Diagnosis and management of acute rheumatic fever and rheumatic heart disease in Australia

National Heart Foundation of Australia
and the Cardiac Society of Australia and New Zealand
Diagnosis and management of acute rheumatic fever and rheumatic heart disease in Australia
National Heart Foundation of Australia
and the Cardiac Society of Australia and New Zealand
Diagnosis and management
Diagnosis and management
of acute rheumatic fever
of acute rheumatic fever
and rheumatic heart disease
and rheumatic heart disease
in Australia
in Australia
An evidence-based review
An evidence-based review
–
An evidence-based r
eview
Heartsite www.heartfoundation.com.au
Heartline 1300 36 27 87
© June 2006 National Heart Foundation of Australia
PP–590
ABN 98 008 419 761

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©June 2006 National Heart Foundation of Australia. All rights reserved.
Phone (08) 8224 2888
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ISBN 1 921226 02 1
Suggested citation:
National Heart Foundation of Australia (RF/RHD guideline development working group) and the
Cardiac Society of Australia and New Zealand. Diagnosis and management of acute rheumatic
fever and rheumatic heart disease in Australia – an evidence-based review. 2006.
Please contact Heartline on 1300 36 27 87 or heartline@heartfoundation.com.au
for the following materials related to this publication:
•
Diagnosis of acute rheumatic fever (Quick reference guide for health professionals)
•
Management of acute rheumatic fever (Quick reference guide for health professionals)
•
Secondary prevention of acute rheumatic fever (Quick reference guide for health professionals)
•
Rheumatic heart disease control programs (Quick reference guide for health organisations)
•
Management of rheumatic heart disease (Quick reference guide for health professionals)

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National Heart Foundation of Australia
and the Cardiac Society of Australia and New Zealand
Diagnosis and management
of acute rheumatic fever
and rheumatic heart disease
in Australia
An evidence-based review

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Lead authors
Endorsing organisations
Professor Jonathan Carapetis (Chair);
As well as the National Heart Foundation of
Dr Alex Brown; Dr Warren Walsh.
Australia and the Cardiac Society of Australia
and New Zealand, these guidelines are endorsed
Writing group
by the following organisations.
Dr Keith Edwards; Dr Clive Had?eld;
Professor Diana Lennon; Ms Lyne?e Purton;
Dr Gavin Wheaton; and Dr Nigel Wilson.
Secretariat support
Mr Traven Lea and Ms Kelley O’Donohue.
Australian Society for
Infectious Diseases
Other reviewers and contributors
Dr Leslie E Bolitho; Dr Andrew Boyden;
Dr Christian Brizard; Dr Richard Chard;
Ms Eleanor Clune; Dr Sophie Couzos;
Dr Arthur Coverdale; Professor Bart Currie;
Australian Indigenous Doctors’ Association
Dr James Edward; Dr Tom Gentles; Professor
Marcia George; Dr Je?ery Hanna; Dr Noel
Hayman; Dr Ana Herceg; Dr Marcus Ilton;
Dr Jennifer Johns; Dr John Knight; Dr John
McBride; Dr Malcolm McDonald; Dr Johan
Morreau; Dr Michael Nicholson; Dr Ross
Nicholson; Ms Sara Noonan; Dr Briar Peat;
Dr Peter Pohlner; Dr Jim Ramsey; Dr Jenny
Reath; Ms Emma Rooney; Dr Warren Smith;
Dr Andrew Tonkin; Dr Lesley Voss; Dr Mark
National Aboriginal Community
Wenitong; Mr Chris Wilson; Dr Elizabeth
Controlled Health Organisation
Wilson; and Dr Keith Woollard.
Organisations
Australasian Society for Infectious Diseases;
Australasian Society of Cardiac and Thoracic
Surgeons; Australian College of Rural and
Remote Medicine; Australian Health Ministers’
Advisory Council; Australian Indigenous’
Doctors Association; Communicable Diseases
Network of Australia; Council of Remote Area
Disclaimer
Nurses of Australia; Internal Medicine Society of
Australia and New Zealand; National Aboriginal
This document has been produced by the
Community Controlled Health Organisation;
National Heart Foundation of Australia and the
National Heart Foundation of Australia Clinical
Cardiac Society of Australia and New Zealand
Issues Commi?ee; National Heart Foundation of
for the information of health professionals. The
New Zealand; National Strategies Heart, Stroke
statements and recommendations it contains are,
and Vascular Group; O?ce of Aboriginal and
unless labelled as “expert opinion”, based on
Torres Strait Islander Health; Royal Australasian
independent review of the available evidence.
College of Physicians; Royal Australian College
Interpretation of this document by those without
of General Practitioners; Royal College of
appropriate health training is not recommended,
Nursing Australia; and Standing Commi?ee on
other than at the request of, or in consultation
Aboriginal and Torres Strait Islander Health.
with, a relevant health professional.
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Diagnosis and management of acute rheumatic fever and rheumatic heart disease in Australia

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SUMMARY
Acute rheumatic fever (ARF) is an illness caused
There is also considerable regional variation
by a reaction to a bacterial infection, which o?en
within countries. In Australia, ARF and RHD are
results in lasting damage to heart valves. This
highly prevalent among Aboriginal and Torres
is known as rheumatic heart disease (RHD) and
Strait Islander communities, mostly a?ecting
it is an important cause of premature mortality.
young people. Aboriginal and Torres Strait
Almost all cases of RHD and associated deaths
Islander people are up to eight times more likely
are preventable.
than non-Aboriginal and Torres Strait Islander
people to be hospitalised for ARF and RHD,
The burden of ARF in industrialised countries
and nearly 20 times as likely to die.
declined dramatically during the 20th century,
due mainly to reduced transmission and be?er
The National Heart Foundation of Australia
availability of medical care. In most a?uent
(NHFA) and the Cardiac Society of Australia
populations, including much of Australia, ARF
and New Zealand (CSANZ) jointly developed
is now rare and RHD occurs predominantly in
this evidence-based review to address factors
the elderly.
contributing to inadequate diagnosis and
management of ARF and RHD in Australia.
However, ARF and RHD remain common in
The review covers diagnosis and management
many developing countries. RHD is the most
of ARF, secondary prevention and RHD control
frequent form of heart disease in children
programs, and diagnosis and management of
worldwide.
chronic RHD.
DIAGNOSIS AND MANAGEMENT OF ACUTE RHEUMATIC FEVER
ARF is an auto-immune response to bacterial
While it is widely thought that only upper
infection with group A streptococcus (GAS).
respiratory tract infection with GAS can
People with ARF are o?en in great pain and
cause ARF, there is evidence that GAS skin
require hospitalisation. Despite the dramatic
infections may play a role in certain populations,
nature of the acute episode, ARF leaves no
including Aboriginal and Torres Strait Islander
lasting damage to the brain, joints or skin.
Australians.
However, RHD may persist. People who have
ARF is predominantly a disease of children aged
had ARF previously are much more likely
5–14 years, although people can have recurrent
than the wider community to have subsequent
episodes well into their forties. The prevalence
episodes. Recurrences of ARF may cause
of RHD peaks in the third and fourth decades.
further valve damage, leading to steady
Therefore, although ARF is a disease with its
worsening of RHD.
roots in childhood, its e?ects are felt throughout
Although the exact causal pathway is
adulthood, especially in the young adult years
unknown, it seems that some strains of GAS are
when people might otherwise be at their most
“rheumatogenic” and that a small proportion
productive.
of people in any population (3–5%) have an
inherent susceptibility to ARF.
Summary
v

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Diagnosis of ARF
All patients with suspected or con?rmed ARF
Accurate diagnosis of ARF is important. Over-
should undergo echocardiography, if available,
diagnosis results in unnecessary treatment
to con?rm or refute the diagnosis of rheumatic
over a long time, while under-diagnosis leads
carditis. Echocardiographic evidence of valve
to further a?acks of ARF, cardiac damage and
damage (subclinical or otherwise), diagnosed
premature death. Diagnosis remains a clinical
by a clinician with experience in ARF and RHD,
decision, as there is no speci?c laboratory test.
may be included as a major manifestation in the
diagnosis of ARF.
The diagnosis of ARF is usually guided by the
Jones criteria and the more recent World Health
Management of ARF
Organization (WHO) criteria. In this guideline,
the Jones and WHO criteria have been further
In the ?rst few days a?er presentation, the
modi?ed to form the 2006 Australian criteria
major priority is con?rming the diagnosis.
for the diagnosis of acute rheumatic fever.
With the exception of heart failure management,
none of the treatments o?ered to patients with
ARF has been proven to alter the outcome of
Many medical practitioners in Australia
the acute episode, or the amount of damage
have never seen a case of ARF, because
to heart valves. Thus, there is no urgency to
the disease has largely disappeared from
begin de?nitive treatment. Non-steroidal anti-
the populations among which they train and
in?ammatory drugs reduce the pain of arthritis,
work. It is very important that health staff
arthralgia and fever of ARF, but can confuse
receive appropriate education about ARF
the diagnosis. Paracetamol and codeine are
before postings to remote areas.
recommended for pain relief until the diagnosis
is con?rmed. Corticosteroids are sometimes
used for severe carditis, although there is
Many of the clinical features of ARF are
no evidence that they alter the longer-term
non-speci?c, so a wide range of di?erential
outcome.
diagnoses should be considered. In a region
with high compared to low incidence of ARF,
Ideally, all patients with suspected ARF (?rst
a person with fever and arthritis is more likely
episode or recurrence) should be hospitalised
to have ARF. Some post-streptococcal syndromes
as soon as possible a?er onset of symptoms.
may be confused with ARF but these diagnoses
This ensures that all investigations are
should rarely, if ever, be made in high-risk
performed and, if necessary, the patient
populations.
observed to con?rm the diagnosis before
commencing treatment.
SECONDARY PREVENTION AND RHEUMATIC HEART DISEASE CONTROL
Secondary prevention refers to the early
Secondary prophylaxis
detection of disease and implementation of
Secondary prophylaxis with BPG is
measures to prevent recurrent and worsening
recommended for all people with a history of
disease.
ARF or RHD. Four-weekly BPG is currently
Secondary prophylaxis with benzathine
the treatment of choice, except in patients
penicillin G (BPG) is the only RHD control
considered to be at high risk, for whom 3-weekly
strategy shown to be e?ective and cost-e?ective
administration is recommended. The bene?ts
at both community and population levels.
of 3-weekly BPG injections are o?set by the
Randomised controlled trials have shown that
di?culties of achieving good adherence, even
regular administration is required to prevent
to the standard 4-weekly regimen. Prospective
recurrent ARF.
data from New Zealand showed that few, if any,
recurrences occurred among people who fully
adhered to a 4-weekly BPG regimen.
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Diagnosis and management of acute rheumatic fever and rheumatic heart disease in Australia

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Alternatives to BPG are available, although they
Infective endocarditis is a dangerous
are less e?ective and require careful monitoring.
complication of RHD and a common adverse
event following prosthetic valve replacement
•
In patients who refuse intramuscular BPG,
in Aboriginal and Torres Strait Islander
oral penicillin can be o?ered, although it is
Australians. People with established RHD
less e?ective than BPG in preventing GAS
or prosthetic valves should receive antibiotic
infections and subsequent recurrences of
prophylaxis prior to procedures expected to
ARF. For patients taking oral penicillin,
produce bacteraemia (eg dental procedures,
the consequences of missed doses must be
surgical procedures where infection is present).
emphasised, and adherence monitored.
Adherence to secondary prophylaxis
•
In patients who may be allergic to penicillin,
an allergist should be consulted. The rates
Persistent high rates of recurrent ARF in
of allergic and anaphylactic reactions to
Australia highlight the continued failure of
monthly BPG are low, and fatal reactions are
secondary prevention. In the Top End of the
exceptionally rare. There is no increased risk
Northern Territory in the 1990s, 28% of patients
with prolonged BPG use.
on secondary prophylaxis missed half or more
of their scheduled BPG injections over a
•
In patients with a con?rmed, immediate and
12-month period, while 45% of all episodes
severe allergic reaction to penicillin, a non-
of ARF were recurrences.
beta-lactam antimicrobial (eg erythromycin)
should be used instead of BPG.
A variety of factors, mainly sociological,
combine to limit the e?ectiveness of secondary
•
In pregnant patients, penicillin prophylaxis
prophylaxis. The major reasons for poor
should continue for the duration of
adherence in remote Australian Aboriginal
pregnancy to prevent recurrent ARF.
and Torres Strait Islander communities are the
There is no evidence of teratogenicity.
availability and acceptability of health services,
Erythromycin is also considered safe in
rather than personal factors such as injection
pregnancy, although controlled trials have
refusal, pain of injections, or a lack of knowledge
not been conducted.
or understanding of ARF and RHD. Adherence
•
In anticoagulated patients, BPG injections
is improved when patients feel a sense of
should be continued unless there is
personalised care and “belonging” to the clinic,
evidence of uncontrolled bleeding,
and when recall systems extend beyond the
or the international normalised ratio is
boundaries of the community.
outside the de?ned therapeutic window.
Hospitalisation for ARF provides an ideal
Intramuscular bleeding is rare when BPG
opportunity to begin secondary prophylaxis,
injections are used in conjunction with
and to educate patients and families on how
anti-coagulation therapy.
important it is to prevent future episodes of
The appropriate duration of secondary
ARF. Continuing education and support by
prophylaxis is determined by age, time since
primary care sta?, using culturally appropriate
the last episode of ARF, and potential harm
educational materials, should follow once the
from recurrent ARF.
patient has returned home.
All people with ARF or RHD should continue
Secondary prevention of further episodes of
secondary prophylaxis for a minimum of
ARF is a priority. It should include strategies
10 years after the last episode of ARF or
aimed at improving the delivery of secondary
until the age of 21 years (whichever is
prophylaxis and patient care, the provision
longer). Those with moderate or severe RHD
of education, coordinating available health
should continue secondary prophylaxis up
services and advocacy for necessary and
to the age of 35–40 years.
appropriate resources.
Summary
vii

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Strategies to promote continuing adherence
RHD control programs
include:
A coordinated control program, including
•
routine review and care planning;
specialist review and echocardiography, is
•
recall and reminder systems;
the most e?ective approach to improving
BPG adherence and clinical follow-up of
•
having local sta? members dedicated
people with RHD.
to secondary prophylaxis and coordinating
routine care;
Recommended elements of RHD control
programs include the following:
•
supporting and utilising the expertise,
experience, community knowledge and
•
a single, centralised (preferably computer-
language skills of Aboriginal health workers;
ised) ARF/RHD register for each program;
•
improving sta? awareness of diagnosis
•
a dedicated coordinator (this is critical
and management of ARF and RHD;
to the success of the program); and
•
taking measures to minimise sta? turnover;
•
integration of activities into the established
and
health system to ensure the control program
continues to function well despite sta?ng
•
implementing measures to reduce the pain
changes.
of injections (eg use a 23-gauge needle,
warm syringe to room temperature, apply
Control programs for ARF and RHD should
pressure with thumb before inserting
be evaluated using criteria for routine care
needle, deliver injection very slowly).
and key epidemiological objectives.
DIAGNOSIS AND MANAGEMENT OF CHRONIC RHEUMATIC HEART DISEASE
It is di?cult and expensive for Aboriginal and
•
adequate monitoring of anticoagulation
Torres Strait Islander people to travel to major
therapy in patients with atrial ?brillation
centres for cardiac services which are o?en
and/or mechanical prosthetic valves; and
hospital based. Although specialist outreach
•
secondary prevention with penicillin
services are improving in many regions, access
prophylaxis.
to specialist care is suboptimal in rural and
remote areas.
All patients with murmurs suggestive of valve
disease, or a past history of rheumatic fever,
Implementing guidelines on the diagnosis
require echocardiography. This will detect any
and management of chronic RHD has major
valvular lesion, and allow assessment of its
implications for Aboriginal and Torres Strait
severity and of le? ventricular (LV) size and
Islander health care services, especially in rural
systolic function. Serial echocardiographic data
and remote regions. In addition to access to
play a critical role in helping to determine the
appropriate primary care services, best practice
timing of surgical intervention.
for RHD requires:
•
access to a specialist physician and/or
The fundamental goal in long-term
cardiologist (preferably the same specialist
management of chronic RHD is to avoid,
over a long time);
or at least delay, valve surgery. Therefore,
prophylaxis with BPG to prevent recurrent
•
access to echocardiography — portable
ARF is a crucial strategy in managing
echocardiography may be required so that
patients with chronic RHD. Where adherence
all RHD patients in Australia have access to
to secondary prevention is poor, there is
echocardiography, regardless of location;
greater need for surgical intervention,
and long-term surgical outcomes are
not as good.
viii
Diagnosis and management of acute rheumatic fever and rheumatic heart disease in Australia

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