Rheumatic heart disease

Question 1

what is the definition of rheumatic heart disease?

Answer 1

delayed inflammatory complication of group A B-hemolytic streptococcal pharyngitis (GAS) -> acute tonsillitis or pharyngitis

• occurs within 2 - 4 weeks of acute infection
• immunologically mediated systemic process that may progress to rheumatic heart disease -> acute pancarditis
  - > chronic cardiac valvular changes
• more common in 5-15 years

Question 2

What is the pathophysiology of rheumatic heart disease?

Answer 2

1- acute tonsillitis/pharyngitis caused by GAS without antibiotic treatment
2- antibodies develop against strep M protein
3- cross-reaction of antibodies with nerve & myocardial proteins (myosins)
4- type II hypersensitivity reaction (IgM & IgG)
5- acute inflammatory sequela

Question 3

What are the clinical features of rheumatic valvular disease?

Answer 3

• fever, malaise, fatigue
• migratory polyarthritis
• pancarditis
• mitral stenosis
• dilated cardiomyopathy
• sydenham chorea
• subcutaneous nodules
• erythema marginatum

J<3NES
joints - pancarditis - nodules - erythema marginatum - sydenham chorea

Question 4

What is the diagnostic approach to rheumatic heart disease?

Answer 4

• evaluation for evidence of preceding GAS infection -> increased antistreptolysin O titer (ASO) -> +ive throat culture -> positive rapid GAS carb antigen detenction test
• acute rheumatic fever is diagnosed using revised Jones criteria
• cardiac workup for patients with acute rheumatic fever or suspected

Question 5

How is acute rheumatic fever diagnosed using the revised Jones criteria?

Answer 5

2 major OR 1 major + 2 minor

MAJOR
- migratory polyarthritis 
- carditis 
- Sydenham chorea 
- subcutaneous nodules 
- erythema marginatum 
MINOR 
- polyarthralgia 
- high fever (>38.5C)
- elevated ESR or CRP
- ECG showing prolonged PR interval

Question 6

What are the non specific signs of infection?

Answer 6

CBC

• leukocytosis
• anemia of chronic disease

acute phase reactants
- CRP & ESR -> elevated

Question 7

What cardiac workup should be done for suspected or confirmed acute rheumatic fever patients?

Answer 7

ECG

• prolonged PR interval
• second degree AV block
• complete heart block
• sinus tachycardia
• accelerated junctional rhythm
• ECG features of pericarditis

ECHO

• acute infection -> features of rheumatic valvulitis (mitral or aortic regurg)
• chronic -> mitral stenosis

Question 8

How is rheumatic heart disease managed?

Answer 8

• treat strep throat with penicillin or amoxicillin -> if allergic to PCN use cephalosporin or macrolides (azithromycin or clarithromycin)
• acute rheumatic fever is treated with NSAIDs or steroids
• C-reactive protein is used to monitor treatment
• treat valvular pathology

treatment of strep throat & acute rheumatic fever continues for 10 days

Question 9

Who should get secondary prophylaxis for ARF?

Answer 9

• patients with history of ARF (high risk of recurrence)
• all patients require antibiotic prophylaxis -> IM penicillin G benzathine every 4 weeks
• start immediately after completion of antibiotics for ARF
• if patient allergic to penicillin -> sulfadiazine

Question 10

How long should secondary prophylaxis be continued?

Answer 10

does the patient have carditis?

• if NO -> prophylactic antibiotics for 5 years or until patient is 21 (whichever is longer)
• if YES -> does the patient have permanent valvular heart defects? -> NO -> 10 years or until patient reaches 21
  - > YES -> 10 years or until patient reaches 40