eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Rheumatic Heart Disease: Differential Diagnoses & Workup

Author: Thomas K Chin, MD, Associate Professor in Pediatrics, Chief of Pediatric Cardiology, University of Tennessee College of Medicine; Consulting Staff, Department of Pediatric Cardiology, St Jude Children's Research Center
Coauthor(s): Eric M Chin, California Institute of Technology; Tariq Siddiqui, MD, Staff Physician, Department of Anesthesiology, University of Louisville Medical Center; Ann-Kristin Sundell, MD, Staff Physician, Department of Pediatrics, East Tennessee State University
Contributor Information and Disclosures

Updated: Oct 10, 2008

Differential Diagnoses

Aortic Stenosis, Valvar
Human Immunodeficiency Virus Infection
Aortic Valve Insufficiency
Kawasaki Disease
Aortic Valve, Bicuspid
Mitral Stenosis, Congenital
Appendicitis
Mitral Valve Insufficiency
Arthritis, Septic
Mitral Valve Prolapse
Cardiac Tumors
Myocarditis, Viral
Cardiomyopathy, Dilated
Pericardial Effusion, Malignant
Carnitine Deficiency
Pericarditis, Bacterial
Coccidioidomycosis
Pericarditis, Viral
Endocarditis, Bacterial
Sarcoidosis
Heart Failure, Congestive
Systemic Lupus Erythematosus
Histoplasmosis
Transient Synovitis

Other Problems to Be Considered

Glomerulonephritis

Workup

Laboratory Studies

  • Throat culture: Throat culture findings for group A beta hemolytic Streptococcus are usually negative by the time symptoms of rheumatic fever or rheumatic heart disease appear. Attempts should be made to isolate the organism before the initiation of antibiotic therapy to help confirm a diagnosis of streptococcal pharyngitis and to allow typing of the organism if it is isolated successfully.
  • Rapid antigen detection test: This test allows rapid detection of group A streptococcal antigen and allows the diagnosis of streptococcal pharyngitis and the initiation of antibiotic therapy while the patient is still in the physician's office. Because the rapid antigen detection test has a specificity of greater than 95% but a sensitivity of only 60-90%, a throat culture should be obtained in conjunction with this test.
  • Antistreptococcal antibodies: The clinical features of rheumatic fever begin at the time antistreptococcal antibody levels are at their peak. Thus, antistreptococcal antibody testing is useful for confirming previous group A streptococcal infection. The elevated level of antistreptococcal antibodies is useful, particularly in patients that present with chorea as the only diagnostic criterion. Sensitivity for recent infections can be improved by testing for several antibodies. Antibody titers should be checked at 2-week intervals in order to detect a rising titer.
    • The most common extracellular antistreptococcal antibodies tested include antistreptolysin O (ASO), antideoxyribonuclease (DNAse) B, antihyaluronidase, antistreptokinase, antistreptococcal esterase, and anti-DNA. Antibody tests for cellular components of group A streptococcal antigens include antistreptococcal polysaccharide, antiteichoic acid antibody, and anti–M protein antibody.
    • In general, the ratio of antibodies to extracellular streptococcal antigens rises during the first month after infection and then plateaus for 3-6 months before returning to normal levels after 6-12 months. When the ASO titer peaks (2-3 wk after the onset of rheumatic fever), the sensitivity of this test is 80-85%. The anti-DNAse B has a slightly higher sensitivity (90%) for detecting rheumatic fever or acute glomerulonephritis. Antihyaluronidase results are frequently abnormal in rheumatic fever patients with a normal level of ASO titer and may rise earlier and persist longer than elevated ASO titers during rheumatic fever.
  • Acute phase reactants: The C-reactive protein and erythrocyte sedimentation rate are elevated in rheumatic fever due to the inflammatory nature of the disease. Both tests have a high sensitivity but low specificity for rheumatic fever. They may be used to monitor the resolution of inflammation, detect relapse when weaning aspirin, or identify the recurrence of disease.
  • Heart reactive antibodies: Tropomyosin is elevated in acute rheumatic fever.
  • Rapid detection test for D8/17: This immunofluorescence technique for identifying the B cell marker D8/17 is positive in 90% of patients with rheumatic fever. It may be useful for identifying patients who are at risk for developing rheumatic fever.

Imaging Studies

  • Chest roentgenography: Cardiomegaly, pulmonary congestion, and other findings consistent with heart failure may be seen on chest radiography. When the patient has fever and respiratory distress, chest radiography helps differentiate heart failure from rheumatic pneumonia.
  • Doppler-echocardiogram: In acute rheumatic heart disease, Doppler-echocardiography identifies and quantitates valve insufficiency and ventricular dysfunction. Studies in Cambodia and Mozambique demonstrated a 10-fold increase in the prevalence of rheumatic heart disease when echocardiography is used for clinical screening compared with strictly clinical findings.3
    • With mild carditis, Doppler evidence of mitral regurgitation may be present during the acute phase of disease but resolves in weeks to months. In contrast, patients with moderate-to-severe carditis have persistent mitral and/or aortic regurgitation.
    • The most important echocardiographic features of mitral regurgitation from acute rheumatic valvulitis are annular dilatation, elongation of the chordae to the anterior leaflet, and a posterolaterally directed mitral regurgitation jet.
    • During acute rheumatic fever, the left ventricle is frequently dilated in association with a normal or increased fractional shortening. Thus, some cardiologists believe that valve insufficiency (from endocarditis), rather than myocardial dysfunction (from myocarditis), is the dominant cause of heart failure in acute rheumatic fever.
    • In chronic rheumatic heart disease, echocardiography may be used to track the progression of valve stenosis and may help determine the time for surgical intervention. The leaflets of affected valves become diffusely thickened, with fusion of the commissures and chordae tendineae. Increased echodensity of the mitral valve may signify calcification.
  • Heart catheterization: In acute rheumatic heart disease, this procedure is not indicated. With chronic disease, heart catheterization has been performed to evaluate mitral and aortic valve disease and to balloon stenotic mitral valves.
    • Postcatheterization precautions include hemorrhage, pain, nausea and vomiting, and arterial or venous obstruction from thrombosis or spasm.
    • Complications may include mitral insufficiency after balloon dilation of the mitral valve, tachyarrhythmias, bradyarrhythmias, and vascular occlusion.

Other Tests

  • On ECG, sinus tachycardia most frequently accompanies acute rheumatic heart disease. Alternatively, some children develop sinus bradycardia from increased vagal tone. No correlation between bradycardia and the severity of the carditis is noted.
  • First-degree atrioventricular (AV) block (prolongation of the PR interval) is observed in some patients with rheumatic heart disease. This abnormality may be related to localized myocardial inflammation involving the AV node or to vasculitis involving the AV nodal artery. First-degree AV block is a nonspecific finding and should not be used as a criterion for the diagnosis of rheumatic heart disease. Its presence does not correlate with the development of chronic rheumatic heart disease.
  • Second-degree (intermittent) and third-degree (complete) AV block with progression to ventricular standstill have been described. Heart block in the setting of rheumatic fever, however, typically resolves with the rest of the disease process.
  • When acute rheumatic fever is associated with pericarditis, ST segment elevation may be present and is marked most in lead II, III, aVF, and V4 -V6.
  • Patients with rheumatic heart disease also may develop atrial flutter, multifocal atrial tachycardia, or atrial fibrillation from chronic mitral valve disease and atrial dilation.

Histologic Findings

  • Pathologic examination of the insufficient valves may reveal verrucous lesions at the line of closure.
  • Aschoff bodies (perivascular foci of eosinophilic collagen surrounded by lymphocytes, plasma cells, and macrophages) are found in the pericardium, perivascular regions of the myocardium, and endocardium. The Aschoff bodies assume a granulomatous appearance with a central fibrinoid focus and eventually are replaced by nodules of scar tissue.
  • Anitschkow cells are plump macrophages within Aschoff bodies.
  • In the pericardium, fibrinous and serofibrinous exudates may produce an appearance of "bread and butter" pericarditis.

More on Rheumatic Heart Disease

Overview: Rheumatic Heart Disease
Differential Diagnoses & Workup: Rheumatic Heart Disease
Treatment & Medication: Rheumatic Heart Disease
Follow-up: Rheumatic Heart Disease
Multimedia: Rheumatic Heart Disease
References
[ CLOSE WINDOW ]

References

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

  2. 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].

  3. 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].

  4. 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].

  5. 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].

  6. 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].

  7. 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].

  8. 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].

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

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

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

  12. 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].

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

  14. 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].

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

  16. 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].

  17. 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].

  18. 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].

  19. 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].

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

  21. Pickering LK, et al. 2000 Red Book: Report of the Committee on Infectious Diseases. 25th ed. Elk Grove Village, IL: American Academy of Pediatrics; 2000.

  22. [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].

  23. 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].

  24. 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].

  25. 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].

  26. 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].

  27. 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].

  28. 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].

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

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

rheumatic heart disease, acute rheumatic carditis, rheumatic fever, pharyngitis, heart failure, valve insufficiency, pericarditis, chronic rheumatic heart disease, valve stenosis, regurgitation, atrial dilation, arrhythmias, ventricular dysfunction, mitral valve stenosis, valve replacement, pancarditis, group A beta-hemolytic Streptococcus, Streptococcus pyogenes, impetigo, cellulitis, myositis, pneumonia, sepsis, endocarditis, myocarditis, polyarthritis, chorea, subcutaneous nodules, erythema marginatum, chest pain, edema, orthopnea, mitral insufficiency, congestive heart failure, Carey-Coombs murmur, hepatomegaly, arthralgias, epistaxis, Huntington chorea, pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections, PANDAS, Sydenham chorea, aortic stenosis

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Thomas K Chin, MD, Associate Professor in Pediatrics, Chief of Pediatric Cardiology, University of Tennessee College of Medicine; Consulting Staff, Department of Pediatric Cardiology, St Jude Children's Research Center
Thomas K Chin, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Society for Pharmacology and Experimental Therapeutics, American Society of Echocardiography, and Society for Pediatric Research
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.

Medical Editor

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, Heart Rhythm Society, New York Academy of Sciences, Society for Pediatric Research, Texas Medical Association, and Texas Pediatric Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

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.

CME Editor

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.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
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.