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.
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Epidemiology
An increased prevalence in females has been reported 4.
Risk factors
socioeconomic factors, e.g. overcrowding, poverty, poor access to healthcare 9
recurrent group A streptococcus infections
Pathology
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.
Myocardial involvement
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.
Pericardial involvement
Initially results in a pericardial inflammation and an effusion. Fibrinous pericarditis can occur. Pericardial calcification may occur as a later sequela.
Valvular involvement
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.
Radiographic features
Plain radiograph
pulmonary ossification: due to mitral valve disease 4
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global cardiomegaly from a dilated cardiomyopathy
left atrial enlargement (particularly appendage) from mitral valve disease
valvular calcification
diffuse alveolar hemorrhage can result from severe mitral stenosis
CT
valvular or pericardial calcification
dilated ventricles and atria
MRI
dilated cardiac chambers
pericardial inflammation
myocardial inflammation