• 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.
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • Aortic regurgitation
  • Cardiac arrhythmias
  • Heart failure

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