Rheumatic heart disease
Rheumatic heart disease is chronic valvular damage following acute rheumatic fever, an autoimmune sequela of group A streptococcal pharyngitis. It predominantly scars the mitral and aortic valves, causing stenosis and regurgitation years later; management is secondary antibiotic prophylaxis, treatment of valve lesions, and prevention of recurrent rheumatic fever.
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What this article covers
- Overview
Definition: rheumatic heart disease is chronic valvular damage resulting from acute rheumatic fever, an immune-mediated sequela of group A streptococcal pharyngitis.
- Epidemiology
A major cause of acquired valve disease worldwide, concentrated in low- and middle-income regions.
- Risk factors
Recurrent or untreated group A streptococcal pharyngitis.
- Pathophysiology
Molecular mimicry: antibodies and T cells against streptococcal antigens cross-react with cardiac tissue.
- Clinical features
Acute rheumatic fever: migratory polyarthritis, carditis, chorea, subcutaneous nodules, and erythema marginatum after streptococcal pharyngitis.
- Differential diagnosis
Degenerative (calcific) valve disease: age-related, without commissural fusion (Aortic stenosis).
- Diagnosis
Establish acute rheumatic fever by the Jones criteria and chronic disease by echocardiographic valve findings: the diagnostic basis for acute rheumatic fever is the revised Jones criteria (major and…
- Management
First-line, treat acute rheumatic fever and eradicate streptococcus: give anti-inflammatory therapy (aspirin or an NSAID such as naproxen 500 mg PO twice daily) for arthritis and carditis, and…
- Complications
Progressive valve stenosis and regurgitation.
- Prognosis
Depends on the severity of valve damage and recurrence prevention.
- Prevention
Primary prevention: prompt antibiotic treatment of streptococcal pharyngitis.
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- Mitral stenosis
Mitral stenosis is obstruction of left atrial emptying from a narrowed mitral valve, overwhelmingly rheumatic in origin. It causes left atrial hypertension, atrial fibrillation, and pulmonary congestion, with a diastolic rumble and opening snap; severe symptomatic disease is treated with percutaneous balloon valvuloplasty or valve surgery.
- Mitral regurgitation
Mitral regurgitation is systolic backflow from the left ventricle into the left atrium, classified as primary (valve disease) or secondary (ventricular or annular disease). Chronic disease causes volume overload with an apical holosystolic murmur, while acute severe regurgitation causes pulmonary edema; severe primary disease is treated with valve repair or replacement.
- Aortic regurgitation
Aortic regurgitation is diastolic backflow of blood from the aorta into the left ventricle from valve or aortic root disease. Chronic disease causes volume overload with a wide pulse pressure and a decrescendo diastolic murmur, while acute severe regurgitation is a surgical emergency; severe symptomatic or decompensating disease is treated with valve replacement.
- Aortic stenosis
Aortic stenosis is obstruction to left ventricular outflow from a narrowed aortic valve, most often calcific degeneration in older adults or a bicentric bicuspid valve earlier in life. Symptoms of angina, syncope, or heart failure mark a poor prognosis, and severe symptomatic disease is treated with valve replacement (surgical or transcatheter).
- Infective endocarditis
Infective endocarditis is microbial infection of the endocardium, usually a heart valve, producing vegetations that cause valve destruction, embolism, and systemic infection. Diagnosis uses the modified Duke criteria combining blood cultures and echocardiography, and treatment is prolonged targeted intravenous antibiotics with surgery for complications.
- Mitral valve prolapse
Mitral valve prolapse is systolic displacement of one or both mitral leaflets into the left atrium, usually from myxomatous degeneration, marked by a mid-systolic click and a late systolic murmur. Most cases are benign, but it is the leading cause of primary mitral regurgitation and a small subset carry arrhythmic risk. Management is reassurance for benign disease, beta-blockers for symptoms, and mitral valve repair for severe regurgitation.
- Myocarditis
Myocarditis is inflammation of the myocardium, most often postviral or immune-mediated, presenting across a spectrum from a mild chest-pain syndrome mimicking myocardial infarction to fulminant heart failure and arrhythmia. Diagnosis relies on cardiac MRI (or biopsy for severe or refractory cases), and treatment is supportive heart-failure care, with cause-specific immunosuppression for giant cell, checkpoint-inhibitor, and autoimmune myocarditis.