Remote Primary Health Care Manuals

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
Red Flags — Urgent Medical Consult
  • Signs of heart failure — short of breath, pink frothy sputum, swollen ankles or legs
  • Recurrent ARF

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  

Diagnosis Minimal duration of Bicillin L-A (years) Minimum age to cease Bicillin L-A
Which ever is longer
ARF No cardiac involvement 5 years after most recent episode of ARF 21
With cardiac involvement As for RHD
RHD Borderline Discuss with cardiologist
Mild History ARF 10 years after most recent episode of ARF 21
No history of ARF and under 35 5 years after RHD diagnosis 21
No history of ARF and 35 or over No prophylaxis required –
Moderate History ARF 10 years after most recent episode of ARF 35
No history of ARF and under 35 5 years after RHD diagnosis 35
No history of ARF and 35 or over No prophylaxis required –
Severe History ARF 10 years after most recent episode of ARF 40
No history ARF 5 years after RHD diagnosis 40

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