Rheumatic Heart Disease Overview

Rheumatic Heart Disease

• Diffuse inflammatory disease caused by an abnormal immune response to group A beta-hemolytic streptococci.
• Occurs post-streptococcal group A infection.
• Predisposing factors include recurrent streptococcal infections of the pharynx and tonsils, age between 5-15 years (rare under 5 and over 25), and overcrowding.
• Latent period is 1-3 weeks.

Major and Minor Criteria

• Major criteria:
  • Carditis
  • Arthritis
  • Chorea (Sydenham's chorea)
  • Subcutaneous Nodule
  • Erythema marginatum
• Minor criteria:
  • Prolonged PR interval in the ECG
  • Elevated ESR
  • Arthralgia (painful joints without swelling)
  • Elevated CRP (C-reactive protein)
  • Elevated temperature (fever)

Clinical Manifestations

• Carditis: found in 50% of patients
  • Pericarditis:
    • Dry pericarditis: causes stitching pain and pericardial rub.
  • Myocarditis:
    • Tachycardia/palpitation
    • Heart failure
    • Arrhythmia
  • Endocarditis (valvulitis): Mitral > Aorta > Tricuspid > Pulmonary
    • Acute stage: stenosis due to swelling of the cusps
    • Later (over years): fibrosis may lead to stenosis, regurgitation or both.
• Arthritis: found in 75% of patients
  • Polyarthritis
  • Affects large joints: knees, ankles, elbows
  • Asymmetrical
  • Migratory
  • Hotness, redness, tenderness, swelling, and limitation of movement
  • Dramatic response to aspirin
  • Duration of arthritis is about 2-6 weeks
  • No deformity
• Chorea: 10% of cases
  • Involuntary, sudden movements with emotional instability
  • Female > Male
  • Appears very late
  • Self-limiting
• Subcutaneous Nodule: rare
  • Small, painless, tender, firm nodule, not adherent to the skin
  • Site: over bony prominences and tendons, e.g., dorsum of hands and feet
• Erythema marginatum:
  • Non-pruritic, non-painful erythema with central pallor
  • Site: trunk and proximal part of limbs
• Other manifestations include pallor, pleurisy, pneumonia, and peritonitis.

Diagnosis

• 2 major criteria OR 1 major and 2 minor criteria PLUS evidence of recent strep infection (+ve throat culture, raised ASO)

Complications

• Acute:
  • Heart failure
  • Arrhythmia and heart block
• Chronic: Complications of valvular heart diseases:
  • Calcification
  • Rheumatic activity
  • Infective endocarditis
  • Arrhythmia
  • Thromboembolism: stroke
  • Arterial enlargement
  • Pulmonary congestion
  • Pulmonary infection
  • Pulmonary embolism (secondary to DVT)
  • LSHF/RSHF
  • Complications of surgery (artificial valves)

Investigations

• No specific confirmatory test.
• Laboratory: (Evidence of recent streptococcal infection)
  • CBC: leukocytosis
  • ESR: raised
  • CRP: raised
  • ASO titre (Anti Streptolysin O titre): raised
  • Throat culture: may reveal group A streptococci
• Cardiac: (Evidence of carditis)
  • X-ray
  • ECG
  • Echo

Treatment

• Prophylactic:
  • Primary prevention: (To prevent rheumatic fever)
    • Proper management of URT infection
    • Tonsillectomy for chronically infected tonsils
  • Secondary prevention: (To prevent recurrence of rheumatic activity)
    • Long-acting penicillin: Benzathine penicillin (Retarpen) OR penicillin V OR erythromycin (in case of allergy to penicillin)
    • For 5 years after the last attack or until the age of 25 (whichever is longer) and may be lifelong in some cases.
  • Tertiary prevention: (Prevention of infective endocarditis)
• Curative:
  • Complete bed rest until improvement of all symptoms and signs
  • Diet: Light meals and salt restriction
  • Antibiotics: Benzyl penicillin G, penicillin V, erythromycin
  • Anti-inflammatory drugs: aspirin (for arthritis) and corticosteroids (for carditis)
  • Treatment of complications

Infective Endocarditis

• Infection of the endocardium
• Etiology: combination of infection and underlying cardiac disease.
• Infection:
  • Gram-positive cocci:
    • Streptococcus viridans (most common): e.g., tooth extraction
    • Streptococcus foecalis: e.g., GIT procedures
    • Staphylococcus aureus: e.g., cardiac catheterization
  • Gram-negative bacilli: Haemophilus influenza, Klebsiella, Pseudomonas
  • Others: Fungi, Rickettsia, Chlamydia
• Underlying cardiac disease:
  • Valvular disease and prosthetic (artificial) valve
  • Congenital heart disease
  • Myocardial infarction

Clinical Picture

• Cardiac manifestations:
  • Features of the underlying cardiac disease
  • Precipitation of heart failure
  • New murmurs due to perforated cusps or rupture of chordae tendineae.
• Extracardiac Manifestations:
  • Face:
    • Fever (usually low grade and prolonged)
    • Pallor and toxic facies
  • Eye:
    • Subconjunctival hemorrhage
    • Roth spots (small, white spots surrounded by hemorrhage in the retina)

Rheumatic Heart Disease

• A diffuse inflammatory disease triggered by an abnormal immune response to Group A Beta Hemolytic Streptococcus.
• Occurs after a Group A streptococcal infection.
• Factors contributing to the disease:
  • Antigenic similarity between the bacteria and human tissues
  • Altered antigenicity of the bacterial proteins.
• Predisposing factors:
  • Recurrent streptococcal infections of the pharynx and tonsils.
  • Age: common between 5-15 years (rare < 5 &> 25)
  • Overcrowding.
• Latent period: 1-3 weeks.
• Major criteria:
  • Carditis
  • Arthritis
  • Chorea
  • Subcutaneous Nodule
  • Erythema Marginatum
• Minor criteria:
  • Prolonged PR interval in the ECG
  • Elevated ESR
  • Arthralgia (painful joints without swelling)
  • Elevated CRP
  • Elevated temperature (fever)

Carditis

• Present in 50% of patients.
• Types of Carditis:
  • Pericarditis:
    • Dry pericarditis: causing stitching pain and pericardial rub
  • Myocarditis:
    • Tachycardia/palpitation
    • Heart failure
    • Arrhythmia.
  • Endocarditis (valvulitis):
    • Affects Mitral > Aorta > Tricuspid > Pulmonary valve
    • Acute stage: Stenosis due to swelling of the cusps
    • Later stage (over years): Fibrosis may lead to stenosis, regurgitation, or both.

Arthritis

• Present in 75% of patients.
• Polyarthritis affecting large joints: knees, ankles, elbows.
• Asymmetrical and Migratory.
• Characterized by redness, tenderness, swelling, and limited movement.
• Dramatic response to aspirin.
• Duration: 2-6 weeks.
• No deformity

Chorea

• Sydenham’s Chorea: 10% of cases.
• Involuntary, sudden movements with emotional instability.
• More common in females.
• Appears late, is self-limiting.

Subcutaneous Nodule

• Rare, small, painless, tender, firm nodule, not adherent to the skin.
• Location: over bony prominences and tendons, e.g., dorsum of hands and feet.

Erythema marginatum

• Non-pruritic and non-painful erythema with central pallor.
• Location: Trunk and proximal part of limbs.

Other Manifestations of Rheumatic Fever

• Pallor
• Pleurisy
• Pneumonia
• Peritonitis

Diagnosis of Rheumatic Fever

• 2 major criteria, OR 1 major and 2 minor criteria.
• Evidence of recent streptococcal infection (positive throat culture, elevated ASO).

Complications of Rheumatic Fever

• Acute complications:
  • Heart failure
  • Arrhythmia and heart block
• Chronic Complications: Due to valvular heart disease:
  • Calcification
  • Rheumatic activity
  • Infective Endocarditis
  • Arrhythmia
  • Thromboembolism (stroke)
  • Arterial enlargement
  • Pulmonary congestion
  • Pulmonary infection
  • Pulmonary embolism (secondary to DVT)
  • Left-Sided/Right-sided Heart Failure
  • Complications of surgery (artificial valves).

Investigations

• No specific confirmatory test.
• Laboratory tests (evidence of recent streptococcal infection):
  • CBC: Leukocytosis
  • ESR: Elevated
  • CRP: Elevated
  • ASO titre: Elevated
  • Throat culture: may reveal Group A streptococci
• Cardiac tests (evidence of carditis):
  • X-ray
  • ECG
  • Echocardiogram

Treatment of Rheumatic Fever

• Prophylactic:
  • Primary prevention:
    • Proper management of upper respiratory tract infections
    • Tonsillectomy for chronically infected tonsils
  • Secondary prevention:
    • Long-acting penicillin: Benzathine penicillin, Penicillin V, or Erythromycin (in case of a penicillin allergy)
    • Duration: 5 years after the last attack or until age 25 (whichever is longer), and may be lifelong
  • Tertiary prevention:
    • Prevention of Infective Endocarditis
• Curative:
  • Complete bed rest until improvement of all symptoms and signs.
  • Diet: Light meals and salt restriction.
  • Antibiotics:
    • Benzyl penicillin G
    • Penicillin V
    • Erythromycin
  • Anti-inflammatory drugs:
    • Aspirin: for Arthritis
    • Corticosteroids: for Carditis
  • Treatment of complications.

Infective Endocarditis

• Infection of the endocardium.
• Etiology: Combination of infection and underlying cardiac disease.
• Infection:
  • Gram-positive cocci:
    • Streptococcus viridans (most common): e.g. tooth extraction
    • Streptococcus faecalis: e.g. gastrointestinal procedures
    • Staphylococcus aureus: e.g. cardiac catheterization
  • Gram-negative bacilli: Haemophilus influenza, Klebsiella, Pseudomonas
  • Others: Fungi, Rickettsia, Chlamydia
• Underlying cardiac disease:
  • Valvular disease and Prosthetic (artificial) valve
  • Congenital heart disease.
  • Myocardial infarction

Clinical Picture of Infective Endocarditis

• Cardiac manifestations:
  • Features of the underlying cardiac disease.
  • Precipitation of heart failure.
  • New murmurs due to perforated cusps or rupture of chordae tendineae.
• Extra-cardiac manifestations:
  • Face:
    • Fever (usually low-grade and prolonged)
    • Pallor and toxic facies
    • Eyes:
      • Subconjunctival hemorrhage
      • Roth spots
      • Osler's nodes
  • Splenomegaly
  • Petechiae
  • Janeway lesions
  • Clubbing of fingers.
  • Neurological:
    • Embolic stroke
    • Meningitis
    • Encephalitis.

This quiz covers the key aspects of rheumatic heart disease, including its causes, clinical manifestations, and the major and minor criteria for diagnosis. It is essential for understanding the implications of streptococcal infections and the importance of early detection in young patients.