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Rheumatic heart disease

Rheumatic heart disease

Rheumatic heart disease

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Etiopathogenesis - Strep's Sneaky Attack

  • Trigger: Untreated Group A β-hemolytic Streptococcus pyogenes pharyngitis.
  • Mechanism: Type II Hypersensitivity via molecular mimicry.
    • Strep M-protein & N-acetyl-beta-D-glucosamine resemble human cardiac antigens (myosin, laminin).
    • Antibodies produced against Strep cross-react with host cardiac tissues.
  • Timeline: Acute rheumatic fever develops 2-4 weeks after the initial strep throat infection.

Histopathologic Hallmark: Aschoff bodies (granulomas with fibrinoid necrosis) containing Anitschkow cells ("caterpillar cells" with wavy nuclei) are pathognomonic for acute rheumatic carditis.

Acute Rheumatic Fever - Jones's Rules of the Game

Diagnosis requires evidence of a preceding Group A Strep infection + 2 major criteria OR 1 major & 2 minor criteria.

📌 Mnemonic: JONES

Major CriteriaMinor Criteria
Joints (migratory polyarthritis)Arthralgia
O (Carditis)Fever (≥38.5°C / 101.3°F)
Nodules (subcutaneous)Elevated ESR or CRP
Erythema marginatumProlonged PR interval
Sydenham chorea

⭐ Carditis is the most severe sign, potentially affecting all heart layers (pancarditis). It is the only major criterion that can lead to permanent damage, most commonly causing mitral regurgitation in the acute phase.

Cardiac Pathology - The Heart's Scars

  • Gross Findings:

    • Pericardium: Fibrinous pericarditis ("bread and butter" appearance).
    • Valves (Chronic): Leaflet thickening, commissural fusion, and chordae tendineae shortening leading to "fish-mouth" or "buttonhole" stenosis. Most commonly affects mitral valve.
    • Endocardium: MacCallum patches (irregular thickening) in the left atrium.
  • Microscopic Hallmark:

    • Aschoff Body: The pathognomonic finding. A focus of fibrinoid necrosis surrounded by inflammatory cells.
    • Anitschkow Cells: Plump macrophages with caterpillar-like chromatin found within Aschoff bodies.

Aschoff body and Anitschkow cells in rheumatic heart disease

⭐ Chronic rheumatic heart disease is overwhelmingly the most frequent cause of mitral stenosis.

Chronic RHD & Management - The Long Game

  • Pathology: Progressive, irreversible valve fibrosis developing years after ARF. Mitral valve is most affected (>85%), leading to classic "fish-mouth" or "buttonhole" stenosis. Aschoff bodies are replaced by fibrous scar tissue.
  • Clinical Course: A long latent period, often 10-20 years, between the initial ARF and the onset of clinical symptoms.
  • Common Sequelae:
    • Mitral stenosis (most common) → LA enlargement → Atrial fibrillation → Thromboembolism risk.
    • Mitral regurgitation.
    • Aortic valve disease (stenosis/regurgitation) is less frequent.
  • Management:
    • Focuses on complications: Diuretics, rate control (β-blockers), and anticoagulation for AFib.
    • Surgical: Valve repair or replacement for severe, symptomatic disease.

Rheumatic Heart Disease: "Fish-Mouth" Mitral Valve

⭐ The clinical features of chronic RHD, particularly mitral stenosis, typically manifest 10-20 years after the initial episode of acute rheumatic fever, highlighting a significant asymptomatic latent period.

  • Results from molecular mimicry following Group A Strep pharyngitis (Type II hypersensitivity).
  • Aschoff bodies (pathognomonic granulomas) and Anitschkow cells are key histologic findings.
  • Acute phase presents as a pancarditis affecting all heart layers.
  • Mitral valve is most commonly affected, leading to chronic stenosis; aortic valve is second.
  • Diagnosis relies on Jones criteria and evidence of recent strep infection.
  • Chronic RHD causes "fish-mouth" deformity and ↑ risk of atrial fibrillation.

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