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 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.
Valvular involvement is related to an 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 a 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.
- pericardial calcification
- pulmonary oedema
- pulmonary ossification: due to mitral valve disease 4
- global cardiomegaly from a dilated cardiomyopathy
- left atrial enlargement (particularly appendage) from mitral valve disease
- valvular calcification
- diffuse alveolar haemorrhage can result from severe mitral stenosis
- valvular or pericardial calcification
- dilated ventricles and atria
- dilated cardiac chambers
- pericardial inflammation
- pericardial effusion
- myocardial inflammation
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- 8. Carapetis JA, Beaton A, Cunningham MW et al. Nature Reviews Disease Primers volume 2, Article number: 15084 (2016) https://www.nature.com/articles/nrdp201584
- 9. Katzenellenbogen JM, Ralph AP, Wyber R, Carapetis JR. Rheumatic heart disease: infectious disease origin, chronic care approach. BMC Health Serv Res. 2017;17(1):793. Published 2017 Nov 29. doi:10.1186/s12913-017-2747-5