RHEUMATIC HEART DISEASE

ELIZABETH TABITHA BOTCHWAY

 OUTLINE

• Introduction
• Acute Rheumatic Fever
• Pathogenesis
• Clinical features
• Investigations
• Management
• Chronic Rheumatic Heart Disease
 INTRODUCTION

• A major cause of cardiovascular disease in developing nations

• A cardiac inflammation and scarring triggered by autoimmune
reaction to infection with Group A Streptococci
 ACUTE RHEUMATIC FEVER

• Usually affects children – 5-15years

• Now rare in the West
• More endemic in Africa, South America and Asia
• Most common acquired heart disease in childhood and adolescence
 PATHOGENESIS

• Triggered by immune-mediated delayed response to infection with

specific strains of Group A Streptococci

• They have antigen that may cross-react with cardiac myosin and
sacrolemmal membrane

• These Antibodies cause inflammation in the endocardium,

myocardium and pericardium (show picture of the heart), as well as
the joints and the skin
 PATHOGENESIS

• Histology
• Fibrinoid degeneration
• Aschoff bodies (pathognomonic)
• Subacute and Chronic
• Multinucleated giant cells
• Surrounded by macrophages and T lymphocytes
 CLINICAL FEATURES

• Symptoms occur 2-3 weeks after Streptococcal pharyngitis

• There may be no history of sore throat
• Multisystem disorder
• Fever, anorexia, lethargy, joint pain
• Arthritis – 75%
• Revised Jones Criteria is used
C L I N I C A L F E AT U R E S
CLINICAL FEATURES
 CLINICAL FEATURES

• Diagnosis
• 2 OR MORE MAJOR MANIFESTATION
• 1 MAJOR AND TWO OR MORE MINOR MANIFESTATION

• PLUS
• EVIDENCE OF STREPTOCOCCAL INFECTIONS
 CLINICAL FEATURES

1. CARDITIS
• Pancarditis of varying degrees
• Incidence declines with age: 90% at 3 years, 30% in adolescence
• Features: breathlessness, tachycardia, cardiac enlargement, new or
changed murmurs
• Carey Coombs murmurs
• Valvular heart disease
• Cardiac failure
 CLINICAL FEATURES

2. ARTHRITIS
• Most common major manifestation
• Occurs early when streptococcal antibody titres are high
• Acute painful asymmetric and migratory inflammation of the large joints:
Knees, Ankles, Elbows and Wrists
• Joints are usually red, swollen and tender for between a day and 4 weeks
• Pain responds to Aspirin
 CLINICAL FEATURES
3. SKIN LESIONS
• Erythema marginatum – 5%
• Starts as Red macules that fade in the centre
• Subcutaneous nodules – 5-7%
• Firm, painless, best felt over the extensor surfaces of bones and tendons
4. SEYDENHAM’S CHOREA (ST VITUS DANCE)
• Late neurological manifestation
• Appears at least 3 months after the episode of acute rheumatic fever
• Most of the other signs had disappeared
• Purposeless, Involuntary choreiform movements of the hands, face and
feet
INVESTIGATIONS
 INVESTIGATIONS

• ESR and CRP – useful for monitoring

• Positive throat cultures – 10-25% of cases
• ASO Titres – normal in 1/5th of adult cases
• Echocardiogram
• Mitral regurgitation
• Aortic regurgitation
• Pericardial effusion
 MANAGEMENT – ACUTE ATTACK

• IM Benzyl penicillin – 1.2 megaunits once OR

• Oral Penicillin V 250mg 4 times daily for 10 days

• PENICILLIN ALLERGIC
• Erythromycin or Cephalosporin
 MANAGEMENT – ACUTE ATTACK

• Bed Rest
• Aspirin
• Corticosteroids
• Used in carditis and severe arthritis
• Cardiac failure
• Treated as appropriate
 MANAGEMENT – SECONDARY
PREVENTION

• Secondary Prophylaxis
• IM Benzathine Penicillin 1.2mU monthly or
• Oral Pen V 250mg bd daily
• PENICILLIN ALLERGIC
• Sulfadiazine
• Erythromycin
 MANAGEMENT – SECONDARY
PREVENTION

• Given until the child is 21 years OR

• If an attack occurred in the last 5 years or patient lives in an area of
high prevalence or has an occupation with high exposure to
streptococcal infection - treatment is extended

• In those with Residual Heart Disease……

• Prophylaxis is continued until 10 years after the last attack or
• 40 years of age, whichever occurs later
 CHRONIC RHEUMATIC HEART DISEASE

• Happens in at least half of the patients with rheumatic carditis

• 2/3 of patients occur in women
• Valves affected
• Mitral Valve – 90%
• Aortic Valve
• Tricuspid Valve
• Pulmonary valve
 CHRONIC RHEUMATIC HEART DISEASE

• Mostly a sequel to Acute Rheumatic Fever

• Majority of patients lack a history of past Acute Rheumatic Fever
• Pathological process – progressive fibrosis
• Heart valves are affected but involvement of the pericardium and
myocardium contribute to heart failure
• Presents as Heart failure
• The Mitral Valve is involved in nearly all cases