Pediatric Rheumatic Heart Disease Follow-up

  • Author: Thomas K Chin, MD; Chief Editor: Stuart Berger, MD   more...
 
Updated: Aug 4, 2010
 

Further Outpatient Care

Patients usually show significant improvement after initiation of anti-inflammatory therapy. However, they should not be allowed to resume full activities until all clinical symptoms have abated and laboratory values have returned to normal levels.

The importance of prophylaxis against recurrent streptococcal pharyngitis and rheumatic fever should be emphasized with each patient. Each recurrent episode of rheumatic carditis produces further valve damage and increases the likelihood that valve replacement will be required. Patients should remain on antibiotic prophylaxis at least until their early twenties. Many physicians believe that lifelong prophylaxis is appropriate.

Patients should be examined regularly to detect signs of mitral stenosis, pulmonary hypertension, arrhythmias, and congestive heart failure.

Next

Deterrence/Prevention

Primary prevention of rheumatic fever consists of diagnosis and treatment of group A beta-hemolytic streptococcal pharyngitis.

Previous
Next

Complications

Potential complications include heart failure from valve insufficiency (acute rheumatic carditis) or stenosis (chronic rheumatic carditis). Associated cardiac complications include atrial arrhythmias, pulmonary edema, recurrent pulmonary emboli, infective endocarditis, intracardiac thrombus formation, and systemic emboli.

Previous
Next

Prognosis

Manifestations of acute rheumatic fever resolve over a period of 12 weeks in 80% of patients and may extend as long as 15 weeks in the remaining patients.

Rheumatic fever was the leading cause of death in people aged 5-20 years in the United States 100 years ago. At that time, the mortality rate was 8-30% from carditis and valvulitis but decreased to a 1-year mortality rate of 4% by the 1930s.

Following the development of antibiotics, the mortality rate decreased to almost 0% by the 1960s in the United States; however, it has remained 1-10% in developing countries. The development of penicillin has also affected the likelihood of developing chronic valvular disease after an episode of acute rheumatic fever. Before penicillin, 60-70% of patients developed valve disease as compared to 9-39% of patients since penicillin was developed.

In patients who develop murmurs from valve insufficiency from acute rheumatic fever, numerous factors, including the severity of the initial carditis, the presence or absence of recurrences, and the amount of time since the episode of rheumatic fever, affect the likelihood that valve abnormalities and the murmur will disappear. The type of treatment and the promptness with which treatment is initiated does not affect the likelihood of disappearance of the murmur. In general, the incidence of residual rheumatic heart disease at 10 years is 34% in patients without recurrences but 60% in patients with recurrent rheumatic fever. Disappearance of the murmur, when it occurs, happens within 5 years in 50% of patients. Thus, significant numbers of patients experience resolution of valve abnormalities even 5-10 years after their episode of rheumatic fever. The importance of preventing recurrences of rheumatic fever is evident.

Previous
Next

Patient Education

Emphasize the importance of prophylaxis against recurrent streptococcal pharyngitis and rheumatic fever with each patient.

For excellent patient education resources, visit eMedicine's Heart Center. Also, see eMedicine's patient education article Mitral Valve Prolapse.

Previous
 
Contributor Information and Disclosures
Author

Thomas K Chin, MD  Professor of Pediatrics, Chief of Pediatric Cardiology and Medical Director of the Pediatric Heart Institute, University of Tennessee College of Medicine; Director of Cardiology and Endowed Chair for Excellence in Cardiology, St Jude Children's Research Hospital

Thomas K Chin, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Heart Association

Disclosure: Nothing to disclose.

Coauthor(s)

Eric M Chin  California Institute of Technology

Disclosure: Nothing to disclose.

Tariq Siddiqui, MD  Staff Physician, Department of Anesthesiology, University of Louisville Medical Center

Disclosure: Nothing to disclose.

Ann-Kristin Sundell, MD  Staff Physician, Department of Pediatrics, East Tennessee State University

Ann-Kristin Sundell, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Specialty Editor Board

Jeffrey Allen Towbin, MD, MSc, FAAP, FACC, FAHA  Professor, Departments of Pediatrics (Cardiology), Cardiovascular Sciences, and Molecular and Human Genetics, Baylor College of Medicine; Chief of Pediatric Cardiology, Foundation Chair in Pediatric Cardiac Research, Texas Children's Hospital

Jeffrey Allen Towbin, MD, MSc, FAAP, FACC, FAHA is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American College of Cardiology, American College of Sports Medicine, American Heart Association, American Medical Association, American Society of Human Genetics, Cardiac Electrophysiology Society, New York Academy of Sciences, Society for Pediatric Research, Texas Medical Association, and Texas Pediatric Society

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Hugh D Allen, MD  Professor, Department of Pediatrics, Division of Pediatric Cardiology and Department of Internal Medicine, Ohio State University College of Medicine

Hugh D Allen, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Pediatric Society, American Society of Echocardiography, Society for Pediatric Research, Society of Pediatric Echocardiography, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Paul D Petry, DO, FACOP, FAAP  Consulting Staff, Freeman Pediatric Care, Freeman Health System

Paul D Petry, DO, FACOP, FAAP is a member of the following medical societies: American Academy of Osteopathy, American Academy of Pediatrics, American College of Osteopathic Pediatricians, and American Osteopathic Association

Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD  Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart Center, Children's Hospital of Wisconsin

Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, and Society for Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author Clyde Worley, MD, to the development and writing of this article.

References

  1. WHO. Rheumatic Fever and Rheumatic Heart Disease. 2004. WHO technical report series. [Full Text].

  2. Pickering LK, et al. 2009 Red Book: Report of the Committee on Infectious Diseases. 28th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2009:616-628.

  3. Guilherme L, Ramasawmy R, Kalil J. Rheumatic fever and rheumatic heart disease: genetics and pathogenesis. Scand J Immunol. Aug-Sep 2007;66(2-3):199-207. [Medline]. [Full Text].

  4. Marijon E, Ou P, Celermajer DS, et al. Prevalence of rheumatic heart disease detected by echocardiographic screening. N Engl J Med. Aug 2 2007;357(5):470-6. [Medline].

  5. AHA. Guidelines for the diagnosis of rheumatic fever. Jones Criteria, 1992 update. Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease of the Council. on Cardiovascular Disease in the Young, the American Heart Association;JAMA 1992 Oct 21; 268(15):2069-73. [Medline].

  6. Minich LL, Tani LY, Pagotto LT, Shaddy RE, Veasy LG. Doppler echocardiography distinguishes between physiologic and pathologic "silent" mitral regurgitation in patients with rheumatic fever. Clin Cardiol. Nov 1997;20(11):924-6. [Medline].

  7. 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. J Am Dent Assoc. Jan 2008;139 Suppl:3S-24S. [Medline]. [Full Text].

  8. 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]. [Full Text].

  9. Abernethy M, Bass N, Sharpe N, et al. Doppler echocardiography and the early diagnosis of carditis in acute rheumatic fever. Aust N Z J Med. Oct 1994;24(5):530-5. [Medline].

  10. Asbahr FR, Garvey MA, Snider LA, et al. Obsessive-compulsive symptoms among patients with Sydenham chorea. Biol Psychiatry. May 1 2005;57(9):1073-6. [Medline].

  11. Braunwald E. Rheumatic fever. In: Heart Disease: A Textbook of Cardiovascular Medicine. 5th ed. Philadelphia, Pa: WB Saunders Co; 1997.

  12. Carapetis JR, McDonald M, Wilson NJ. Acute rheumatic fever. Lancet. Jul 9-15 2005;366(9480):155-68. [Medline].

  13. Cilliers AM, Manyemba J, Saloojee H. Anti-inflammatory treatment for carditis in acute rheumatic fever. Cochrane Database Syst Rev. 2003;CD003176. [Medline].

  14. Clinical trial. The natural history of rheumatic fever and rheumatic heart disease. Ten-year report of a cooperative clinical trial of ACTH, cortisone, and aspirin. Circulation. Sep 1965;32(3):457-76. [Medline].

  15. Cotran RS, Kumar V, Collins T, Robbins SL. Robbins Pathologic Basis of Disease. 6th ed. Philadelphia, Pa: WB Saunders Co; 1999.

  16. Dajani A, Taubert K, Ferrieri P, et al. Treatment of acute streptococcal pharyngitis and prevention of rheumatic fever: a statement for health professionals. Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease of the Council. on Cardiovascular Disease in the Young, the American Heart Association;Pediatrics 1995 Oct; 96(4 Pt 1):758-64. [Medline].

  17. Doshi H, Shukla V, Korula RJ. Emergency valve replacement in rheumatic heart disease. J Heart Valve Dis. Jul 2003;12(4):516-9. [Medline].

  18. Ellis NM, Li Y, Hildebrand W, Cunningham MW. T cell mimicry and epitope specificity of cross-reactive T cell clones from rheumatic heart disease. J Immunol. Oct 15 2005;175(8):5448-56. [Medline].

  19. Fae KC, Oshiro SE, Toubert A, et al. How an autoimmune reaction triggered by molecular mimicry between streptococcal M protein and cardiac tissue proteins leads to heart lesions in rheumatic heart disease. J Autoimmun. Mar 2005;24(2):101-9. [Medline].

  20. Guilherme L, Fae K, Oshiro SE, Kalil J. Molecular pathogenesis of rheumatic fever and rheumatic heart disease. Expert Rev Mol Med. Dec 8 2005;7(28):1-15. [Medline].

  21. Massell BF, Fyler DC, Roy SB. The clinical picture of rheumatic fever: diagnosis, immediate prognosis, course, and therapeutic implications. Am J Cardiol. Apr 1958;1(4):436-49. [Medline].

  22. Narula J, Virmani R, Reddy KS, Tandon R. Rheumatic Fever. Washington DC: American Registry of Pathology; 1999.

  23. [Best Evidence] Rimoin AW, Hamza HS, Vince A, et al. Evaluation of the WHO clinical decision rule for streptococcal pharyngitis. Arch Dis Child. Oct 2005;90(10):1066-70. [Medline].

  24. Robertson KA, Volmink JA, Mayosi BM. Antibiotics for the primary prevention of acute rheumatic fever: a meta-analysis. BMC Cardiovasc Disord. May 31 2005;5(1):11. [Medline].

  25. Sampaio RO, Grinberg M, Leite JJ, et al. Effect of enalapril on left ventricular diameters and exercise capacity in asymptomatic or mildly symptomatic patients with regurgitation secondary to mitral valve prolapse or rheumatic heart disease. Am J Cardiol. Jul 1 2005;96(1):117-21. [Medline].

  26. Shrivastava S, Dev V, Vasan RS, et al. Percutaneous balloon mitral valvuloplasty in juvenile rheumatic mitral stenosis. Am J Cardiol. Apr 15 1991;67(9):892-4. [Medline].

  27. Swedo SE, Leonard HL, Garvey M, et al. Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections: clinical description of the first 50 cases [published erratum appears in Am J Psychiatry 1998 Apr;155(4):578]. Am J Psychiatry. Feb 1998;155(2):264-71. [Medline].

  28. Talwar S, Rajesh MR, Subramanian A, et al. Mitral valve repair in children with rheumatic heart disease. J Thorac Cardiovasc Surg. Apr 2005;129(4):875-9. [Medline].

  29. Veasy LG, Wiedmeier SE, Orsmond GS, et al. Resurgence of acute rheumatic fever in the intermountain area of the United States. N Engl J Med. Feb 19 1987;316(8):421-7. [Medline].

  30. Walker KG, Lawrenson J, Wilmshurst JM. Neuropsychiatric movement disorders following streptococcal infection. Dev Med Child Neurol. Nov 2005;47(11):771-5. [Medline].

 
Previous
Next
 
Parasternal long-axis view demonstrating the typical systolic mitral insufficiency jet observed with rheumatic heart disease (blue jet extending from the left ventricle into the left atrium). The jet is typically directed to the lateral and posterior wall. (LV=left ventricle; LA=left atrium; Ao=aorta; RV=right ventricle).
Parasternal long-axis view demonstrating the typical diastolic aortic insufficiency jet observed with rheumatic heart disease (red jet extending from the aorta into the left ventricle). (LV=left ventricle; LA=left atrium; Ao=aorta; RV=right ventricle).
 
 
 
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.