Special Report

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Rheumatic heart disease and

its control in the Pacific
Expert Rev. Cardiovasc. Ther. 7(12), 15171524 (2009)

Samantha M Rheumatic fever and rheumatic heart disease continue to be a huge public-health burden on
Colquhoun, many Pacific Island countries. Prevalence reported in some nations are some of the highest seen
JonathanR Carapetis, globally, yet many countries in the region do not have national disease registers. Despite the
will of many Pacific Island countries, there are a number of barriers to the implementation and
Joseph H Kado and
sustainability of effective coordinated prevention programs, including limited funding and
Andrew C Steer competing health priorities. In promising recent developments, a number of countries in the

Author for correspondence

4480 Oak Street, Vancouver,
BCV6H 3V4, Canada
Tel.: +1 604 875 2345
Fax: +1 604 875 2414
andrew.steer@rch.org.au
region have been able to develop or strengthen national rheumatic heart disease registers. These
registers allow for more effective delivery of secondary prophylaxis, the mainstay of disease
control in the Pacific. Primary prevention of rheumatic fever and screening for rheumatic heart
disease are important adjunctive strategies. Recent advances in screening methods, focusing
on portable echocardiography, may allow for the early detection of rheumatic heart disease in
the community.

Keywords : acute rheumatic fever developing countries epidemiology Pacific Islands rheumatic heart disease

Acute rheumatic fever (ARF) is an autoimmune
disease that follows group A streptococcal (GAS)
infection. It is the long-term cardiac sequelae that
are of greatest concern because, with a severe first
episode or with multiple episodes of ARF, an
individual is at an increased risk of developing
chronic rheumatic heart disease (RHD) [1] .
Acute rheumatic fever has all but disappeared
from industrialized countries; however, avail-
able data suggest that the disease has remained
a major problem in developing countries over the
past century [2,3] . Two global regions, Africa and
the Pacific, have been identified as hot spots for
ARF/RHD [2,3] .
Pacific region
There are 24 countries, or territories, in the
Pacific region (including Australia and New
Zealand) with diverse geography, cultures,
economies and politics. The Pacific is divided
into four main regions: Melanesia (to the west),
Polynesia (to the southeast), Micronesia (to
the north) and Australasia (to the southwest)
(F igure 1) . While the region is vast (>30 mil-
lion km 2 ), more than 95% of the region is
ocean. The total population is relatively small
(~32million people) given the size of the region
[101] . Population size varies considerably between
nations; the largest populations are in Australia
(21 million) and Papua New Guinea (6 million)
and the smallest in Tokelau (1100) [4,101] . Aside
from Australia, New Zealand and the French
and American Territories, the majority of coun-
tries in the region have gross national incomes
below US$4000 per capita [5] . Geopolitically, the
region is very diverse and includes independent
nations, as well as countries in varying levels of
political association with larger nations outside
of the region. All of these factors impact on the
delivery of acute and preventative healthcare.
Burden of RHD in the Pacific
Epidemiologic studies
For some time, RHD has been identified as an
important health issue in the Pacific by clini-
cians[6,7] . Table 1 summarizes the available RHD
data from specifically conducted epidemiologic
studies since 1985. A pooled estimate of data
from studies conducted in the period 1985
2005 suggests that the prevalence in the Pacific
in school-aged children is approximately 3.5 per
1000, second only to Sub-Saharan Africa (5.7 per
1000) and tenfold higher than that in established
market economy countries (0.3 per 1000) [3,8] .
More recently, two studies conducted among
primary school children in Fiji and Tonga using
echoc ardiography found much higher preva-
lences than previously reported from these coun-
tries [9,10] . The Tongan study found the highest
published prevalence of echocardiographically

www.expert-reviews.com 10.1586/ERC.09.145 2009 Expert Reviews Ltd ISSN 1477-9072 1517

 Special Report Colquhoun, Carapetis, Kado & Steer

USA
Northern
Mariana Islands
Marshall
Islands
Guam

Federated States of Kiribati

Palau Micronesia
Nauru Tokelau
Papua New Guinea Tuvalu
Solomon Islands

Wallis and Samoa

Futuna French Polynesia
Vanuatu American Samoa
Fiji
Islands Niue
Tonga Cook Islands
New Caledonia
Australia Pitcairn Islands

New Zealand

Figure 1. The 24 countries of the Pacific region. Melanesia: Papua New Guinea, Solomon Islands, Vanuatu, New Caledonia and Fiji;
Micronesia: Palau, Guam, Northern Mariana Islands, Federated States of Micronesia, Nauru, Marshall Islands and Kiribati;
Polynesia:Tuvalu, Wallis and Futuna, Samoa, American Samoa, Tonga, Niue, Tokelau, Cook Islands, French Polynesia and Pitcairn Islands;
Australasia: Australia and New Zealand.

confirmed RHD ever reported (33.2 per 1000; 95% CI: 30.1
40.3), but it should be noted that echocardiographs were per-
formed on all children with a heart murmur, whether clinically
significant or innocent; therefore the results of this study are not
comparable with the others in Table 1. The prevalence found in
the Fijian study (8.4 per 1000) may be more representative of the
true prevalence of clinical RHD in Pacific school-aged children.
The data on ARF incidence from the Pacific are less reliable
than those on RHD prevalence because of a paucity of studies
and inadequacy of surveillance in most countries. Rates of hospi-
talized ARF in Fiji that are consistent with the high prevalence of
RHD have not been found [11] . This may be because the diagnosis
of ARF can easily be missed in these settings. In the Northern
Territory of Australia, nearly half of all patients diagnosed with
RHD have no prior history of ARF; in most cases, earlier episodes
of ARF are misdiagnosed, possibly owing to reduced awareness by
health professionals of the disease or because some cases of ARF
are mild and, therefore, may not present for medical care [12] .
This scenario appears to be similar in the Pacific Islands, with
children commonly presenting with RHD at first clinical pre-
sentation with no recorded or reported prior episodes of ARF.
A recent review undertaken in Fijian primary-care clinics found
an incidence of ARF twice that of hospital presentations, and a
substantial number of additional cases of possible ARF presenting
to primary care in whom there was insufficient testing or clinical
documentation to allow for a definite diagnosis [13] .
The ARF burden in New Zealand is almost found exclusively
in the Maori and Pacific Island populations, and the propor-
tion of notifications in these populations has increased in recent
years, comprising 83% of all ARF notifications in 2003 and
91% in 2005 [14] . From 1996 to 2005, the New Zealand rates
for non-Maori, non-Pacific Island populations decreased from
1.2 to 0.4 per 100,000. These observations highlight the vast
and diverging disparity among disease rates when calculated by
ethnicity. Similar disparity in prevalence by ethnicity between
Pacific Island and other nonindigenous populations have been
highlighted by studies in Fiji and Hawaii [11,15] .
Informal data & economic cost
The screening research projects conducted in Fiji and Tonga used
well-defined population denominators, allowing for the accurate
calculation of RHD prevalence. There are additional data available

1518 Expert Rev. Cardiovasc. Ther. 7(12), (2009)

 Rheumatic heart disease & its control in the Pacific Special Report

Table 1. Published burden of rheumatic heart disease data from the Pacific region.
Country/region Year Prevalence per Comments Age (years) Ref.
1000 population
Melanesia
Fiji 2006 8.4 Cross-sectional survey of school children aged 514 [9]
514 years; auscultation screening followed by
echocardiographic confirmation
New Caledonia 2006 26.8 Cross-sectional school echocardiography survey of 614 [56]
primary school children
Polynesia
Tonga 2003 33.2 Cross-sectional survey of school children; 412 [10]
echocardiographic screening of children with any
heart murmur (innocent or pathological)
Samoa 1997 77.8 Cross-sectional survey of school children 517 [57]
usingauscultation without
echocardiographicconfirmation
Tonga 1986 0.7 School survey 515 [42]
(Tongatapu)
French Polynesia 1985 8.0 Community survey All ages [42]

Tonga (Euaisland) 1985 2.7 School survey auscultation only 512 [58]

Micronesia
Federated States of 1988 0.4 Hospital survey All ages [59]
Micronesia
Australia
Northern Territory 2002 17 (indigenous) Compiled from register data All ages [60]
(top end) <2 (nonindigenous)
Central 2002 13 (indigenous) Compiled from register data All ages [60]
<1 (nonindigenous)
Indigenous (top end) 1996 9.6 Hospital and community survey 514 [61]

North Queensland 1991 13.9 Community survey 518 [62]

Central 1990 7.912.3 Clinic record review >5 [63]

from other sources throughout the Pacific that aid in our under-
standing of the disease burden and, in some cases, suggest that the
disease burden may be much higher than previously calculated in
individual countries. For example, in American Samoa, a retro
spective chart audit found that there were 95 children diagnosed
with ARF between 2000 and 2006 in a pediatric population of
slightly more than 21,000, equating to a minimum annual incidence
of 65per 100,000 children aged 316 per year [16] . The authors
indicated that there were limitations to their retrospective data; how-
ever, the incidence of newly diagnosed ARF in American Samoa is
comparable to that in Samoan populations resident in Hawaii [15,16] .
A recent school-based clinical survey, undertaken in western Fiji,
utilized rapid echocardiography as the primary screening tool and
suggested that the RHD prevalence may be as high as 70 per 1000,
although this study did not use standardized methods or diagnostic
criteria, as were used in the Tongan and other Fijian studies [17] .
Rheumatic heart disease causes considerable strain on public
finance in many Pacific Island countries because of the high cost
of cardiac surgical procedures for patients with severe disease.
Samoa and Tonga spend up to 15% of their health budgets refer-
ring cases overseas for surgery [18; Fakakovikaetau T. Pers. Comm]. Some
countries receive visiting cardiac surgical teams from Australia
and New Zealand. A cardiac surgical team from Australia has vis-
ited Fiji annually for the past 20 years, and 80% of the cases oper-
ated on at any one visit are on patients with RHD [19] . The cost of
sending one patient overseas for valvular surgery is equivalent to
the annual running cost for a national RHD prevention program
in a Pacific Island country with a small population (e.g., Tuvalu
or Nauru), with the cost of two to three surgical cases equivalent
to the cost of running a prevention program in a country with a
larger population (e.g., Fiji or Vanuatu); yet, most countries in the
region do not have coordinated prevention programs. The annual
cost of running a secondary prevention program in a country such
as Fiji or Samoa is approximately US$25,000, which includes
funding for a dedicated national nurse coordinator, maintenance
of a national RHD register, training workshops for health profes-
sionals, health promotion, case finding and provision, and delivery
of benzathine penicillin G. To send a single RHD case overseas

www.expert-reviews.com 1519
 Special Report Colquhoun, Carapetis, Kado & Steer
Box 2. WHO core recommendations for a register-
based rheumatic heart disease control program.
Centralized ARF/RHD register, linked to local registers,
established within existing healthcare networks
Commitment from government, national, regional and local
services to ensure long-term funding
Activities guided by locally relevant, evidence-based guidelines
Dedicated, centrally based coordinator for each control program
Effective advisory committee
Prioritization of secondary prophylaxis
Stable supply of benzathine penicillin G
Procedures to find new cases of ARF and RHD and to monitor
burden of disease
Education for health practitioners
ARF: Acute rheumatic fever; RHD: Rheumatic heart disease.
Data from [1].
for valve repair or replacement surgery varies depending on the
country providing service, with a range of US$25,00045,000
per case [20] .
Prevention & control of RHD
There are two recognized methods of control of ARF and RHD
primary and secondary prophylaxis. Primary prophylaxis refers to
the timely and appropriate treatment of GAS pharyngitis, which
has clearly been shown to be effective in preventing ARF [2123] .
Secondary prophylaxis involves regular administration of anti
biotics (usually 3- or 4-weekly benzathine penicillin G) to prevent
recurrent ARF, which has been shown to lead to regression of
existing heart valve lesions and reduce RHD mortality [24,25] .
Primary prevention is effective at the individual level [22] , and
there are some observational data to support community-based pri-
mary prevention programs [2628] . However, the only randomized,
controlled trial of community (school)-based primary prevention
failed to show a statistically significant effect on the incidence of
ARF (relative risk: 0.81; 95% CI: 0.471.39) [29] . This was a very
high-quality and well-designed study that included over 85,000
person-years of observation. Primary prophylaxis is also less cost
effective than other approaches [28] . There are other barriers toward
the easy implementation of these programs in developing coun-
tries, including the lack of appropriate and easily accessible micro-
biologic facilities that are required for the timely confirmation of
group A streptococcal pharyngitis [30] . Secondary prevention is
effective and cost effective [31] . Delivery of benzathine penicil-
lin G is inexpensive, and large numbers of patients can be man-
aged by a relatively small number of staff at the primary health
level[16] . Achieving satisfactory levels of adherence can be difficult,
so delivering secondary prevention within a centralized register-
based program, which also includes a focus on health education
and support of families and health staff, is an important factor [32] .
With these factors in mind, the focus of efforts to control RHD in
many Pacific countries has been on secondary prevention.
Although primary and secondary prophylaxis-based control
strategies are the cornerstone of RHD control, primordial preven-
tion, including reduction in overcrowding and the alleviation of
1520
poverty, are most likely to have the greatest long-term effect in
curbing RHD. In Denmark, the incidence of ARF fell by half
prior to the introduction of penicillin, almost certainly owing to
a concomitant rise in the standard of living [33] .
A primary prevention strategy for the future will be the use
of a vaccine. There are a number of GAS vaccines in preclinical
development; however, only one vaccine has reached clinical trials
in the last 30 years [34,35] . A widely available, effective and inex-
pensive vaccine is, therefore, at least several years away [35,36] . A
vaccine that successfully prevents ARF may need to prevent both
GAS pharyngitis and GAS impetigo. In Australian Indigenous
communities, in which rates of pharyngitis are very low but in
which impetigo rates are very high, it has been hypothesized that
GAS skin infection may play a role in the pathogenesis of ARF and
RHD, although this link is yet unproven and is controversial [36,37] .
RHD control programs
The WHO first established register-based, secondary preven-
tion projects for the control of ARF and RHD in the 1970s
in seven countries in Africa, the Americas and Asia; an addi-
tional seven countries in Latin American were added in a study
coordinated by the Pan American Health Organization. Both
these projects demonstrated that, in developing countries, pub-
lic health campaigns to control RHD were feasible and cost
effective [3841] . In 1984, the Cardiovascular Unit of the WHO
in collaboration with the International Society and Federation
of Cardiology (which subsequently became the World Heart
Federation [WHF]) launched a Global Program for Control of
ARF/RHD in 16 participating countries (including one country
in the Pacific Tonga) [42] . As part of this program, almost 1.5
million school-age children were screened, and over 3000 cases
of RHD or prior ARF were detected[43] . A review of the program
highlighted its successes, including improved compliance with
secondary prophylaxis despite financial constraints[44] . In spite
of this, only a few countries expanded their programs beyond
the pilot phase, and funding for the WHO Global Program
ceased in 2001. Some countries have experienced individual suc-
cess in RHD control. A register-based control program aimed
at reducing ARF recurrences with an emphasis on education
of health professionals in Martinique and Guadaloupe in the
French Caribbean Islands demonstrated a rapid decline in ARF
incidence over 10 years from 1981. Overall, there was a 78%
reduction in the frequency of ARF in Martinique and a 74%
reduction in Guadeloupe [45] .
RHD control in the Pacific
New Zealand has led the way with register-based prevention
programs in the Pacific region. A series of disease registers were
established in the 1970s, predominantly in regions with high
rheumatic fever rates [46] . In New Zealand, ARF is a notifiable
disease with statistics collated by the Ministry of Health. A recent
study showed rates of ARF in Maori and Pacific Island popula-
tions were 10.0- and 20.7-times higher, respectively, than those in
New Zealand European and other resident populations [14] . The
Northern Territory of Australia established the first register-based
Expert Rev. Cardiovasc. Ther. 7(12), (2009)
 Rheumatic heart disease & its control in the Pacific Special Report

RHD control program in Australia in

Table 2. Rheumatic heart disease register data in selected Pacific
1997. In 2007, the Australian govern-
countries (20052009).
ment allocated more than AU$11 million
for a national RHD strategy, including Country Cases on register Cases on register Minimum all-age RHD
the establishment of a national coordina- (approximate (year at (year of most prevalence (per 1000)
tion unit, to address this overwhelming population) *
commencement) recent data) calculated from most
burden of RHD and ARF in indigenous recent register data
Australians [47] . Fiji 305 1393 1.7
In 2003, a program of supporting Pacific (837,000) (2005) (2009)
Island nations to establish RHD control New Caledonia 230 2372 10.3
programs was established, with support (231,000) (1999) (2009)
from the WHF. Vanuatu was the first Samoa 337 587 3.2
country to benefit, with the establishment (181,000) (2004) (2007)
of a register-based prevention program. In Tonga 431 598 5.9
2004, the program expanded to include (102,000) (2006) (2009)
Fiji and Samoa. The emphasis for this proj-
Tuvalu 22 50 4.3
ect was on strengthening the capacity of (12,000) (2007) (2009)
local health services to create integrated
and sustainable control programs in line Nauru 84 104 11.6
(9000) (2007) (2009)
with WHO recommendations for RHD
prevention (Box 1) [1] . Fiji and Samoa were Total 1409 5104 3.7
(1,372,000)
established as demonstration sites, with the
*
Data from [4].
addition of Tonga in 2006. National RHD RHD: Rheumatic heart disease.
registers have been developed in the three Data from [20].
demonstration sites, resulting in a marked
increase in the number of cases registered since the commence- is not known [50,51] . Studies in three countries Mozambique,
ment of the demonstration programs in 2005 (Table 2) . The major- Cambodia and Tonga have shown that echocardiography as
ity of patients presenting to hospitals and clinics with ARF are a primary screening tool for RHD is extremely sensitive; up to
recurrent cases. Therefore, a focus on education at the primary 13-times more sensitive than auscultation [10,52] . However, there
health level to enhance early diagnosis has been a central part of is debate as to whether echocardiography is overly sensitive. Part
the program in the demonstration sites [48] . In addition, screen- of the debate centers around specific technical issues in the inter-
ing activities have aimed to improve the early detection of RHD pretation of echoc ardiographs of otherwise normal children,
in school children. Effectiveness of the program has not yet been including determining normal values for regurgitation at the
formally assessed. mitral valve as measured by Doppler, and determining normal
Two regional training meetings were also held for health offi- values for mitral valve thickness as measured on 2D imaging[53] .
cials from 18 Pacific Island countries. Delegates at both meetings Fortunately, large studies are currently underway to establish vali-
issued a call-to-action document highlighting the magnitude of dated criteria for the diagnosis of RHD on echocardiograph to
the burden of RHD in the Pacific and calling on governments and determine the sensitivity and specificity of echocardiography as
international aid organizations to establish and maintain RHD a screening tool [50,51] .
control programs[102] The aforementioned Fijian screening study included a first stage
New Caledonia has a well-coordinated RHD prevention of auscultation to detect clinically significant heart murmurs, fol-
program, managed through the Department of Health, with lowed by echocardiographic confirmation [9] . The Tongan study,
activities focused on primary and secondary prevention and by contrast, included a number of cases without clinically signifi-
health promotion, as well as annual echocardiography screen- cant murmurs so-called subclinical RHD [10] . There remains
ing of school-aged children. Clinical and health promotion a lack of understanding of the natural history of cases identified
resources are available in French through the New Caledonia through the screening process with subclinical RHD. These chil-
Department of Health website and in English through the dren have no murmur detectable on auscultation and usually have
WHF RHDnet [103,104] . only subtle changes on echocardiographs. In high-prevalence set-
tings, many clinicians are often not willing to risk the development
Screening for RHD of RHD, so place most, if not all, of these children on antibiotic
The WHO recommends school-based screening for RHD to prophylaxis. There are some preliminary data available from Tonga
identify previously undiagnosed patients in high-prevalence that provide part of an answer to this issue: 23-year follow-up of
regions and populations [49] . The rationale for screening is to children found to have borderline subclinical RHD on screening
recognize patients with mild disease who stand to benefit the found that approximately 30% of the cases subsequently developed
most from secondary prophylaxis. The ideal method of screening further valvular disease [Fakakovikaetau T. Pers. Comm.] .

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 Special Report Colquhoun, Carapetis, Kado & Steer

The clinical and ethical responsibility to provide appropriate
management of children identified as having RHD comes with
screening. Therefore, screening should not be conducted until
infrastructure is in place to ensure reliable delivery of secondary
prophylaxis through a register-based system [54] .
Expert commentary & five-year view
Gaps in the disease burden data in Pacific Island countries
need to be addressed to allow all countries within the region to
determine if RHD is a local disease-control priority. An epide-
miological tool similar to the rapid assessment tool developed
for Haemophilus influenzae type b infection by the US CDC
may enable the rapid assessment of ARF/RHD disease burden
in countries where these gaps exist [55] .
An increased focus on prevention and control of RHD by the
strengthening of existing register-based programs (or develop-
ment of such programs where they are absent) in countries with
high disease burdens that improve primary care and increase
awareness of ARF and RHD is fundamental. In addition,
the high prevalence of RHD throughout the region and the
relatively small populations of many Pacific Island countries
mean that a regional collaborative approach may be appro-
priate. A regional coordinating center could provide techni-
cal assistance, as well as support for echocardiographic RHD
screening programs. Securing funding to develop and expand
RHD prevention and control activities is fundamental. To date,
small-scale funding has been secured by individual nations
and on a regional level through a number of international
nongovernment organizations. Governments, as well as clini-
cians, must prioritize RHD control in order to secure ongoing
funding and recognition from large regional organizations.
Further research is needed to define models of echocardio-
graphic screening that are practical, affordable and widely
applicable. Research currently underway in Fiji and Tonga aims
to pilot nurse-led echocardiography screening using relatively
inexpensive and portable echocardiography machines. Nurses
will undergo basic field training to allow them to determine
which children during simple echocardiographic screening
require referral for furtherassessment.
In 5 years, it is hoped that reliable and well-run, register-
based programs will be established in all Pacific countries where
RHD is an important health problem. In addition, echocar-
diography-based screening programs, and even primary prophy-
laxis programs, may also be established in some high-incidence
countries. However, none of this will occur without commit-
ment and dedicated funding from Pacific country governments
and external funding agencies.
Financial & competing interests disclosure
The authors have no relevant affiliations or financial involvement with
any organization or entity with a financial interest in or financial conflict
with the subject matter or materials discussed in the manuscript. This
includes employment, consultancies, honoraria, stock ownership or
options, expert testimony, grants or patents received or pending, or
royalties.
No writing assistance was utilized in the production of this manuscript.

Key issues
Rheumatic heart disease (RHD) is common in the Pacific and causes considerable health and economic impact.
Adequate control of RHD in the Pacific faces many barriers, including competing disease priorities, difficult access to small populations
in remote areas, migration of skilled healthcare workers and the chronic underfunding of programs.
Few countries in the Pacific have coordinated prevention and control programs in place.
A register-based secondary prevention program is the most effective method of disease control in developing countries.
An epidemiological tool is required to allow rapid assessment of disease burden in countries where this information is
currentlyunavailable.
Echocardiographic screening of children in developing countries may detect approximately ten-times as many cases of RHD than clinical
assessment alone, although the significance of mild findings on echocardiograms is not certain.
Sustainable and affordable RHD-screening methodologies are required for an early case detection.
Increased focus on prevention and control strategies could considerably reduce the need for expensive surgical intervention and could
encourage more cost-effective use of limited health funding both by local governments and by regional nongovernment organizations.

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2009
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frontpage&Itemid=1].
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Action (2008)
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do/demonstration-projects/rheumatic-
heart-disease-demonstration-projects/
pacific-islands/]
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nt&task=view&id=81&Itemid=14
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scientific-advisory-board/rheumatic-heart-
disease-network/
Affiliations
Samantha M Colquhoun
Menzies School of Health Research,
Charles Darwin University, Darwin,
Australia
and
Centre for International Child Health,
University of Melbourne, Melbourne,
Australia
Jonathan R Carapetis
Menzies School of Health Research,
Charles Darwin University, Darwin,
Australia
Joseph H Kado
Fiji Ministry of Health, Suva, Fiji
Andrew C Steer
Centre for International Child Health,
University of Melbourne, Melbourne,
Australia
and
Division of Infectious and Immunologic
Diseases, Department of Pediatrics,
University of British Columbia, Vancouver,
BC, Canada
Tel.: +1 604 875 2345
Fax: +1 604 875 2414
andrew.steer@rch.org.au
Expert Rev. Cardiovasc. Ther. 7(12), (2009)
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