Updated: 10/29/2020

Rheumatic Heart Disease

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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

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Questions (4)
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(M1.CV.15.75) A 38-year-old man presents to his primary care physician complaining of increasing shortness of breath over the past 2 months. He reports experiencing an extended illness of several weeks as a child that required him to miss school. He is unsure but believes it involved a sore throat followed by a fever and joint pains. He does not recall seeing a physician or receiving treatment for this. Today, on physical examination, cardiac auscultation reveals an opening snap after the second heart sound followed by a diastolic murmur. A follow-up echocardiogram is conducted. Which of the following best explains the pathophysiology of this patient's condition?

QID: 106727
1

Amyloid deposition

27%

(9/33)

2

Epitope homology

64%

(21/33)

3

Myocardial ischemia

0%

(0/33)

4

Atherosclerosis

3%

(1/33)

5

Congenital heart defect

6%

(2/33)

M 1 D

Select Answer to see Preferred Response

(M1.CV.13.112) A 10-year-old male presents with his mother with multiple complaints. A few weeks ago, he had a sore throat for several days that improved without specific therapy. Additionally, over the past several days he has experienced pain in his ankles and wrists and, more recently, his left knee. His mother also noted several bumps on both of his elbows, and he has also had some pain in his center of his chest. He thinks the pain is better when he leans forward. On physical examination, he is noted to be mildly febrile, and a pericardial friction rub is auscultated. Which of the following histopathologic findings is most likely associated with this patient's condition?

QID: 100628
1

Atypical lymphocytes noted on peripheral blood smear with an initial positive heterophil antibody test

15%

(5/34)

2

Plasmodium falciparum ring forms in red blood cells on peripheral blood smear

3%

(1/34)

3

Needle-shaped, negatively birefringent crystal deposits surrounded by palisading histiocytes in the synovial fluid of an affected joint

6%

(2/34)

4

Interstitial myocardial granulomas containing plump macrophages with nuclei incorporating central wavy ribbons of chromatin

44%

(15/34)

5

Sterile vegetations on both the ventricular and atrial aspects of the mitral valve, a proliferative glomerulonephritis, and serum anti-dsDNA and anti-Sm positivity

29%

(10/34)

M 2 D

Select Answer to see Preferred Response

(M1.CV.12.223) A 9-year-old male visited his primary care physician for a check-up three months after a throat infection. Upon examination, the patient exhibits painless subcutaneous nodules on the back of the wrist, the outside elbow, and the front of the knees, as well as inflammation in the joints of the lower extremities. Which of the following symptoms is most likely to also be present in this patient?

QID: 100739
1

Renal failure

18%

(6/33)

2

Hepatomegaly

6%

(2/33)

3

Chorea

76%

(25/33)

4

Pleural effusion

0%

(0/33)

5

Butterfly rash

0%

(0/33)

M 2 E

Select Answer to see Preferred Response

Evidence (4)
EXPERT COMMENTS (10)
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