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Clinical guidelines

National guide to a preventive health assessment for Aboriginal and Torres Strait Islander people Second edition

Rheumatic heart disease

Author Dr Nicolette de Zoete
Expert reviewer Professor Jonathan Carapetis

Overview

Rheumatic heart disease (RHD) is caused by long term damage to the heart muscle or heart valves as a result of acute rheumatic fever (ARF). Acute rheumatic fever is a delayed autoimmune response to a throat infection caused by Group A streptococcus (GAS) bacteria, which results in an illness that mainly affects the heart, joints, brain and skin.1,2 An aetiologic link between GAS skin infections and acute rheumatic fever has also been proposed, but further research is needed to substantiate this. In 2012, Rheumatic Heart Disease Australia published the most up-to-date prevention and management guideline for RHD. In this guideline the Australian criteria for ARF diagnosis have been revised and are shown in Table 5.1. The major changes from previous guidelines include:

  • the ability to diagnose a recurrence of ARF in a patient from a high risk group who has only one major plus one minor manifestation, provided that other, more likely diagnoses are excluded
  • the inclusion of mono-arthralgia as a minor manifestation in patients from high-risk groups
  • fever can be considered a minor manifestation based on a reliable history (in the absence of documented temperature) if anti-inflammatory medication has already been administered
  • the ability to diagnose probable ARF (level C), so as to include patients who do not satisfy the criteria for definite ARF, but in whom the clinician feels that ARF is the most likely diagnosis.
Table 5.1. Australian guideline criteria for acute rheumatic fever
 High risk groups*
Initial episode of ARF Two major or one major and two minor manifestations
Plus
Evidence of a preceding GAS infection†
Recurrent attack of ARF in a patient with known past ARF or RHD 2 major or 1 major and 1 minor or 3 minor manifestations
Plus
Evidence of a preceding GAS infection†
Probable ARF (first episode or recurrence)

A clinical presentation that falls short by either 1 major or 1 minor manifestation, or in the absence of streptococcal serology results, but one in which ARF is considered the most likely diagnosis. Such cases should be further categorised according to the level of confidence with which the diagnosis is made:

  • highly suspected ARF
  • uncertain ARF
Major manifestations Carditis (including evidence of rheumatic
valvulitis on echocardiogram)
Polyarthritis or aseptic mono-arthritis or
polyarthralgia‡
Chorea§
Erythema marginatum#
Subcutaneous nodules
Carditis (excluding subclinical evidence
of rheumatic valve disease on
echocardiogram)
Polyarthritis‡
Chorea§
Erythema marginatum
Subcutaneous nodules
Minor manifestations Mono-arthralgia
Fever ≥38°C¶
ESR ≥30 mm/hr or CRP
≥30 mg/L
Prolonged P-R interval on ECG**
Fever ≥38°C¶
Polyarthralgia or aseptic mono-arthritis‡
ESR ≥30 mm/hr or CRP ≥30 mg/L
Prolonged P-R interval on ECG**
ARF = acute rheumatic fever, CRP = C-reactive protein, ECG = electrocardiogram, ESR = erythrocyte sedimentation rate, GAS = group A streptococcus infection, RHD = rheumatic heart disease
* High risk groups are those living in communities with high rates of ARF (incidence >30 per 100 000 per year in those aged 5–14 years) or RHD (all-age prevalence >2 per 1000). Aboriginal and Torres Strait Islander people living in rural or remote settings are known to be at high risk
† Elevated or rising antistreptolysin O or other streptococcal antibody, or a positive throat culture or rapid antigen test for GAS
‡ A history of arthritis is sufficient to satisfy this manifestation. Other causes of arthritis/arthralgia should be carefully excluded
§ Rheumatic (Sydenham) chorea does not require other manifestations or evidence of preceding GAS infection, provided other causes of chorea are excluded
# Erythema marginatum is a distinctive rash. Care should be taken not to label other rashes, particularly non-specific viral exanthemas, as erythema marginatum
¶ Oral, tympanic or rectal temperature ≥38°C on admission or documented during the current illness
** Note that if carditis is present as a major manifestation, prolonged P-R interval cannot be considered an additional minor manifestation in the same person
Source: Rheumatic Heart Disease Australia 20123

Individuals with ARF are generally quite unwell, in significant pain from arthritis or arthralgia. They may require hospitalisation. Although acute episodes can be quite dramatic, ARF is not known to cause lasting damage to the brain, joints or skin (although there is some emerging evidence to suggest that chorea is sometimes associated with long term cognitive or behavioural disturbances). By contrast, damage to the heart, or more specifically the mitral and/or aortic valves, may remain once the acute episode has resolved. RHD is the most common cause of heart disease in children worldwide and is an important cause of preventable premature mortality. It is also where the greatest disparity between Aboriginal and Torres Strait Islander and non-Indigenous cardiovascular mortality exists, with the death rates in Aboriginal and Torres Strait Islander males and females being 15.1 and 23 times greater, respectively, than non-Indigenous males and females.4

Both ARF and RHD are important and preventable causes of ill health and death. They are typically associated with overcrowding, poor sanitary conditions and other aspects of socioeconomic disadvantage.5 ARF and RHD are rare in developed counties, but in Australia they are highly prevalent among Aboriginal and Torres Strait Islander people living in rural and remote areas, particularly in northern Australia. Data obtained from the Northern Territory’s RHD control program show that [in the Northern Territory] 92% of people with RHD are Aboriginal and/or Torres Strait Islander and of these 85% live in remote communities and towns. Since 2000, the prevalence rate of RHD has continued to increase, with almost 2% of the Aboriginal and Torres Strait Islander population in the Northern Territory being affected, 3.2% of whom are aged 35–44 years. The incidence of ARF remains highest in people aged 5–14 years, ranging from 150 to 380 per 100 000.6 

GAS accounts for 15–30% of all cases of pharyngitis in children between the ages of 5 and 15 years, and approximately 10% of adults with sore throats.7 In a small group of susceptible people (3–5% of the population), GAS throat infections lead to ARF and acute poststreptococcal glomerulonephritis.8 Acute poststreptococcal glomerulonephritis can also be caused by impetigo due to GAS (‘nephritogenic strains’). While outbreaks of acute poststreptococcal glomerulonephritis can occur warranting mass chemoprophylaxis of contacts, rheumatic fever outbreaks have not been described in Aboriginal and Torres Strait Islander communities, so mass chemoprophylaxis has no role in the primary prevention of ARF at a community level.5 

However, it appears that Aboriginal children in the Top End of the Northern Territory have a low throat carriage of GAS (1–3%) and symptomatic pharyngitis is rare in this population.9 In these children it appears that impetigo is the most common manifestation of GAS disease and more specifically, infection secondary to scabies infection appears to be a major contributor.

This has led to the hypothesis that pyoderma (bacterial infection of the skin), as the major manifestation of GAS infection, has a significant role in the development of ARF. In one Australian study, up to 50% of pyodermas were caused by superinfected scabies. Community based programs aimed at eradicating scabies in these endemic regions may impact on the incidence of ARF and resulting morbidity and mortality due to RHD, although this has not been proven.10,11

Treating pharyngitis in high risk populations with appropriate antibiotics has been shown to reduce the rate of acute otitis media and quinsy and to prevent individual cases of ARF that would result following symptomatic GAS pharyngitis.12,13 The preferred recommended treatment of streptococcal throat infection is intramuscular benzathine penicillin G (BPG). Oral phenoxymethyl penicillin for 10 days is a second line alternative as BPG is known to be more effective and lead to better adherence rates.

Addressing other socioeconomic factors that may contribute to the spread of GAS in the community, such as household overcrowding, is also likely to reduce the incidence of ARF.13

The risk of ARF after the first attack of GAS pharyngitis is approximately 0.3–3%, but with subsequent infection in someone who has already had ARF this risk rises to 25–75%. In addition, those who suffer carditis during their initial attack are significantly more likely to develop further carditis with subsequent streptococcal throat infections.13 Forty percent of people newly diagnosed with RHD in northern Australia have not been previously diagnosed with ARF,14 which suggests that improvements in early detection are needed.

Early detection of RHD, combined with secondary prophylaxis, is the key to minimising the severity of valvular lesions. Echocardiography has been demonstrated to be superior to auscultation in detecting subclinical RHD in high risk populations, however, the optimal screening strategy remains to be confirmed.15,16 In 2011, the World Heart Federation developed a standardised and evidence based criteria for the echocardiographic diagnosis of RHD (Table 5.2). For people aged ≤20 years, two categories, ‘definite RHD’ and ‘borderline RHD’, are included to identify individuals in high risk populations who may not have had time to develop the full echocardiographic manifestations of RHD.

Table 5.2. World Heart Federation criteria for echocardiographic diagnosis of rheumatic heart disease
1.  Echocardiographic criteria for individuals ≤20 years of age
Definite RHD (either A, B, C or D)
A. Pathological MR* and at least 2 morphological features of RHD of the mitral valve†
B. MS mean gradient ≥4 mmHg (must exclude congenital mitral valve anomalies)
C. Pathological AR‡ and at least 2 morphological features of RHD of the aortic valve§ (must exclude bicuspid aortic valve and dilated aortic root)
D. Borderline disease of both aortic and mitral valve#
Borderline RHD (either A, B or C) (in high risk populations only)
A. At least two morphological features of RHD of the mitral valve† without pathological MR or MS
B. Pathological MR*
C. Pathological AR†
Normal echocardiographic findings (all of A, B, C and D)
A. MR that does not meet all four Doppler criteria (physiological MR)*
B. AR that does not meet all four Doppler criteria (physiological AR)‡
C. An isolated morphological feature of RHD of the mitral valve (eg. valvular thickening), without any associated pathological stenosis or regurgitation
D. Morphological features of RHD of the aortic valve§ (eg. valvular thickening), without any associated pathological stenosis or regurgitation
2.  Echocardiographic criteria for individuals >20 years of age
Definite RHD (either A, B, C or D)
A. Pathological MR* and at least 2 morphological features of RHD of the mitral valve†
B. MS mean gradient ≥4 mmHg (must exclude congenital mitral valve anomalies)
C. Pathological AR‡ and at least two morphological features of RHD of the aortic valve§ (must exclude bicuspid aortic valve and dilated aortic root)
D. Pathological AR‡ and at least two morphological features of RHD of the mitral valve†
AR = aortic regurgitation, MR = mitral regurgitation, ms = mitral stenosis, rhd = rheumatic heart disease
* Pathological mr is defined as meeting all four of the following doppler criteria: (A) seen in 2 views; (B) in at least one view, jet length 2 cm; (C) peak velocity ≥3 m/s; (D) pan-systolic jet in at least one envelope
† Morphological features of rhd in the mitral valve: anterior mitral valve leaflet thickening ≥3 mm (age specific), chordal thickening, restricted leaflet motion, excessive leaflet tip motion during systole
‡ Pathological aortic regurgitation is defined as meeting all four of the following doppler criteria: (A) seen in 2 views; (B) in at least one view, jet length 2 cm; (C) peak velocity ≥3 m/s; (D) pan-systolic jet in at least one envelope
§ Morphological features of rhd in the aortic valve: irregular or focal thickening, coaptation defect, restricted leaflet motion, prolapse
# Combined ar and mr in high prevalence regions and in the absence of congenital heart disease is regarded as rheumatic
Source: Rheumatic Heart Disease Australia 20123

Although these new, standardised criteria will greatly contribute to improved diagnosis of RHD, questions remain about the role of echocardiography in screening programs. Echocardiography based screening may lead to overdiagnosis, which will require careful review to exclude RHD and avoid unnecessary treatment. A recent study in New Zealand, using portable echocardiograms and cardiology assessment to screen 1142 (predominately high risk) children aged 10–13 years, found high rates of undetected RHD. It also showed that echocardiography alone overestimated the prevalence of RHD, as it did not distinguish underlying congenital and physiological changes from RHD.17 The 2011 World Heart Federation criteria will help to minimise this. A population based survey in four sites across the north of Australia has been undertaken to determine the prevalence of RHD among Aboriginal, Torres Strait Islander and non-Indigenous children by screening children aged 5–14 years for RHD and is due to report in 2012. This study should help to determine the most sensitive and specific screening method for RHD and hopefully this information with help guide future screening programs. Until these results are published, it is not recommended that current screening programs be changed.3 

The severity of, and morbidity due to, RHD can be significantly reduced by secondary prophylaxis. Intramuscular BPG is beneficial in the prevention of recurrent ARF. It is superior to oral penicillin in the reduction of both recurrent ARF (87–96% reduction) and streptococcal pharyngitis (71–91% reduction). High levels of medication adherence are associated with regression of heart disease in approximately 50–70% of people. It is recommended that the duration of secondary prophylaxis be based on the patient’s age and time since last episode of ARF and that echocardiography be performed prior to stopping it. For individuals with probable ARF that is categorised as ‘highly suspected’ (Table 5.1), it is recommended that prophylaxis be continued for a minimum of 10 years after the most recent suspected episode of ARF or until another diagnosis is made. For those with ‘uncertain ARF’ it is recommended they continue prophylaxis for 12 months after diagnosis and then reassess. If there is no evidence of RHD then consider ceasing prophylaxis, otherwise manage as per RHD severity. For individuals with a history of ARF and no or mild RHD, prophylaxis is continued for a minimum of 10 years since last episode of ARF or age 21 (whichever is longer). For those with moderate RHD it is continued for a minimum of 10 years or until age 35 (whichever is longer) and for those with severe RHD it is continued for a minimum of 10 years or until age 40 (whichever is longer).3

The fundamental goal in long term management of chronic RHD is to prevent the need for valve surgery. Penicillin secondary prophylaxis (for prevention of recurrent ARF) is crucial to achieving this. When adherence with penicillin prophylaxis is low, there is greater need for surgical intervention and long term surgical outcomes are not as good. The specific valvular lesions of RHD are beyond the scope of this chapter, but many patients (eg. 47% of RHD patients in the Top End of the Northern Territory) will have involvement of two or more valves, most commonly mitral and aortic, although pathology in one is usually dominant. The assessment of severity of each valvular lesion is made by echocardiography as well as clinical review. Echocardiographic surveillance in individuals with a history of ARF and RHD is an important part of ongoing management with the frequency dependant on the severity of the RHD. It is also recommended that high risk individuals with murmurs suggestive of RHD are assessed with echocardiography.3

Coordinated control programs are the most effective approach to improving BPG adherence and clinical follow up of people with RHD and for promoting primary prevention of ARF. They enable the provision of education and training for healthcare providers and education and health promotion for individuals, families and the community.3 A key component of these programs is local registers of people with RHD or a history of ARF. These can assist with routine assessment and surveillance and recording of prophylaxis delivery and recall of patients. Centralised registers (jurisdictional or national level) can support the provision of prophylaxis for those who move between communities. Not only do they help increase adherence to secondary prophylaxis, and ultimately reduce the need for valve surgery, but registers improve case detection, reduce recurrences of ARF and decrease hospitalisations from ARF/RHD. In 2009, Rheumatic Health Disease Australia was established as the national coordination unit to support the control of RHD in Australia (see Resources). There are now a number of national RHD control programs in the Top End of the Northern Territory, central Australia, far northern Queensland and the Kimberley in Western Australia.3 

As part of coordinated control programs, the 2012 Rheumatic Heart Disease Australia guideline recommends that a structured care plan should be used for all individuals with a history of ARF or with established RHD and that this care plan be based on a priority classification.3 Priority classifications 1–3 replace the previous categories of severe, moderate and mild RHD respectively and are outlined in Table 5.3.

As for all patients with chronic disease, routine dental care is also important in patients with a history of ARF and/or RHD. All patients should receive education about oral hygiene, and should be referred promptly for dental assessment and treatment when required. This is especially important prior to valvular surgery (indications of which are beyond the scope of this chapter), when all oral/dental pathology should be investigated and treated accordingly. As infective endocarditis is a dangerous complication of RHD; it is recommended in the Australian antibiotic therapeutic guidelines that additional antibiotic prophylaxis be given prior to dental or surgical procedures for all patients with established RHD. Individuals with a history of ARF but no valvular damage do not require antibiotic prophylaxis.3 Although this has not been proven, its use is supported by animal models of endocarditis and empirical observations that have demonstrated a reduction in bacteraemia.18

Recommendations: Rheumatic heart disease
Preventive intervention typeWho is at risk?What should be done?How often?Level/strength of evidence
Screening All people Recommend auscultation of the heart to assess for previously undiagnosed RHD
Echocardiography is not currently recommended to screen for previously undiagnosed RHD*
Opportunistic and as part of an annual health assessment GPP3
All people with a past history of ARF or murmurs suggestive of valve disease Referral for echocardiography and subsequent follow up is recommended. See management guidelines for specific advice3 As per management guidelines 3 GPP3
Behavioural People with a past history of ARF or RHD Advise that the rate for recurrence of ARF is 50% and that there is a need for prophylactic antibiotics (see below) Opportunistic GPP5
Recommend dental hygiene, and regular review (see Chapter 4: Dental health) 6–12 monthly GPP3
Chemoprophylaxis All people in high risk communities where GAS infections are common and ARF is prevalent Any sore throat should be treated as if it is a GAS pharyngitis infection with a single intramuscular benzathine penicillin injection (preferred) or 10 days of oral penicillin (see specific management guidelines18)
Where possible this should be based on confirmation with a throat swab culture
Opportunistic GPP5
All people with GAS pharyngitis Treat with 10 days of oral penicillin or a single intramuscular benzathine penicillin injection (see specific management guidelines18)
There is no need to treat family contacts of those with GAS pharyngitis
Opportunistic IA13
All people with ARF/RHD Identify patients for secondary antibiotic prophylaxis through inclusion in local recall systems and centralised rheumatic fever register where one exists Opportunistic GPP3
Categorise patients according to their priority status (see Table 5.3) and implement a tailored secondary prevention strategy (see management guidelines for priority specific recommendations3) As per individual recall plan IA3
Recommend antibiotic prophylaxis for dental and other high risk procedures for those with established RHD (see Chapter 4: Dental health, and management guidelines18) GPP18
Environmental Communities where GAS infections are common and ARF is prevalent Assess for overcrowding and refer to social support services for housing assistance if indicated (see Chapter 7: Hearing loss)
If high rates of impetigo and underlying scabies, consider community healthy skin program (see Chapter 2: Child health)
N/A IIIB19
* Echocardiography is likely to be a superior strategy to auscultation and lead to improved case detection of subclinical RHD. The optimal echocardiography based screening strategy, however, is the subject of ongoing research and until the outcomes of this research is known routine echocardiography screening is not recommended (see preamble for more details)
Table 5.3. Priority classifications for developing management plans
ClassificationCriteria
Priority 1 (severe) Any of the following:
  • severe valvular disease
  • moderate/severe valvular lesion with symptoms
  • mechanical prosthetic valves, tissue prosthetic valves and valve repairs including balloon valvuloplasty
Priority 2 (moderate) Any moderate valve lesion in the absence of symptoms and with normal LV function
Priority 3 (mild) ARF with no evidence of RHD or trivial to mild valvular disease
Priority 4 (inactive) Patients with a history of ARF (no RHD) for whom secondary prophylaxis has been ceased
Source: Rheumatic Heart Disease Australia 20123

Resources

Updated Australian guidelines for diagnosis and management of ARF and RHD (Rheumatic Heart Disease Australia)
www.rhdaustralia.org.au

Rheumatic heart disease: all but forgotten. A 60 minute program outlining strategies for the treatment, control and eradication of ARF and RHD (Rural Health Education Foundation)
www.rhef.com.au/programs/program-1/?program_id=37.

References

  1. McDonald M. Acute rheumatic fever: a chink in the chain that links the heart to the throat? Lancet Infect Dis 2004;4:240–45.
  2. World Health Organization. WHO technical report series 923. Rheumatic fever and rheumatic heart disease. Report of a WHO Expert Consultation Committee. Geneva: WHO, 2004.
  3. Rheumatic Heart Disease Australia (ARF/RHD writing group), National Heart Foundation of Australia and the Cardiac Society of Australia and New Zealand. Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease, 2nd edn. RHD Australia, 2012 (in press).
  4. Australian Bureau of Statistics & Australian Institute of Health and Welfare. The health and welfare of Australia’s Aboriginal and Torres Strait Islander peoples, 2008. ABS cat no. 4704.0. Canberra: ABS, 2008. Cited October 2011. Available at www.aihw.gov.au/ publications/index.cfm/title/10583.
  5. Couzos S, Murray R. Aboriginal Primary healthcare: an evidence-based approach, 3rd edn. Melbourne: Oxford University Press, 2008.
  6. Parnaby MG, Carapetis JR. Rheumatic fever in Indigenous Australian children. J Paediatr Child Health 2010;46(9):527–33.
  7. Bisno AL GM, Gwaltney JM Jr, Kaplan EL, Schwartz RH. Practice guidelines for the diagnosis and management of group A streptococcal pharyngitis. Infectious Diseases Society of America. Clin Infect Dis 2002;35(2):113.
  8. Lennon D. Longitudinal study of post streptococcal disease in Auckland; rheumatic fever, glomerulonephritis, epidemiology and M typing 1981–86. NZ Med J 1988;101(Pt 2):396–8.
  9. McDonald MI. Low rates of streptococcal pharyngitis and high rates of pyoderma in Australian Aboriginal communities where acute rheumatic fever is hyperendemic. Clin Infect Dis 2006;43:683–9.
  10. Carapetis JR. Success of a scabies control program in an Australian Aboriginal community. Pediatr Infect Dis J 1997(16):494–49.
  11. Wong LC. Outcome of an interventional program for scabies in an Indigenous community. Med J Aust 2001;175:367–70.
  12. Del Mar CB GP, Spinks AB. Antibiotics for sore throat. Cochrane Database Syst Rev 2004(2):CD000023.
  13. National Heart Foundation of New Zealand. Guidelines for rheumatic fever: diagnosis, management and secondary prevention. Auckland: National Heart Foundation of New Zealand, 2006. Cited January 2012. Available at www.world-heart-federation.org/fileadmin/ user_upload/documents/RHD-net/AUS_NZ_resources/Guidelines/ RHDnet_NZ_RF_GL_1_diagnosis_management_ and_2ary_prevention.pdf.
  14. Carapetis JR. Acute rheumatic fever and rheumatic heart disease in the Top End of Australia’s Northern Territory. Med J Aust 1996;164:146–49.
  15. Carapetis JR, Hardy M, Fakakovikaetau T, et al. Evaluation of a screening protocol using auscultation and portable echocardiography to detect asymptomatic rheumatic heart disease in Tongan schoolchildren. Nat Clin Pract Cardiovasc Med 2008;5(7):411–7.
  16. Marijon E, Ou P, Celermajer DS, et al. Prevalence of rheumatic heart disease detected by echocardiographic screening. N Engl J Med 2007;357(5):470–6.
  17. Webb RH, Wilson NJ, Lennon DR, et al. Optimising echocardiographic screening for rheumatic heart disease in New Zealand: not all valve disease is rheumatic. Cardiol Young 2011;21(04):436–43.
  18. Antibiotic Expert Group. Therapeutic guidelines: antibiotic. Version 14. Melbourne: Therapeutic Guidelines Limited, 2010. Cited October 2011. Available at www.tg.org.au/?sectionid=41.
  19. National Heart Foundation of New Zealand. Guidelines for rheumatic fever: proposed rheumatic fever primary prevention programme. Auckland: National Heart Foundation of New Zealand, 2009. Cited January 2012. Available at www.toiteorapublichealth.govt.nz/ vdb/document/368.
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