Updated: 2/1/2018

Rheumatic Heart Disease

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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
  • 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
  • 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 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
  • 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 nail bed
      • vegetations seen on valves on imaging
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

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Questions (3)
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(M2.CV.15.36) A 15-year-old girl presents with four days of malaise, painful joints, nodular swelling over her elbows, low-grade fever, and a rash on her chest and left shoulder. Two weeks ago, she complained of a sore throat that gradually improved but was not worked up. She was seen for a follow-up approximately one week later. At this visit her cardiac exam was notable for a late diastolic murmur heard best at the apex in the left lateral decubitus position with no radiation. Which of the following is the best step in the management of this patient?

QID: 104042
1

Penicillin therapy

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NSAIDS for symptomatic relief

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Aortic valve replacement

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Mitral valve repair

8%

(7/86)

5

Reassurance that this is a benign murmur and send home

3%

(3/86)

M 7 B

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(M2.CV.15.4662) A 21-year-old Cambodian patient with a history of rheumatic heart disease presents to his primary care physician for a routine check-up. He reports being compliant with monthly penicillin G injections since being diagnosed with rheumatic fever at age 15. He denies any major side effects from the treatment, except for the inconvenience of organizing transportation to a physician's office every month. On exam, the patient is found to have a loud first heart sound and a mid-diastolic rumble that is best heard at the apex. Which of the following is the next best step?

QID: 107050
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Stop penicillin therapy

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(3/26)

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Stop penicillin therapy in 4 years

15%

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Decrease frequency of injections to bimonthly

0%

(0/26)

4

Switch to intramuscular cefotaxime, which has fewer side effects

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(0/26)

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Continue intramuscular penicillin therapy

69%

(18/26)

M 7 B

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