Acute rheumatic fever (ARF) and rheumatic heart disease (RHD)
  • ARF occurs after throat or skin infection with Group A beta haemolytic streptococcus (Strep A or GAS)
  • RHD is damage to the heart valves after ARF
  • Risk of RHD starts with first episode of ARF. Each episode of ARF increases risk of RHD developing or getting worse 
  • ARF and RHD are common in Northern and Central Australia among Aboriginal and Torres Strait Islander, Maori and Pacific Islander peoples
    • Those living in remote or rural areas and with household crowding at highest risk 
    • ARF most common from 5–14 years. Also occurs under 5 and between 15–35 years. Less common over 35 years
    • More common in females than males — preconception planning is essential for all females of childbearing age
  • RHD is preventable — regular injections of long acting penicillin (usually 4 weekly) prevents recurrent ARF and reduces RHD risk
  • People with moderate/severe RHD usually need heart surgery. Severe RHD can lead to heart failure, stroke, sudden death
  • ARF and RHD are notifiable in NT, WA, QLD, SA and NSW — contact state ARF/RHD control program or Public Health Unit if ARF or RHD is suspected or confirmed

Preventing ARF

  • ​Treat all skin infections throat and with antibiotics  as directed in these protocols
  • Treat scabies to reduce risk of skin infection
  • Reduce risk of Strep A infection
    • Promote good nutrition and hygiene
    • Support improved social determinants of health, eg housing, education
Suspect ARF in children or adults presenting with

Fever, sore joint/s

  • Fever, unwell
  • Painful, swollen joint/s (arthritis)
    • May be single joint — knee, ankle, elbow, wrist are common
    • May be several joints or move from 1 joint to another over days
    • Can be history of recent injury, but still need to exclude ARF
    • Also consider joint infection, other arthritis, bone infection

Heart problems (carditis)

  • New heart murmur
  • Signs of heart failure — shortness of breath, fast pulse

Movement sickness (chorea)

  • Fidgety movements that can’t be controlled but go away when asleep
    • Usually one side of body, but can be both sides
  • Often mood swings
  • No fever
  • Sometimes heart problems (carditis) — often not obvious

Note: Often no history of recent sore throat or skin infection

Ask

  • Recent throat or skin infections
  • Any previous ARF or RHD
  • Have they been prescribed regular benzathine benzylpenicillin (Bicillin L-A) injections
    • Have they missed any
  • Family history of ARF or RHD

Check

  • Calculate age-appropriate REWS
    • Adult — AVPU, RR, O2 sats, pulse, BP, Temp
    • Child (less than 13 years) — AVPU, respiratory distress, RR, O2 sats, pulse, central capillary refill time, Temp
  • Weight, BGL
  • Head-to-toe exam — with attention to possible major criteria for ARF
    • Sore or swollen joint or joints
    • Heart murmur
    • Abnormal movements — Sydenham chorea
    • Raised nodes
    • Erythema marginata (circular, blanching, snake-like skin rash) — can be hard to see on darker skin

ECG   — check for

  • Prolonged P–R interval
    • Upper limit of normal P–R interval
    • 3–11 years — 0.16 seconds
    • 12–16 years — 0.18 seconds
    • 17 years and over — 0.20 seconds
  • Second degree or complete heart block, accelerated rhythm

Do not

  • Do not give aspirin or NSAID (eg ibuprofen) without a diagnosis — can be given on medical advice after diagnosis confirmed  

Do

  • Medical consult 
    • Send to hospital if signs of heart failure
    • Otherwise transfer all suspected and confirmed cases within 24 hours 
    • If delay in coming to clinic and fever and joint pain already settled — monitor and arrange transfer within 72 hours
    • Refer for urgent echocardiogram ASAP if not done in hospital
  • Before giving antibiotics 
    • Throat swab for culture
    • Blood for ASOT, Anti-DNAse B, C reactive protein, FBC, ESR, blood cultures
  • Give  Bicillin L-A (benzathine benzylpenicillin) IM
    • Child less than 20kg — 600,000 units/1.17mL (450mg) (eg 1 x 1.17mL syringe)
    • Child 20kg or more and adult — 1,200,000 units/2.3mL (900mg) (1 x 2.3mL syringe)
    • Allergy to penicillin is rare. If penicillin allergy — doctor should get advice from allergy specialist 
  • If fever/pain — give paracetamol — adult 1g, child 15mg/kg/dose up to 1g, up to 4 times a day (qid) 
    • If paracetamol not effective (pain can be severe) — medical consult

Preventing recurrent ARF and RHD

Recurrent ARF and development of RHD can be prevented 

Everyone with history of ARF or RHD needs Bicillin L-A (benzathine benzylpenicillin) injection every 21–28 days 

  • There is an increased chance of recurrent ARF if injections are not given by the due date
  • Every day missed after day 28 is a day at risk

Bicillin L-A (benzathine benzylpenicillin) injections

  • Oral penicillin not recommended — do not use without discussion with specialist and family
  • Give as soon as person comes to clinic — do not ask them to wait
  • Give opportunistically if person in clinic prior to due date (days 21–28) and risk of non-adherence
  • Clinics need to organise a team approach to the ARF/RHD prevention program and recalls to make sure all Bicillin L-A injections are given on time
    • Use recall system for all people on regular Bicillin L-A — include mobile phones, SMS
    • Set recall reminder in person/carer's phone at each clinic visit
    • Consider offering an outreach or home visit service
    • If person travelling away from community — send reminder that will reach them (eg by mobile phone) and contact that clinic
  • Give education and support at every contact — need to know the importance of receiving injections on time
  • Give hand-held record of diagnosis and treatment to person/carer

Giving Bicillin L-A injections

First injection (and all Bicillin L-A injections) should be as pain free as possible — person may have 15 years of injections ahead of them. Be calm, respectful and reassuring. Use good technique

  • Give as deep IM injection
    • Do not use deltoid muscle of the arm
    • Ventrogluteal — preferred site OR dorsogluteal  (upper outer quadrant of buttock) OR vastus lateralis  (outside thigh)
  • Use needle provided with pre-loaded syringe
    • Do not change to smaller bore needle — more likely to get blocked
    • Do not pre-load needle — leave hollow of needle empty
  • Draw back to check not in vein (no blood in needle) — change site if needed
  • Inject slowly (2–3 minutes) or as preferred by the person

To lessen pain when giving injection

  • Ask person where they would like to receive injection
  • Ice pack to site beforehand
  • Firm thumb pressure on injection site for 30–60 seconds before giving
  • Use vibration device, eg Buzzy bee

If more relief needed — consider

  • Giving oral pain relief beforehand
  • Applying anaesthetic spray beforehand
  • Adding lidocaine (lignocaine) to injection — do not give if person has second or third degree heart block
    • Attach a drawing-up needle to 3mL syringe
    • Draw amount of Bicillin L-A needed (2.3mL for 1,200,000-unit dose and 1.2mL for 600,000-unit dose) from pre-filled syringe into the 3mL syringe 
    • Using new needle — draw up 0.5mL of 1% lidocaine or 0.25mL of 2% lidocaine into the tip of 3mL syringe
    • Do not mix — keep lidocaine in the tip of syringe
    • Push plunger up carefully to remove any air in syringe
    • Remove the drawing-up needle
    • Attach IM needle (eg 21G) to the syringe

How long to give Bicillin L-A

  • Decision to continue or stop Bicillin L-A injections only made by specialist in consultation with person — usually after echocardiogram

Table 7.5  

RHD management plan

  • Follow ‘priority classification’ and recommended follow-up — RHD Australia guidelines
  • If pregnant — see Rheumatic heart disease in pregnancy
  • Dental check within 3 months of diagnosis, then every 6 months — every 12 months if no valve damage
  • Yearly health check — adult, school-aged child
  • Ensure immunisations are up to date
  • If severe valve disease, symptoms and/or had valve surgery
    • Medical follow-up every 3–6 months
    • Specialist review and echocardiogram every 3–6 months
  • If moderate valve disease, no symptoms
    • Medical follow-up every 6 months
    • Specialist review and echocardiogram every 12 months
  • If ARF but no valve damage
    • Medical follow-up every 12 months
    • Echocardiogram every 2 years for children. Every 2–3 years for adults

Prevention of endocarditis

  • Highest risk of endocarditis (infection inside heart) in people with
    • RHD
    • Artificial heart valve
    • Heart transplant
    • History of bacterial endocarditis
    • Certain congenital heart problems
  • Preventive antibiotics recommended before dental, surgical, invasive procedures or if established infection
    • Check management plan
    • Always do medical/dental consult
  • For dental procedures involving gums, mucous membrane — extraction, implant placement, biopsy
    • Give 1 hour before procedure amoxicillin oral — adult 2g, child 50mg/kg/dose up to 2g — doses — single dose
    • OR 30 minutes before procedure amoxicillin OR ampicillin IV — adult 2g, child 50mg/kg/dose up to 2g — doses — single dose. Max rate 100mg/mL/min
    • If allergy — medical consult

Supporting resources

  • RHD Australia ARF/RHD guidelines
  • ARF/RHD  diagnosis calculator app
  • Treatment tracker app for patients
  • Giving Bicillin L-A e-learning module