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 login to view 2 more bullets disease is characterized by early stage login to view 1 more bullet late stage login to view 3 more bullets 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 login to view 2 more bullets mitral stenosis login to view 2 more bullets aortic regurgitation login to view 1 more bullet aortic stenosis login to view 1 more bullet 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 login to view 1 more bullet sulfadiazine indications login to view 2 more bullets Operative valve repair or replacement indication login to view 1 more bullet modalities login to view 2 more bullets Complications Aortic regurgitation Cardiac arrhythmias Heart failure