High risk groups 1

Consider in people who meet the below criteria with reduced exercise tolerance or breathlessness:

  • Living in an area with high acute rheumatic fever (ARF) rates
  • Aboriginal and Torres Strait Islander people living in rural or remote settings
  • Previous or current household overcrowding (>2 people per bedroom) or low socioeconomic status
  • History of ARF
  • Family or household recent history of ARF/Rheumatic heart disease (RHD)
  • Migrant or refugee from low or middle-income country
  • Prior resident or traveller to a high ARF risk setting

Considerations in pregnancy 1

  • Moderate or severe RHD increases cardiac and adverse fetal risks
  • Regular secondary prophylaxis is safe in pregnancy and breastfeeding
  • Provide pre-conception counselling for all women with known RHD

Urgent referral 1

  • Cardiologist, obstetrician or MO/NP if suspicion of a RHD diagnosis exists or there are signs of Heart failure.

Acute rheumatic fever (ARF)

Notifiable diseases

1. What is RHD? 1

  • The immune response to Group A Streptococcus bacterium (Strep A) infection, can cause acute generalised inflammation affecting the heart, joints, brain and skin. This is called acute rheumatic fever (ARF)
  • RHD develops when the body's mitral and aortic heart valves are permanently damaged by recurrent ARF
  • RHD is classified as borderline, mild, moderate or severe
  • Many patients appear asymptomatic until they develop moderate-severe RHD, leading to Heart failure.

2. Diagnosis of RHD 1

  • Echocardiography is the primary method to detect valvular lesion(s) and diagnose RHD in patients with reduced exercise tolerance, breathlessness, a new murmur or ARF
  • Specialist cardiologist assessment of echocardiographic data will determine:
    • RHD severity (borderline, mild, moderate or severe)
    • management plan and referral
    • secondary prophylaxis schedule and cessation
    • ongoing monitoring and echocardiogram to assess need for valve repair or replacement
  • Many people with RHD do not have a documented history of ARF

3. Management of RHD 1

  • The goal of managing RHD is to prevent the progression of valve disease and ARF recurrences by:
    • regular secondary prophylaxis of intramuscular antibiotics. See Table 1.
    • swiftly identifying and addressing any skin and throat infections
  1. Supporting patient self-management
    • Provide RHD resources and education outlining:
      • how it progresses and its association with throat and skin infections
      • the signs and symptoms of recurrent ARF and of RHD. See Resource 2.
      • routine scheduled attendance to clinic for management and follow-up
      • effectiveness of prophylactic antibiotics to prevent recurrent ARF and minimise RHD
    • Encourage the patient to identify barriers to adequate lifestyle modification and medical adherence and to set goals to overcome those barriers. See Engaging our patients.
  2. Social-emotional support
    • Ensure a co-ordinated transition from paediatric to adult services for young patients
    • Address, treat and manage client’s injection pain, fear and distress. See 4.2 Benzathine benzylpenicillin administration technique
    • See Social-emotional wellbeing.
  3. Recall for secondary prophylaxis (antibiotics)
    • Place patient on the health service RHD recall system
    • Provide patient with:
      • a scheduled regimen for intramuscular benzathine benzylpenicillin G (BPG) injection. See 4.1 Secondary prophylaxis
      • date of next specialist review and echocardiogram
    • Recall patient from 21 days after the last injection to:
      • ensure injections are given no more than 28 days apart
      • minimise days at risk of streptococcal infection
    • If a patient relocates or travels, provide the prospective health service with the patients medical history and RHD action plan to ensure continuity of care
    • Contact the RHD Register and Control Program (Resource 3.) to:
      • request educational resources
      • advise of BPG administration details, echocardiogram or specialist reviews
      • advise if patient relocates or is travelling
  4. Prevent infections
    • Identify and manage all skin and throat infections promptly to prevent recurring ARF and further valve damage. See Skin (child), and Skin (adult).
  5. Dental care 1,2
    • Poor oral health increases the risk of infective endocarditis
    • Encourage and support dental hygiene and oral health check at each visit
    • Patients require routine dental review according to severity
    • Antibiotic prophylaxis is required prior to some dental procedures and heart valve surgery. See Dental caries and periodontal disease.
  6. Boarding school
    • Many rural and remote children attend boarding schools interstate. Develop a documented RHD action plan with these children and families outlining:
      • parental consent to access BPG injections and treatment
      • BPG recall and management
      • specialist medical appointments
  7. Specialist review 1
    • Ensure timely and scheduled paediatric or adult cardiologist or MO/NP review of:
      • heart and lungs
      • echocardiogram
      • throat, teeth and skin
      • Heart failure signs and symptoms
  8. Heart valve surgery
    • Heart valve replacement or repair prevents left ventricular dysfunction and severe pulmonary hypertension
    • A cardiologist will determine the severity of valve damage and decide the appropriate choice and timing of surgical intervention
    • The risks from heart valve replacement include stroke, infective endocarditis and valve thrombosis

4. Medicines for RHD 1,2,3,4

  1. Secondary prophylaxis 1,2,3,4
    • Secondary prophylaxis involves scheduled administration of BPG to prevent recurrent ARF. See Table 1.
    • Decisions to cease secondary prophylaxis are based on a clinical and echocardiographic assessment by a cardiologist or paediatrician. See Table 2.
  2. BPG administration technique 1,3
    • Initial BPG administration can determine regimen success or failure, especially for children
    • To support adherence and wellbeing, all patients should control how and where they receive their injection. Record their preference
    • Lidocaine mixed with BPG is not approved for use in Queensland
    • Prior to injection warm BPG solution to room temperature by rolling between the hands
    • Inject slowly over 2 minutes to avoid pain from solution under pressure
    • Other techniques prior to injection include:
      • distraction
      • ice pack applied to the site
      • firm pressure to the site for 10 seconds
      • applying vibrating ice pack (e.g. Buzzy®) adjacent to the injection site
      • administering pre-mixed nitrous oxide (Entonox®) during the procedure
      • applying warm pack afterwards and encouraging normal ambulation
      • administering paracetamol before injection
      • administering clonidine before injection for highly distressed children and adolescents despite other strategies

Table 1. Antibiotic regimens for secondary prevention 1,3,4

First line

Benzathine benzylpenicillin G (BPG- LA Bicillin™) ≥ 20 kg 1.2 million units; < 20 kg 600,000 units by deep IM injection every 21–28 days

Second line

  • If IM route is not possible or refused, use oral penicillin
  • Monitor adherence closely

Phenoxymethylpenicillin (penicillin V) 250 mg PO bd

Following documented penicillin allergy

Erythromycin 250 mg PO bd

  1. Anticoagulation therapy 5
  • Safe use of warfarin remains the anticoagulant of choice following a heart valve replacement or Atrial fibrillation.
  • Patients are discharged from hospital on anticoagulation therapy

Table 2. Recommended duration of secondary prophylaxis 1,3,4

Diagnosis

Duration if documented history of ARF

Duration if NO documented history of ARF

Cease if

Echocardiogram timing after cessation

Priority 3.

ARF

  • Min. 5 years after most recent ARF episode, or until 21 yo whichever is longer
  • N/A
  • > 21 yo and no ARF in last 5 years and
  • Normal echocardiogram
  • Yearly then as per Cycle of care

Priority 3.

Borderline RHD

  • Min. 10 years after most recent ARF episode, or until 21 yo whichever is longer
  • ≤ 20 yo then 2 years following diagnosis of Borderline RHD
  • If still present at 2 years, continue for further 2 years
  • > 20 yo and no ARF in last 10 years and
  • Normal echocardiogram for 2 years
  • Yearly then as per Cycle of care

Priority 3.

Mild RHD

  • Min. 10 years after most recent ARF episode, or until 21 yo whichever is longer
  • < 35 yo then 5 years min. following diagnosis of RHD or until 21 yo whichever is longer
  • > 21 yo and no ARF in last 10 years or progression of RHD and
  • Stable echocardiogram for 2 years
  • Yearly then as per Cycle of care

Priority 2.

Moderate RHD

  • Min. 10 years after most recent ARF episode or until 35 yo whichever is longer
  • < 35 yo then 5 years min. following diagnosis of RHD or until 35 yo whichever is longer
  • > 35 yo and no ARF within the last 10 years and
  • Stable echocardiogram for 2 years
  • Yearly then as per Cycle of care

* Priority 1.

Severe RHD

  • Min. 10 years after most recent ARF episode or until 40 yo whichever is longer
  • Min. 5 years following diagnosis of RHD or until 40 yo whichever is longer
  • > 40 yo and no ARF within the last 10 years and
  • Stable valvular disease /cardiac function on serial echocardiogram for 3 years or
  • Patient or family preference to cease due to advancing age and/or end of life care
  • 6 mthly then as per Cycle of care

*Note–Priority 1 classification includes:

  • patients with > 3 episodes of ARF within the last 5 years
  • pregnant women with RHD (with any severity) for the duration of their pregnancy
  • children < 5 years of age with ARF or RHD

5. Cycle of care

Cycle of care summary for rheumatic heart disease

Action

Dx

Frequency

Priority 4.

ARF or Borderline RHD Tx ceased or no RHD

Priority 3.

ARF, Borderline or Mild RHD

Priority 2.

Moderate RHD

Priority 1.

Severe RHD

Benzathine benzylpenicillin G (BPG)

-

Every 21–28, no more than 28 days apart

HW/RN review

12 mthly

4 wkly

Height

12 mthly then once only when patient stops growing

Weight

12 mthly

BMI

12 mthly

Waist circumference

12 mthly

Pulse

12 mthly

6 mthly

3 mthly

Blood pressure

12 mthly

6 mthly

3 mthly

Anticoagulation therapy

 

Nil

As recommended by specialist

INR

Nil

As recommended by specialist

ECG

Nil

12 mthly

Echocardiogram

Within
2 mths

1, 3, 5 years after diagnosis

≤ 21 years:1–2 yrly
Adult:
2–3 yrly

12 mthly

At least 6 mthly

Lifestyle modification

At each visit

Self manage education

12 mthly

4 wkly

Skin and throat

At each visit

Oral care

MO/NP review

12 mthly

6 mthly

3–6 mthly

Dentist

12 mthly

Within 3 mths of Dx then 6 mthly

Medicine review

12 mthly

Antibiotic cover

For Streptococcal infections see the Primary Clinical Care Manual

Influenza, pneumococcal and COVID-19 vaccine

Recommended. See the Australian Immunisation Handbook for schedule

Social-emotional wellbeing

At each visit

Management plan

At each visit

Specialist review

1, 3, 5 years post cessation of secondary prophylaxis

1–3 yearly

12 mthly

6 mthly

With any new symptoms or suspected disease progression

Cardiac rehab

Post heart valve surgery. Cardiac program with discharge summary

6. References

7. Resources

  1. The 2020 Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease
  2. RHD Australia website for patient support and resources
  3. The RHD Register and Control Program (ArfRhdRegister@health.qld.gov.au) Ph. 1300 135 854

Last updated: November 2024

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