By Philip R. Fischer, MD, DTM&H

Professor of Pediatrics, Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, MN

Dr. Fischer reports no financial relationships relevant to this field of study.

SYNOPSIS: With advances in access to good medical care, the global burden of rheumatic heart disease is declining. However, there still is significant disease in resource-limited regions of the world.

SOURCE: Watkins DA, Johnson CO, Colquhoun SM, et al. Global, regional, and national burden of rheumatic heart disease, 1990-2015. N Engl J Med 2017;377:713-722.

As part of the 2015 Global Burden of Disease study, Watkins and colleagues systematically reviewed data about non-fatal and fatal rheumatic heart disease over the course of 25 years, ending in 2015. The researchers determined estimates of prevalence and mortality.

Data indicate that there were approximately 33.4 million cases of rheumatic heart disease in 2015, with a cumulative total of 10.5 million disability-associated life-years due to rheumatic heart disease.

Rheumatic fever, the instigator of rheumatic heart disease, is considered to be a disease of poverty. Indeed, India, China, Pakistan, Indonesia, and the Democratic Republic of Congo account for three-fourths of the world’s new cases of rheumatic heart disease. With many people living with rheumatic heart disease, it is estimated that 1% of the people of South Asia and central Sub-Saharan Africa and 1.5% of those in Oceania are living with rheumatic heart disease.

From 1990 to 2015, the global mortality of rheumatic heart disease decreased by 48%, but this still represented 319,400 deaths in 2015. The highest mortality rates were found in Oceania, South Asia, and Central Africa. Pacific Island nations accounted for very high death rates, with more than 10 deaths per 100,000 population per year reported in the Solomon Islands, Papua New Guinea, Kiribati, Vanuatu, Fiji, the Federated States of Micronesia, and the Marshall Islands.


In the 1980s, the conventional teaching was that penicillin treatment for group A streptococcal pharyngitis reduced the risk of developing rheumatic fever but did not otherwise alter the clinical course of the illness. With rheumatic fever almost eradicated from the United States, there was legitimate academic discussion about whether it was even necessary to treat children with strep throat.1

Then, two things happened. First, in 1984, John Nelson finally published data from a study he had done in the 1950s.2 Randomized and placebo-controlled, the results showed clearly that penicillin treatment was associated with reductions in pharyngeal injection and cervical lymph node tenderness two days after initial evaluation, with improved resolution of fever, and with more rapid family assessment that the child had recovered. Second, there was a resurgence in the number of clinical cases of rheumatic fever in the western United States.3

Appropriate evaluation of febrile children with pharyngitis and early antibiotic treatment of those with positive streptococcal tests have continued in the United States, and rheumatic fever continues to be uncommon in the United States. However, rheumatic heart disease remains common in parts of the world where children are less likely to get care for acute illnesses.

An editorial accompanying Watkins’ paper was titled “Rheumatic Heart Disease — an Iceberg in Tropical Waters.”4 The editorialists pointed out that Watkins only included clinically evident cases of patients suffering from rheumatic heart disease and that echocardiographic screening usually finds three to 10 cases of subclinical rheumatic heart disease (the “submerged and hidden” part of the “iceberg”) for each clinical case. In one area of Kenya, for instance, local screening has demonstrated that 8% of schoolchildren have rheumatic heart disease.

What Can Be Done?

Multifaceted changes in healthcare infrastructure could help, and cardiac surgery could be made more available in resource-limited areas. There is already exploration in these directions in Kenya.5 At the same time, efforts are needed to stop the spread of streptococcal infections and to treat active infections prior to the onset of cardiac disease.6 The New England Journal of Medicine editorialists certainly agree with continuing to focus efforts on major diseases like tuberculosis, HIV, and malaria that each cause three to five times as many deaths as rheumatic heart disease. However, they cleverly point out that more than 500 times as much money is spent on research and development related to those diseases as for rheumatic heart disease. Maybe, as the authors suggest, now would be a good time for renewed scientific investigation and funding.4

In addition, other acquired cardiac conditions still plague children in resource-limited regions of the world, including tuberculous carditis and HIV cardiomyopathy.7 As prevention and management of streptococcal pharyngitis improves and cardiac care expands, there also would be benefit for children with other acquired cardiac conditions in settings where there is no cardiac surgery available.7 


  1. Shulman ST. Pharyngitis: Management in an Era of Declining Rheumatic Fever. New York: Praeger;1984.
  2. Nelson JD. The effect of penicillin therapy on the symptoms and signs of streptococcal pharyngitis. Pediatr Infect Dis 1984;3:10-13.
  3. 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 1987;316:421-427.
  4. Marijon E, Celermajer DS, Jouven X. Rheumatic heart disease — an iceberg in tropical waters. N Engl J Med 2017;377:780-781.
  5. Barasa FA, Vedanthan R, Pastakia SD, et al. Approaches to sustainable capacity building for cardiovascular disease care in Kenya. Cardiol Clin 2017;35:145-152.
  6. Nulu S, Bukhman G, Kwan GF. Rheumatic heart disease: The unfinished global agenda. Cardiol Clin 2017;35:165-180.
  7. Curry C, Zuhlke L, Mocumbi A, Kennedy N. Acquired heart disease in low-income and middle-income countries. Arch Dis Child 2017; in press, doi:10.1136/archdischild-2016-312521.