Heartsite www.heartfoundation.com.au
Heartline 1300 36 27 87
© June 2006 National Heart Foundation of Australia
PP–590 ABN 98 008 419 761
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
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
Diagnosis and management of acute rheumatic fever and rheumatic heart disease in Australia – An evidence-based review
Heart Foundation Offices
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©June 2006 National Heart Foundation of Australia. All rights reserved.
This work is copyright. No part may be reproduced in any form or language without prior written
permission from the National Heart Foundation of Australia (national office). Enquiries concerning
permissions should be directed to
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
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)
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
Diagnosis and management of acute rheumatic fever and rheumatic heart disease in Australia
ii
Endorsing organisations
Writing group
Secretariat support
Other reviewers and contributors
Organisations
Disclaimer
Australian Society for
Infectious Diseases
Australian Indigenous Doctors’ Association
National Aboriginal Community
Controlled Health Organisation
Lead authors
Professor Jonathan Carapetis (Chair);
Dr Alex Brown; Dr Warren Walsh.
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ȱǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯŗś
Řǯşȱ ȱȱȱ¢ȱȱȱȱȱȱȱȱȱ
ȱȱǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯŗŜ
ŘǯŗŖȱ ȱȱȱȱȱȱǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯŗŝ
Řǯŗŗȱ ȱȱȱȱȱȱǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯŗŝ
ŘǯŗŘȱ ȱȱȱȬȱȱǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯŗş
Řǯŗřȱ ȱȱȱȱȱȱǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯŘř
řǯŗȱ ¢ȱȱȱȱȱ¢ȱȱȱȱȱȦȱ
ȱȱȱǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯŘś
řǯŘȱ ȱȱȱȱ¢ȱȱȱǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯŘŞ
řǯřȱ ȱȱěȱȱȱȱ¢ȱ¢¡ȱǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯŘŞ
řǯŚȱ ȱȱ¢ȱ¢¡ȱȱǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯŘş
řǯśȱ ȱȱȱȱȱ¢ȱȱ¢ȱ¢¡ȱȱǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯřŖ
řǯŜȱ ȱȱ¢ȱȱȱȱȱ£ȱȱȱȱǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯřŗ
řǯŝȱ ȱȱȱ¢¡ȱȱǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯřŘ
řǯŞȱ ȱȱȱ¢¡ȱȱȱǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯřŘ
řǯşȱ ȱȱȱȱȱȱȱǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯřř
řǯŗŖȱ ¢ȱȱȱȱȱȱȱȱǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯřś
řǯŗŗȱ ȱȱȱȱȱȱȱȱȱǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯřŜ
řǯŗŘȱ ȱȱȱȱȱȱȦȱȱȱȱȱǯǯǯǯǯǯǯǯǯǯřŞ
řǯŗřȱ ȱȱȱȱȱȱȦȱȱȱȱ
ȱȱǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯŚŗ
Śǯŗȱ ¢ȱȱȱȱȱȱȱǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯŚŞ
ŚǯŘȱ ¢ȱȱȱȱȱȱȱǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯśŘ
Śǯřȱ ¢ȱȱȱȱȱȱȱǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯśŝ
ŚǯŚȱ ¢ȱȱȱȱȱȱȱǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯśş
Śǯśȱ ¢ȱȱȱȱ¢ȱȱȱȱȱȱȱȱǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯǯŜś
v
Summary
SUMMARY
DIAGNOSIS AND MANAGEMENT OF ACUTE RHEUMATIC FEVER
Diagnosis and management of acute rheumatic fever and rheumatic heart disease in Australia
vi
Diagnosis of ARF
Management of ARF
SECONDARY PREVENTION AND RHEUMATIC HEART DISEASE CONTROL
Secondary prophylaxis
Many medical practitioners in Australia
have never seen a case of ARF, because
the disease has largely disappeared from
the populations among which they train and
work. It is very important that health staff
receive appropriate education about ARF
before postings to remote areas.
vii
Summary
Adherence to secondary prophylaxis
•
•
•
•
•
All people with ARF or RHD should continue
secondary prophylaxis for a minimum of
10 years after the last episode of ARF or
until the age of 21 years (whichever is
longer). Those with moderate or severe RHD
should continue secondary prophylaxis up
to the age of 35–40 years.
Secondary prevention of further episodes of
ARF is a priority. It should include strategies
aimed at improving the delivery of secondary
prophylaxis and patient care, the provision
of education, coordinating available health
services and advocacy for necessary and
appropriate resources.
Diagnosis and management of acute rheumatic fever and rheumatic heart disease in Australia
viii
RHD control programs
•
•
•
•
•
•
•
•
•
•
The fundamental goal in long-term
management of chronic RHD is to avoid,
or at least delay, valve surgery. Therefore,
prophylaxis with BPG to prevent recurrent
ARF is a crucial strategy in managing
patients with chronic RHD. Where adherence
to secondary prevention is poor, there is
greater need for surgical intervention,
and long-term surgical outcomes are
not as good.
DIAGNOSIS AND MANAGEMENT OF CHRONIC RHEUMATIC HEART DISEASE
•
•
•
•
ix
Summary
Valvular lesions in RHD
Mitral regurgitation
•
•
•
•
Mitral stenosis
•
•
•
Diagnosis and management of acute rheumatic fever and rheumatic heart disease in Australia
x
•
•
•
Aortic regurgitation
•
•
•
•
•
Aortic stenosis
•
xi
Summary
•
•
•
Pregnancy and rheumatic heart disease
1
Introduction
INTRODUCTION
1
•
•
•
•
The NHFA and CSANZ have jointly developed this review with the following aims:
• identifying the standard of care, including preventive care, that should be available to all people
• identifying areas where current management strategies may not be in line with available evidence
• in the interests of equity, ensuring that high-risk populations receive the same standard of care as
that available to other Australians.
2
3
Diagnosis and management of acute rheumatic fever and rheumatic heart disease in Australia
2
TABLE 1.1 LEVELS OF EVIDENCE FOR CLINICAL INTERVENTIONS AND GRADES OF RECOMMENDATION
LEVEL OF
EVIDENCE
STUDY DESIGN
Note
Scope of the review
•
•
•
•
•
•
•
•
•
4–7
8
Target audience
GRADE OF
RECOMMENDATION
3
Overview
1.1 PATHOGENESIS
1 OVERVIEW
5
9,10
11
12,13
1.2 EPIDEMIOLOGY
14,15
16
Diagnosis and management of acute rheumatic fever and rheumatic heart disease in Australia
4
16
17
11
18
Key points
• Acute rheumatic fever, an auto-immune response to group A streptococcus infection of the upper
respiratory tract (or skin, as has been hypothesised in some Aboriginal populations), may result in
damage to the mitral and/or aortic valves — this is known as rheumatic heart disease. Recurrences
are likely in the absence of preventive measures, and may cause further cardiac valve damage.
• Although acute rheumatic fever is rare in industrialised countries, it is a significant cause of disease
among Aboriginal and Torres Strait Islander children. Prevalence of rheumatic heart disease is also
high among these populations, with significant rates of procedures and death among young adults.
5
Diagnosis and management of acute rheumatic fever
2 DIAGNOSIS AND MANAGEMENT OF ACUTE
RHEUMATIC FEVER
2.1 IMPORTANCE OF ACCURATE DIAGNOSIS
•
•
2.2 DIFFICULTIES WITH DIAGNOSIS
2.3 CURRENT APPROACHES TO DIAGNOSIS — JONES CRITERIA AND WHO CRITERIA
19
20
21
6
Diagnosis and management of acute rheumatic fever and rheumatic heart disease in Australia
6
TABLE 2.1 2005 AUSTRALIAN GUIDELINES FOR THE DIAGNOSIS OF ACUTE RHEUMATIC FEVER
HIGH-RISK GROUPS* ALL OTHER GROUPS
or
plus
oror
plus
Notes
≥°
7
Diagnosis and management of acute rheumatic fever
Arthritis
Grade D
22
2.4 CLINICAL FEATURES OF ACUTE RHEUMATIC FEVER — MAJOR MANIFESTATIONS
Level IV, Grade C
Sydenham’s chorea
23,24
24
•
•
•
•
25–27
24,28,29
Diagnosis and management of acute rheumatic fever and rheumatic heart disease in Australia
8
30
24
31,32
1
Level IV, Grade C
Grade D
Carditis
33,34
33
35
Subcutaneous nodules
22
Erythema marginatum
22,36
9
Diagnosis and management of acute rheumatic fever
TABLE 2.2 KEY POINTS IN IDENTIFYING MAJOR MANIFESTATIONS OF ACUTE RHEUMATIC FEVER
MANIFESTATION POINTS FOR DIAGNOSIS
Arthritis •
•
•
•
•
•
Sydenham’s chorea
•
•
•
Carditis •
Subcutaneous
nodules
•
•
Erythema
marginatum
•
•
2.5 CLINICAL FEATURES OF ACUTE RHEUMATIC FEVER — MINOR MANIFESTATIONS
°
Level IV, Grade C
Elevated acute-phase reactants
×
22
Arthralgia
Fever
°
21,37
22
°
22
°
Diagnosis and management of acute rheumatic fever and rheumatic heart disease in Australia
10
Level IV, Grade C
Prolonged P-R interval and other rhythm
abnormalities
1
Level IV, Grade C
38
TABLE 2.3 UPPER LIMITS OF NORMAL OF P-R INTERVAL
AGE GROUP (YEARS) UPPER LIMIT OF NORMAL P-R INTERVAL (SECONDS)
Source
Other less common clinical features
TABLE 2.4 KEY POINTS IN IDENTIFYING MINOR MANIFESTATIONS OF ACUTE RHEUMATIC FEVER
MANIFESTATION POINTS FOR IDENTIFICATION
Arthralgia
Fever
°
Elevated acute-phase
reactants
Electrocardiogram
11
Diagnosis and management of acute rheumatic fever
2.6 EVIDENCE OF A PRECEDING GROUP A STREPTOCOCCAL INFECTION
39
22
40
41
42
43
TABLE 2.5 UPPER LIMITS OF NORMAL FOR SERUM STREPTOCOCCAL ANTIBODY TITRES IN NON-ABORIGINAL
AND TORRES STRAIT ISLANDER CHILDREN IN MELBOURNE
AGE GROUP UPPER LIMIT OF NORMAL (IU/ML)
Years ASO titre Anti-DNase B titre
Source
Streptococcal serology in high-incidence
populations
44,45
45
6,46
Level IV, Grade C