Rheumatic Heart Disease

Rheumatic Heart Disease

Rheumatic Heart Disease

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RHD Pathogenesis - Strep's Delayed Attack

  • Trigger: Group A β-hemolytic Streptococcus (GAS) pharyngitis (untreated/inadequately treated).
  • Mechanism: Molecular mimicry.
    • Bacterial M protein & carbohydrates (e.g., N-acetylglucosamine) share antigenic similarity with host tissues.
    • Host antibodies produced against GAS (e.g., anti-streptolysin O, anti-DNase B) cross-react with cardiac myosin, valvular endothelium, synovium, and brain antigens.
  • Timeline: Delayed, non-suppurative autoimmune sequela, typically 2-4 weeks after acute GAS infection.
  • Key Pathology:
    • Pancarditis: Inflammation affecting endocardium, myocardium, and pericardium.
    • Aschoff bodies: Pathognomonic granulomatous lesions in myocardium; collections of lymphocytes, plasma cells, and characteristic Anitschkow cells (macrophages with caterpillar-like nuclei). Aschoff body histopathology in rheumatic carditis

⭐ Aschoff bodies are pathognomonic for rheumatic carditis.

  • Genetic predisposition influences susceptibility (certain HLA types, e.g., HLA-DR4, DR2).

Jones Criteria - Diagnostic Checklist

Diagnosis (mod/high-risk populations): 2 Major OR 1 Major + 2 Minor criteria, PLUS evidence of recent Group A Strep (GAS) infection.

  • Major Criteria (📌 JONES):
    • Joints: Polyarthritis (migratory)
    • O (♥): Carditis (clinical/subclinical)
    • Nodules: Subcutaneous (painless)
    • Erythema Marginatum
    • Sydenham's Chorea
  • Minor Criteria (mod/high-risk):
    • Monoarthralgia
    • Fever (≥ 38°C)
    • ↑ESR (≥30 mm/hr) OR ↑CRP (≥3.0 mg/dL)
    • Prolonged PR interval (ECG)
  • Essential: Evidence of Preceding GAS Infection:
    • ↑ASO or Anti-DNase B titers
    • Positive GAS throat culture or Rapid Antigen Detection Test (RADT)
    • Recent scarlet fever (if documented)

⭐ Carditis is the most serious manifestation of acute rheumatic fever and the only one causing permanent damage.

Investigations & Valvular Lesions - Heart's Echoes

  • Key Investigations:
    • ↑ ASO titre, ↑ ESR/CRP (inflammation markers).
    • ECG: PR prolongation, chamber enlargement signs.
    • Chest X-ray: Cardiomegaly, pulmonary congestion.
    • Echocardiography (2D Echo & Doppler): Gold standard. Confirms diagnosis, assesses valve damage & function. Echocardiogram of mitral stenosis in RHD
  • Common Valvular Lesions (Echo findings):
    • Acute Phase:
      • Mitral Regurgitation (MR): Most common (📌 Carey Coombs murmur).
      • Aortic Regurgitation (AR).
      • Valvulitis: Annular dilatation, leaflet edema, prolapse, chordal elongation.
    • Chronic Phase:
      • Mitral Stenosis (MS): Most frequent. "Fish-mouth" valve.
      • MR, AR, Aortic Stenosis (AS).
      • Thickened, fibrosed, calcified leaflets; chordal fusion.

⭐ Mitral valve is the most commonly affected valve in chronic RHD, typically leading to mitral stenosis.

Management & Prophylaxis - Shielding the Heart

Acute Rheumatic Fever (ARF) Management:

  • Goals: Control inflammation, eradicate GAS, manage complications.
  • Bed rest; Aspirin (arthritis/fever); Prednisolone (carditis).
  • Eradicate GAS: Benzathine Penicillin G 1.2 MU IM (single dose, 0.6 MU if <27kg) or oral Penicillin V (10 days).

Secondary Prophylaxis (Prevent Recurrence & RHD Progression):

⭐ Secondary prophylaxis with Benzathine Penicillin G is the cornerstone of preventing RHD progression and recurrence.

  • Primary: Benzathine Penicillin G 1.2 MU IM every 3-4 weeks (📌 Benzathine Prevents Going back).
    • Every 3 weeks for high-risk/severe carditis.
  • Alternatives (penicillin allergy): Sulfadiazine, Erythromycin.
  • Duration guided by RHD severity (see flowchart).

High‑Yield Points - ⚡ Biggest Takeaways

  • Most common cause of acquired heart disease in children worldwide.
  • Follows Group A Streptococcal (GAS) pharyngitis; an autoimmune reaction.
  • Revised Jones Criteria (major/minor) for diagnosis, needs prior GAS infection evidence.
  • Pancarditis is common; mitral valve most affected (initially MR, later MS).
  • Aschoff bodies (myocardial granulomas) are pathognomonic.
  • Sydenham's chorea is a late, specific major neurological sign.
  • Long-term penicillin prophylaxis prevents recurrence and valvular damage.

Practice Questions: Rheumatic Heart Disease

Test your understanding with these related questions

A 12 year old girl was brought with fever, malaise, and migrating polyarthritis. She had a history of recurrent throat infections in the past. Elevated erythrocyte sedimentation rate is noted. Which among the following is NOT a major Jones criteria for diagnosis of acute rheumatic fever?

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Flashcards: Rheumatic Heart Disease

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_____ criteria is used to diagnose congenital heart disease in children.

TAP TO REVEAL ANSWER

_____ criteria is used to diagnose congenital heart disease in children.

NADA s

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