Rheumatic Heart Disease

Overview – Rheumatic Heart Disease

Rheumatic heart disease is a chronic valvular condition caused by an autoimmune response to Group A β-haemolytic Streptococcus (GAS) pharyngitis. It develops after repeated or severe episodes of acute rheumatic fever, particularly in genetically susceptible individuals. Understanding this disease is essential for early diagnosis, effective treatment, and the prevention of irreversible cardiac damage in young populations worldwide.

Definition

  • Rheumatic Fever (RF): A delayed autoimmune complication of GAS pharyngitis.
  • Rheumatic Heart Disease (RHD): The chronic sequela of RF, typically manifesting as mitral stenosis due to cumulative valve damage.
  • Distinction:
    • RF = Acute, inflammatory, and potentially reversible.
    • RHD = Chronic, fibrotic, and irreversible.

Aetiology

  • Environmental Trigger: GAS (Streptococcus pyogenes) pharyngitis.
  • Genetic Susceptibility: HLA-DR2 and HLA-DR3 positive individuals (≈3% of population).
  • Autoimmune Reaction:
    • Anti-M protein antibodies cross-react with cardiac connective tissue (molecular mimicry).

Pathophysiology

  • GAS pharyngitis in a genetically susceptible host triggers an immune response.
  • After ~2 weeks, immune cross-reactivity causes systemic inflammation → acute rheumatic fever.
  • Repeated infections lead to stronger immune responses, resulting in progressive fibrosis of heart valves.
  • Most commonly affects the mitral valve (→ mitral stenosis).
Strep Throat - leading cause of rheumatic heart disease
Mp3car, CC BY-SA 3.0 <https://creativecommons.org/licenses/by-sa/3.0>, via Wikimedia Commons
Mitral stenosis from rheumatic heart disease
Source: Unattributable

Clinical Features

Acute Rheumatic Fever

Diagnosed using the Modified Jones Criteria:

  • Requires:
    1. Evidence of recent GAS infection, and
    2. Either 2 major OR 1 major + 2 minor criteria.

Evidence of Recent GAS Infection:

  • ↑Anti-streptolysin-O (ASO) titre
  • ↑Anti-DNase B antibodies
  • Positive throat swab culture

Major Criteria:

  • Joints – Migratory polyarthritis
  • Carditis – Pericarditis, quiet heart sounds, tachycardia
  • Nodules – Subcutaneous, painless
  • Erythema marginatum – Non-pruritic, tinea-like rings
  • Sydenham chorea – Involuntary movements

Minor Criteria:

  • Fever
  • Arthralgia
  • Elevated ESR
  • Prolonged PR interval
https://www.myupchar.com/en, CC BY-SA 4.0 <https://creativecommons.org/licenses/by-sa/4.0>, via Wikimedia Commons

Chronic Rheumatic Heart Disease

  • Valvular pathology (Left-sided most common):
  • Mitral Stenosis Signs:
    • “Mitral facies” – Malar rash over cheeks and nose
    • Mid-diastolic murmur – Loudest at apex on expiration, radiating to axilla
    • Pulmonary congestion, right heart failure
  • Atrial fibrillation (due to left atrial stretch)
  • ↑ Risk of infective endocarditis

Investigations

  • ASO titres / Anti-DNase B
  • ECG – PR prolongation, AF
  • Echocardiography – Valve function, chamber sizes
  • Throat culture (in acute phase)

Management

Primary Prevention

  • Treat initial GAS pharyngitis:
    • 10 days oral Penicillin-V (or Amoxicillin/Cephalexin)

Secondary Prevention

  • Acute RF:
    • Benzathine Penicillin G (IM, single dose)
    • NSAIDs or Codeine for arthritis
    • Carbamazepine or Valproate for chorea
    • ACE inhibitors, β-blockers, diuretics for carditis or CCF
  • Prevent Recurrence:
    • Long-term antibiotic prophylaxis:
      • 1st line: Monthly IM Benzathine Penicillin G
      • 2nd line: BD oral Penicillin-V
      • Penicillin allergy: BD oral Erythromycin

Tertiary Prevention

  • Surgical valve replacement (typically mitral valve)

Complications

  • Irreversible valvular heart disease
  • Heart failure
  • Atrial fibrillation
  • Infective endocarditis
  • Stroke (from embolic events in AF)

Differential Diagnosis

Summary – Rheumatic Heart Disease

Rheumatic heart disease is a preventable, chronic cardiac complication resulting from recurrent or severe acute rheumatic fever, itself a delayed autoimmune response to Group A streptococcal pharyngitis. Early recognition and management—including antibiotic prophylaxis and valve monitoring—are crucial in preventing long-term sequelae such as mitral stenosis, atrial fibrillation, and heart failure. For broader context, see our Cardiovascular Overview page.

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