients who are treated surgically is approximately 4%. Long-term results of balloon valvuloplasty are comparable to the results of surgical repair, with the rate of recurrence of severe PS less than 5%. A recent study shows that long-term follow-up of patients after surgical treatment for isolated pulmonary valve stenosis resulted in a high rate of reinterventions (53% at a median follow-up of 34 years). Thus, close follow-up in postsurgical patients is needed.

Adult patients are more likely to present with subvalvular hypertrophic pulmonic stenosis or valvular fibrocalcific thickening. Secondary subvalvular hypertrophic stenosis regresses following correction of the primary valvular abnormality, and residual dilatation of the pulmonary trunk is not significant clinically, even when marked. Recognizing subvalvular hypertrophy is important, since it may lead to dynamic outflow obstruction during the acute phase following correction of valvular stenosis. With few exceptions, postvalvuloplasty pulmonic regurgitation is of mild-to-moderate severity.

A study by Zdradzinski et al indicated that specific guidelines for the timing of valve replacement need to be developed for patients with isolated PS who develop pulmonic regurgitation after surgical valvotomy. The study, which involved 109 adult patients, including 34 patients with isolated PS and 75 with tetralogy of Fallot, investigated whether recommendations for valve replacement in patients with tetralogy of Fallot who develop pulmonic regurgitation after complete repair can be applied when regurgitation develops after valvotomy for isolated PS.[7]

The report found that the degrees of pulmonic regurgitation and symptom severity were similar between the two groups of patients, but an analysis of biventricular systolic function and QRS width indicated that the morphologic changes that occur in association with tetralogy of Fallot and its repair involve more than just the effects of pulmonic regurgitation.[7]

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Contributor Information and Disclosures
Author

Xiushui (Mike) Ren, MD Cardiologist, The Permanente Medical Group; Associate Director of Research, Cardiovascular Diseases Fellowship, California Pacific Medical Center

Xiushui (Mike) Ren, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Cardiology, 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, Rhode Island Medical Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

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 Physiological Society, American Society for Clinical Investigation, American Society for Investigative Pathology, Association of American Physicians, Texas Medical Association, Southern Society for Clinical Investigation, American Federation for Clinical Research, Association of Professors of Medicine, Association of Professors of Cardiology, American Association for the Advancement of Science, American College of Cardiology, American College of Physicians-American Society of Internal Medicine, American Federation for Medical Research, American Heart Association

Disclosure: Received honoraria from Pfizer for speaking and teaching; Received honoraria from Merck for speaking and teaching; Received consulting fee from Orthoclinica Diagnostica for consulting.

Chief Editor

Richard A Lange, MD, MBA President, Texas Tech University Health Sciences Center, Dean, Paul L Foster School of Medicine

Richard A Lange, MD, MBA is a member of the following medical societies: Alpha Omega Alpha, American College of Cardiology, American Heart Association, Association of Subspecialty Professors

Disclosure: Nothing to disclose.

Additional Contributors

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.

References
  1. [Guideline] Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA, et al. ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease). Circulation. 2008 Dec 2. 118(23):e714-833. [Medline].

  2. Odenwald T, Taylor AM. Pulmonary valve interventions. Expert Rev Cardiovasc Ther. 2011 Nov. 9(11):1445-57. [Medline].

  3. [Guideline] Nishimura RA, Otto CM, Bonow RO, Carabello BA, Erwin JP 3rd, Guyton RA, et al. 2014 AHA/ACC Guideline 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. Circulation. 2014 Jun 10. 129(23):e521-643. [Medline].

  4. [Guideline] Vahanian A, Alfieri O, Andreotti F, Antunes MJ, Barón-Esquivias G, Baumgartner H, et al. Guidelines on the management of valvular heart disease (version 2012). Eur Heart J. 2012 Oct. 33(19):2451-96. [Medline].

  5. 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].

  6. Shaath G, Mutairi MA, Tamimi O, Alakhfash A, Abolfotouh M, Alhabshan F. Predictors of re-intervention in neonates with critical pulmonary stenosis or pulmonary atresia with intact ventricular septum. Catheter Cardiovasc Interv. 2011 Sep 27. [Medline].

  7. Zdradzinski MJ, Qureshi AM, Stewart R, et al. Comparison of long-term postoperative sequelae in patients with tetralogy of Fallot versus isolated pulmonic stenosis. Am J Cardiol. 2014 Jul 15. 114(2):300-4. [Medline]. [Full Text].

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