Rheumatic Fever and Rheumatic heart disease

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Rheumatic Fever and Rheumatic heart disease Dr B.J. Mitchell Division Paediatric Cardiology Dept. of Paediatrics and Child Health University of Pretoria

What is RF? = Over-reaction of body s immune system against its own tissue Group A Streptococcal throat infection Body produces antibodies against infection. Antibodies see heart and other tissue as foreign and attack and damage it.

Who gets RF? Mainly children 5 15 yrs old. Third world problem of Poverty: Overcrowding Poor nutrition Limited access to primary healthcare Genetic predisposition

How do they present? 2-3 weeks after throat infection: Tiredness Poor appetite / weight loss Fever Painful joints Heart failure

Modified Jone s Criteria 5 Major Criteria Carditis Polyarthritis Sydenham chorea Erythema marginatum Subcut. nodules 5 Minor Criteria PR interval on ECG Arthralgia CRP/ ESR/ WBC Fever History of previous RF DIAGNOSIS: 1. Evidence of Strep infection 2. Two Major / one Major and two minor criteria

(Pan)Carditis Resting tachycardia Murmur ECG: PR prolonged, small complexes Heart failure Oedema Hepatomegaly Dyspnoea

Polyarthritis Large joints. Red, swollen, painful. Child won t move. Flits from joint to joint.

Sydenham Chorea Girls > boys Emotional Clumsy Fidgety Abnormal movements Hippus Basal Ganglia

Erythema Marginatum Non-itchy rash Red margin Pale center Swimming trunk distribution

Subcutaneous nodules Rarely seen Painless, firm, mobile Extensor surfaces

Prevention and treatment of RF

Primordial Prevention Socio-economic upliftment: Housing Hygiene Nutrition Access to healthcare Access to antibiotics

Primary Prevention Diagnose GAS pharyngitis Treat it! Treat it correctly!

Clinical diagnosis of GABS Four Centor criteria: 1. Fever 2. Swollen, tender anterior cervical lymph nodes 3. Tonsillar exudate 4. Absence of cough

Treatment of GAS Pharyngitis Drug Dose Freq Route Duration Benzathine Penicillin G < 27 kg: 600 000 IU > 27 kg: 1,2 mil IU Stat IMI Stat Pen VK Amoxicillin < 27 kg: 250 mg > 27 kg: 500 mg 50 mg/kg (max 1g) tds po 10 d od po 10 d Cephalexin 25-100mg/kg/d tds po 10 d AHA Guidelines Circulation March 24, 2009

Penicillin Allergy Drug Dose Freq Route Duration Clindamycin 20 mg/kg/d (max 1.8 g/d) tds po 10 d Erythromycin < 27 kg: 125 mg > 27 kg: 250 mg qid po 10 d Azithromycin 12 mg/kg/d od po 5 d Clarithromycin 15 mg/kg/d (max 250 mg bd) bd po 10 d AHA Guidelines Circulation March 24, 2009

Treatment of Acute RF Antibiotics : Penicillin IM / po Bedrest to rest heart Anti-inflammatory drugs for arthritis Monitor disease activity Manage Chorea Treat CCF

Treatment of Acute RF Drug Dose Freq Route Duration Benzathine Penicillin G < 27 kg: 600 000 IU > 27 kg: 1,2 mil IU Stat IMI Stat Pen VK 50 100 mg/kg/day qid po 10 d Clindamycin* 20 mg/kg/d (max 1.8 g/d) tds po 10 d Erythromycin* < 27 kg: 250 mg > 27 kg: 500 mg qid po 10 d * If allergic to Penicillin AHA Guidelines Circulation March 24, 2009

Follow-up after Acute RF Notify Prophylaxis plan Repeat ECHO after 3 6 mths Valve damage is progressive Often clinically silent! Mocambique: 10 x more RHD diagnosed with Echo than clinically N Engl J Med, Aug 2, 2007

RF prophylaxis AGENT DOSE ROUTE Benzathine Penicillin G < 27 kg: 600 000 IU > 27 kg: 1,2 mil IU every 3 4 weeks IMI Pen VK 250 mg bd PO Sulfadiazine* < 27 kg: 0,5 g daily > 27 kg: 1,0 g daily PO Erythromycin* 250 mg bd PO * If allergic to Penicillin AHA Guidelines Circulation March 24, 2009

Duration of Secondary Prophylaxis CATEGORY 1. CRHD with valve replacement / repair 2. RF with carditis and persistent valvular disease 3. RF with carditis, no residual valvular disease. 4. RF, no carditis, no valvular disease. Lifelong DURATION 10 yrs after last ARF episode or to age 40 yrs (or lifelong*) 10 yrs or until age 21 yrs (whichever longer) 5 yrs or until age 21 yrs (whichever longer) * If in high contact occupation

DISASTER MANAGEMENT

Tertiary Prevention Management of chronic RHD: FOLLOW UP! Ensure COMPLIANCE! Watch for complications Surgery when needed? Careers? Pregnancy

Compliance Typical prescription: Lasix 20mg tds po Slow K 1 tab bd po Digoxin 0.125mg daily po Capoten 12.5mg tds po Pen VK 250mg bd po Warfarin 5mg daily po

Could you do it?

Surgery Valve repair Preferable No Warfarin needed Valve replacement Often inevitable Need Warfarin lifelong! Repeated every 15 20 yrs

Pregnancy? High risk for mother with heart disease Warfarin is teratogenic

PREVENTABLE disease Children still die of this in SA or survive with debilitating heart disease SBAH: one valve replacement per month

Socio-economic upliftment Primary healthcare Education Identify pts with RHD F/up prophylaxis plans

REMEMBER Prevention is better than cure!