Rheumatic heart disease is a chronic condition that results years after acute rheumatic fever, which is caused by group A beta-hemolytic Streptococcus pyogenes. This organism produces a pharyngitis that is followed by rheumatic fever in about 0.3% to 3% of cases and occurs several weeks after the pharyngitis resolves.
Rheumatic heart disease chronically manifests as congestive heart failure from valvular involvement. Most commonly the mitral valve is affected, resulting in mitral stenosis or mitral regurgitation. Less commonly, the aortic valve can be involved; tricuspid valve involvement is rare, but reported. In approximately 50% of cases of rheumatic heart disease, the patient does not give a history of having rheumatic fever as a child.
Rheumatic fever acutely causes symptoms of pericarditis and congestive heart failure, depending on the degree of valvulitis and myocarditis present. Migratory polyarthritis is the most common symptom in acute rheumatic fever. Subcutaneous nodules arise over the bones and tendons, as well as a rash that starts on the trunk and extends to the limbs. The rash has a characteristic erythematous ring with a pale center and is referred to as erythema marginatum. Sydenham's chorea (St. Vitus’ dance) occurs and is described as uncontrollable rapid movements of the arms and facial muscles.
Rheumatic fever is diagnosed with the Jones criteria:
Rheumatic valvular disease is diagnosed predominantly via echocardiography. The mitral valve will give a classic “hockey stick” appearance.
Physical examination findings include inflamed joints, subcutaneous nodules, a pericardial friction rub, findings of congestive heart failure (edema, pulmonary rales, elevated jugular venous pressure), and the rash of erythema marginatum
The Carey Coombs Murmur occurs during acute rheumatic fever. Mitral valvulitis can occur causing thickening of the leaflets. A murmur is created by increased blood flow across the thickened mitral valve. This can be distinguished from rheumatic mitral valve stenosis by the absence of an opening snap. The murmur is described as a mid-diastolic murmur heard at the mitral listening post with the bell of the stethoscope with the patient in the left lateral decubitus position at end expiration. As the rheumatic valvulitis resolves, the murmur disappears.
Rheumatic heart disease chronically will most commonly reveal the murmur of mitral valve stenosis:
Reducing the inflammatory response with corticosteroids is the mainstay of therapy during acute rheumatic fever with significant cardiac involvement. Aspirin historically had been used; however, the risk of Reye’s syndrome causing fulminant hepatic failure and death in children has decreased its use.
Long-term therapy with penicillin is recommended, as re-infection with group A beta-hemolytic Streptococcus can cause a serious recurrence.
See the review on mitral valve stenosis for treatment strategies for rheumatic mitral stenosis, the most common manifestation of rheumatic valvular disease.