How Common Is Rheumatic Heart Disease?

Patient Presentation
A third year medical student noted to his attending physician that he was reading about rheumatic fever. He asked the attending how common it was in the United States. The attending said that the last time she had personally seen a case was during her medical school training in older patients who had had the disease as a child, and she knew of a case that had been transferred from a local facility in the past couple of years. She said, “You have to think about it, but it doesn’t happen very much in the U.S. thankfully, because of antibiotics, less overcrowding and public health measures.”

Acute rheumatic fever (ARF) is a nonsupprative, auto-inflammatory response after group A streptococcus (GAS) that affects multiple organs, including the heart. Chronic heart effects, particularly of the mitral and other heart valves, is termed rheumatic heart disease (RHD). ARF is thought to be caused by an autoimmune phenomenon where antibodies to the GAS cross react with normal host antigens. About 60% of ARF patients develop RHD and this is correlated to the initial carditis’s severity.

ARF symptoms can present 2-5 weeks after the GAS pharyngitis. Diagnosis of ARF includes 2 major, or 1 major and 2 minor Jones’ criteria in the clinical setting of a preceding GAS infection. The updated Jones’ criteria include:

  • Major criteria
    • Carditis – 30-45% of patients, endocarditis and pancarditis are usually seen. Valve disease usually begins with dilation and regurgitation, but chronic disease can lead to stenosis. Subclinical carditis is common.
    • Arthritis – 60-80% of patients – polyarticular, migratory arthritis, usually of large joints that commonly lasts less than 4 weeks.
    • Syndenham’s chorea – 10% of patients – a.k.a St. Vitus’s dance – purposeless, non-rhythmic involuntary movements usually of extremities and face that can also be accompanied by emotional lability and weakness.
    • Erythema marginatum – pink rash on trunk and extremities that is fleeting.
    • Subcutaneous nodules – painless nodules on extensor surfaces.
  • Minor criteria
    • Arthralgias
    • Fever – > 39° C.
    • Elevated inflammatory markers – erythrocyte sedimentation rate, C-reactive protein
    • Prolonged P-R interval on electrocardiogram
  • Evidence of GAS infection
    • Documented preceding streptococcal infection
    • Positive throat culture
    • Elevated or rising anti-streptolysin O titers.

ARF should be considered in the differential diagnosis of septic arthritis, juvenile idiopathic arthritis, reactive arthritis, patellofemoral syndrome and systemic lupus erythematosis among others.

Primary prevention is the best medicine by treating with antibiotics against GAS pharyngitis, improving access to medical care and social interventions for poverty. Potentially, vaccination may also assist primary prevention.

Secondary prevention is for those with histories of ARF or RHD at risk. Treatment is with daily antibiotics against GAS.

Learning Point

The most common cause of acquired heart disease in children and young adults is RHD.

Over the 20th century, the incidence and prevalence of ARF and RHD has decreased particularly in developed countries because of public health, sanitation, higher socio-economic status and improved medical care.
The highest rates of RHD are in developing countries. However, this is not always true. Australia and New Zealand have some of the highest rates when subpopulations are examined, mainly the First Nations peoples. In Australia the indigenous population incidence is 150-380 cases/100,000 population/year.
In the Maoris it is about 200 cases/year. Other subpopulations at risk include Pacific Islanders and people in Sub-Saharan Africa.

Overall the prevalence of RHD is estimated to be 15.6-19.6 million cases worldwide. About 233,000 deaths and 282,000 new cases are diagnosed each year.
Children and young adults are overrepresented in the statistics though with 2.4 million cases worldwide.
In the United States the incidence is <1 case per 100,000 in the pediatric population.

Questions for Further Discussion
1. How common is ARF and RHD in your location?
2. What treatment is recommended for ARF?
3. What is the role of echocardiograms for evaluation of possible ARF and RHD?
4. List other GAS infections.

Related Cases

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for these topics: Heart Valve Diseases and Streptococcal Infections.

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

Carapetis JR. Rheumatic Heart Disease in Developing Countries. NEJM. 2007;357;439-441.

Lee JL, Naguwa SM, Cheema GS, Gershwin ME. Acute rheumatic fever and its consequences: a persistent threat to developing nations in the 21st century. Autoimmun Rev. 2009 Dec;9(2):117-23.

Wilson N. Rheumatic heart disease in indigenous populations–New Zealand experience. Heart Lung Circ. 2010 May-Jun;19(5-6):282-8.

Parnaby MG, Carapetis JR. Rheumatic fever in indigenous Australian children. J Paediatr Child Health. 2010 Sep;46(9):527-33.

ACGME Competencies Highlighted by Case

  • Practice Based Learning and Improvement
    12. Evidence from scientific studies related to the patients’ health problems is located, appraised and assimilated.
    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.
    14. Knowledge of study designs and statistical methods to appraisal clinical studies and other information on diagnostic and therapeutic effectiveness is applied.
    15. Information technology to manage information, access on-line medical information and support the healthcare professional’s own education is used.
    16. Learning of students and other health care professionals is facilitated.

  • Interpersonal and Communication Skills
    17. A therapeutic and ethically sound relationship with patients is created and sustained.
    18. Using effective nonverbal, explanatory, questioning, and writing skills, the healthcare professional uses effective listening skills and elicits and provides information.
    19. The health professional works effectively with others as a member or leader of a health care team or other professional group.

  • Professionalism
    22. Sensitivity and responsiveness to patients’ culture, age, gender, and disabilities are demonstrated.


    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital