Top Things to Know: Contemporary Diagnosis and Management of Rheumatic Heart Disease

Published: October 19, 2020

  1. While the world-wide burden of RHD has declined over the last 30 years, rates remain high in poorer regions of the world where RHD remains endemic.
  2. In non-endemic regions, the burden is greatest among the poorest which include the rural poor and marginalized populations with little or no access to primary health care.
  3. There were an estimated 29.7 million to 43.1 million cases of RHD globally in 2015 with a prevalence of 444 cases per 100,000 population in endemic areas. In comparison, there were 3.4 cases per 100,000 population in non-endemic countries.
  4. This statement identifies gaps in diagnosis and treatment globally such as delays in diagnosis and treatment of group A streptococcal (GAS) infections, unrecognized, and under-treated subclinical mild definite RHD.
  5. Pragmatic strategies towards addressing the challenges faced in low resource environments are discussed, including active screening and case finding, regional registries, and timely prophylaxis.
  6. The most suitable target populations for intervention are school-aged children and pregnant women. The incidence of primary episode acute rheumatic fever (ARF) is highest in the 5 -15 years old age group. Previously undetected latent RHD poses a special risk during pregnancy; if the disease is severe, it could compromise the life of the mother and the baby.
  7. Auscultation with a stethoscope is neither sensitive nor specific enough for RHD and hence not a suitable screening tool. Researchers have developed simplified echocardiographic criteria that could be implemented on cheaper hand-held machines and performed by less skilled or minimally trained health workers.
  8. Registries should be user-friendly for local health workers with minimum data requirements – only those needed to fulfill essential goals for clinical management, or research activities.
  9. Benzathine penicillin (BPG) injections remain the cornerstone of secondary prevention. Challenges with penicillin procurement and concern with adverse reactions in patients with advanced disease remain important issues.
  10. Access to surgery remains to be one of the most important problems in LMICs where RHD is endemic. Except for South Africa, cardiac surgery is performed independently without visiting mission teams in only a few countries. Continued efforts to reduce costs of care are needed.


Kumar RK, Antunes MJ, Beaton A, Mirabel M, Nkomo VT, Okello E, Regmi PR, Reményi B, Sliwa-Hähnle K, Zühlke LJ, Sable C; on behalf of the American Heart Association Council on Lifelong Congenital Heart Disease and Heart Health in the Young; Council on Cardiovascular and Stroke Nursing; and Council on Clinical Cardiology. Contemporary diagnosis and management of rheumatic heart disease: implications for closing the gap: a scientific statement from the American Heart Association [published online ahead of print October 19, 2020]. Circulation. doi: 10.1161/CIR.0000000000000921.