Snapshot A 25-year-old woman presents to her primary care physician’s office for her annual physical. She recently immigrated from a developing country and reports having multiple episodes of pharyngitis in the past. On physical exam, there is a holosystolic murmur at the apex. A follow-up echocardiogram shows mitral regurgitation, concerning for rheumatic heart disease. Introduction Clinical definition a consequence of rheumatic fever characterized by inflammation and scarring of the heart valves Epidemiology demographics female > male most common in developing nations leading cause of pediatric heart disease location mitral valve > aortic valve > tricuspid valve most commonly affects the high-pressure valves risk factors poverty and overcrowding recurrent acute rheumatic fever group A streptococcal pharyngitis Etiology at least 1 episode of acute rheumatic fever from group A streptococci Pathogenesis cumulative inflammation and scarring of the heart valves resulting from an abnormal immune response to group A streptococci molecular mimicry between streptococcal M protein and cardiac proteins cross-reaction of antibodies to streptococcal M protein with self-antigens immune-mediated (type II) hypersensitivity disease is characterized by early stage valve regurgitation, most commonly of the mitral valve late stage valve stenosis, most commonly of the mitral valve mitral valve stenosis leads to increased end-diastolic left atrial pressure, which causes left atrial enlargement left atrial enlargement can manifest as atrial fibrillation or dysphagia Associated conditions rheumatic fever Sydenham chorea carditis arthritis erythema marginatum subcutaneous nodules Prognosis the early stage may last for years and may be asymptomatic onset of symptoms usually occurs 10-20 years after acute rheumatic fever Presentation Symptoms palpitations (most common) fatigue chest pain Physical exam may have dyspnea cardiac exam mitral regurgitation holosystolic murmur may have a systolic thrill mitral stenosis diastolic murmur following opening snap specific to rheumatic heart disease aortic regurgitation early diastolic decrescendo murmur aortic stenosis crescendo-decrescendo systolic ejection murmur Imaging Echocardiography indications when the murmur auscultated on examination is suspicious for rheumatic heart disease to confirm the diagnosis findings valvular abnormalities, including regurgitation or stenosis Studies Labs ↑ anti-streptolysin O (ASO) titers Histology Aschoff bodies (granulomas with giant cells) on heart valves Making the diagnosis based on clinical presentation and confirmed with echocardiography Differential Infective endocarditis distinguishing factors no association with group A streptococcal infection other findings including Roth spots, Osler nodes, Janway lesions, and splinter hemorrhages on the nail bed vegetations seen on valves on imaging Treatment Management approach prophylaxis all patients with rheumatic heart disease should undergo prophylaxis with penicillin for years to prevent recurrence or worsening of rheumatic heart disease treatment depends on type and severity of valve involvement Medical penicillins indication for all patients in need of prophylaxis sulfadiazine indications for all patients in need of prophylaxis if patients are allergic to penicillin Operative valve repair or replacement indication depending on type and severity of valve pathology modalities surgical repair percutaneous intervention Complications Aortic regurgitation Cardiac arrhythmias Heart failure