Rheumatic heart disease

Last revised by Henry Knipe on 17 Mar 2022

Rheumatic heart disease (not to be confused with rheumatoid heart disease) may refer to either the acute cardiac involvement or chronic cardiac sequelae following rheumatic fever. Carditis is a major Revised Jones criterion of rheumatic fever.

Risk factors include:

  • socioeconomic factors eg; overcrowding, poverty, poor access to healthcare 9
  • recurrent group A streptococcus infections
  • an increased prevalence in females has been reported 4

Initial inflammatory events are precipitated by a group A Streptococcus pyogenes infection that causes a type 2 hypersensitivity reaction where antibodies to the bacteria exhibit molecular mimicry to human tissues7. The presence of Aschoff cells is a histological diagnostic feature.

The initial acute phase may result in myocarditis which may progress to a dilated cardiomyopathy as later sequelae. The posterior wall of the left atrium endocardium may be irregular and thickened (known as MacCallum patch, triangle or plaque) 10.

Initially results in a pericardial inflammation and an effusion. Fibrinous pericarditis can occur. Pericardial calcification may occur as a later sequelae.

Valvular involvement is related to endocarditis and can result in either stenosis and/or insufficiency, which can manifest either acutely or several years to decades after the initial onset of rheumatic fever. Most commonly the mitral valve is affected, producing stenosis in later disease 2,6. Aortic regurgitation can also occur. Pathologically commissural fusion of valve leaflets is a characteristic feature.

Valvular disease can develop after either a single severe episode of acute rheumatic fever or after multiple episodes 8

  • valvular or pericardial calcification
  • dilated ventricles and atria

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Cases and figures

  • Case 1
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  • Case 2
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  • Case 3: with MacCallum's patch
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