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Updated: Aug 12 2021

Rheumatic Heart Disease

Images valve.jpg
  • Snapshot
    • A 30-year-old woman presents to her physician’s office for palpitations. She reports that she previously was diagnosed with group A streptococcal pharyngitis as a child and was suspected to have rheumatic fever. She took antibiotics for it, but she was subsequently lost to follow-up. On physical exam, there is a holosystolic murmur at the apex, suspicious for mitral regurgitation. She is sent for further imaging to confirm the diagnosis.
  • Introduction
    • 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
    • Microbiology
      • 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
  • Presentation
    • Symptoms
      • palpitations (most common)
      • fatigue
      • chest pain
    • Physical exam
      • may have dyspnea
      • cardiac exam
        • mitral regurgitation
          • holosystolic murmur
          • may have 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 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
  • 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 nail bed
        • vegetations seen on valves on imaging
  • Diagnosis
    • Making the diagnosis
      • based on clinical presentation and confirmed with echocardiography
  • Treatment
    • Management approach
      • prophylaxis
        • all patients with rheumatic heart disease should undergo prophylaxis with penicillin for the specified time period below
          • no evidence of carditis for 5 years or until age 21 (whichever is longer)
          • evidence of carditis without valvular abnormalities for 10 years or until age 21 (whichever is longer)
          • evidence of carditis and valvular abnormalitis for 10 years or until age 40 (whichever is longer)
        • 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
      • left atrial dilation and atrial fibrillation
    • Heart failure
  • Prognosis
    • The early-stage may last for years and maybe asymptomatic
    • Onset of symptoms usually occurs 10-20 years after acute rheumatic fever
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