DOI: 10.1111/1471-0528.

15938
www.bjog.org

The high prevalence and impact of rheumatic

heart disease in pregnancy in First Nations
populations in a high-income setting: a
prospective cohort study
EA Sullivan,a,b G Vaughan,b Z Li,b MJ Peek,c JR Carapetis,d,e W Walsh,f,g J Frawley,b
MGW Re mond, b
B Remenyi, L Jackson Pulver,i S Kruske,j,k S Belton,h C McLintockl
h

a
Faculty of Health and Medicine, The University of Newcastle, Newcastle, NSW, Australia b Australian Centre for Public and Population
Health Research, Faculty of Health, University of Technology Sydney, Sydney, NSW, Australia c ANU Medical School, College of Health and
Medicine, The Australian National University, Canberra, ACT, Australia d Telethon Kids Institute,The University of Western Australia, Perth,
WA, Australia e Perth Children’s Hospital, Perth, WA, Australia f The University of New South Wales, Sydney, NSW, Australia g Prince of
Wales Hospital, Sydney, NSW, Australia h Menzies School of Health Research,Charles Darwin University, Darwin, NT, Australia i The
University of Sydney, Sydney, NSW, Australia j The University of Queensland, Brisbane, Qld, Australia k Institute for Urban Indigenous
Health Ltd, Brisbane, Qld, Australia l National Women’s Health,Auckland City Hospital, Auckland, New Zealand
Correspondence: Professor EA Sullivan, Deputy Head of Faculty of Health and Medicine, The University of Newcastle, University Drive,
Callaghan, NSW 2308, Australia. Email: E.Sullivan@newcastle.edu.au

Accepted 2 September 2019. Published Online 9 October 2019.

Objective To describe the epidemiology of rheumatic heart disease
(RHD) in pregnancy in Australia and New Zealand (A&NZ).
Design Prospective population-based study.
Setting Hospital-based maternity units throughout A&NZ.
Population Pregnant women with RHD with a birth outcome of
≥20 weeks of gestation between January 2013 and December 2014.
Methods We identified eligible women using the Australasian
Maternity Outcomes Surveillance System (AMOSS). De-identified
antenatal, perinatal and postnatal data were collected and analysed.
Main outcome measures Prevalence of RHD in pregnancy.
Perinatal morbidity and mortality.
Results There were 311 pregnancies associated with women with
RHD (4.3/10 000 women giving birth, 95% CI 3.9–4.8). In
Australia, 78% were Aboriginal or Torres Strait Islander (60.4/
10 000, 95% CI 50.7–70.0), while in New Zealand 90% were
Maori or Pasifika (27.2/10 000, 95% CI 22.0–32.3). One woman
(0.3%) died and one in ten was admitted to coronary or intensive
care units postpartum. There were 314 births with seven stillbirths
(22.3/1000 births) and two neonatal deaths (6.5/1000 births).
Sixty-six (21%) live-born babies were preterm and one in three
was admitted to neonatal intensive care or special care units.
Conclusion Rheumatic heart disease in pregnancy persists in
disadvantaged First Nations populations in A&NZ. It is associated
with significant cardiac and perinatal morbidity. Preconception
planning and counselling and RHD screening in at-risk pregnant
women are essential for good maternal and baby outcomes.
Keywords First Nations, maternal health, perinatal outcomes,
rheumatic heart disease, stillbirth.
Tweetable abstract Rheumatic heart disease in pregnancy persists
in First Nations people in Australia and New Zealand and is
associated with major cardiac and perinatal morbidity.
Linked article This article is commented on by M Knight. To
view this mini commentary visit https://doi.org/10.1111/1471-
0528.15973.

Please cite this paper as: Sullivan EA, Vaughan G, Li Z, Peek MJ, Carapetis JR, Walsh W, Frawley J, Remond MGW, Remenyi B, Jackson Pulver L, Kruske S,
Belton S, McLintock C. The high prevalence and impact of rheumatic heart disease in pregnancy in First Nations populations in a high-income setting: a
prospective cohort study. BJOG 2019; https://doi.org/10.1111/1471-0528.15938.

after severe or repeated episodes of acute rheumatic fever,

Introduction
which result from an autoimmune response to Group A
Rheumatic heart disease (RHD) is the most common heart Streptococcus infection. Globally, over 33 million people
disease of children and adolescents globally. It develops are estimated to have RHD, causing >10 million disability-

ª 2019 Royal College of Obstetricians and Gynaecologists 1

 Sullivan et al.
adjusted life-years lost and 319 000 deaths per year, almost
all in low- and middle-income countries.1 Although a pre-
viously neglected cause of maternal and fetal morbidity and
mortality, RHD has emerged as a global health priority,
particularly in low-income regions where it is the most
common cardiac disease during pregnancy.2–4
Pregnancy is associated with marked changes in the cardio-
vascular system including a 40–50% increase in plasma volume
and cardiac output, variations in systemic vascular resistance
and mean arterial blood pressure, and an increase in atrial and
ventricular diameter and volume.5–7 In addition, pregnancy is
a hypercoagulable state leading to an increased risk of throm-
boembolism.5 These normal physiological changes can com-
promise the haemodynamics of women with cardiac disease,
leading to an increased risk of obstetric complications includ-
ing premature labour, pre-eclampsia and postpartum haemor-
rhage.5 They also increase the risk of cardiac failure,
pulmonary oedema, uncontrolled arrhythmias and maternal
death.7 Furthermore, the offspring of pregnant women with
cardiac disease are at increased risk of complications such as
congenital heart disease, growth restriction and preterm birth,
as well as neonatal mortality.5,7
The burden of maternal cardiovascular disease in high-in-
come countries is primarily due to adult congenital heart dis-
ease8–10 because RHD has been effectively eliminated as a
result of economic development, improved living conditions
and prevention strategies.11 However, the reduced burden of
RHD in high-income countries is not uniform; some disad-
vantaged populations remain at risk, including Aboriginal
and Torres Strait Islander populations in Australia and
Maori and Pasifika populations in New Zealand.11,12
A renewed interest in RHD in the 1990s in Australia and
New Zealand (A&NZ) resulted in the reintroduction of tar-
geted prevention initiatives, primarily focused on vulnerable
children and adolescents. These initiatives are monitored by
RHD Control Programmes and Registers in selected jurisdic-
tions. Despite increasing survival of women with RHD into
reproductive ages, no information on RHD in pregnancy has
been routinely collected. With the exception of one single-
centre study,13 there has been little research on the epidemi-
ology of women with RHD giving birth in A&NZ. Interna-
tionally, a number of studies investigating cardiac disease,
including RHD, in pregnancy have been reported but apart
from one multi-national study14 and another community-
based study in Uganda,15 these have largely comprised sin-
gle-site retrospective methodologies.3,8,16–18 To date, there
have been no prospective, national, population-based studies
of pregnant women with RHD.
This study aimed to describe the epidemiology and assess
the management of, and health outcomes for, RHD in
pregnant women and their babies in A&NZ to inform
health priorities in both disadvantaged populations in
high-income countries and low-resource settings.
2 ª 2019 Royal College of Obstetricians and Gynaecologists
Methods
Study design and population
A bi-national, prospective, population cohort study was
undertaken using the Australasian Maternity Outcomes
Surveillance System (AMOSS). AMOSS is a pregnancy
surveillance and research system involving data collectors at
nearly 300 maternity units. It captures approximately 98–
100% of women giving birth in maternity units in A&NZ.
The AMOSS methods have been described elsewhere.19 Par-
ticipating AMOSS sites responded to monthly emails
requesting information on potential cases. Data sources
included medical case notes, echocardiogram reports and
RHD register information. Enhanced case finding was also
employed in New Zealand and the Northern Territory (NT)
of Australia, based on the expected high prevalence of RHD
in these regions. This included interrogation of health infor-
mation systems at primary healthcare services and specific
clinician queries. Further details of this enhanced case-find-
ing strategy have previously been reported.19
The case definition comprised any pregnant woman
diagnosed with definite RHD according to World Heart
Federation criteria20 before or during pregnancy, and up to
42 days postpartum, with a birth outcome of ≥20 weeks of
gestation between January 2013 and December 2014. De-
identified echocardiogram reports were reviewed and esca-
lated for assessment by clinician study investigator(s) when
inconclusive. For women with more than one pregnancy
during the study, each pregnancy that met the inclusion
criteria was included as a separate data set.
Study factors
Data related to pregnancy and sociodemographic factors
included the following: maternal age, ethnicity, parity, pre-
vious caesarean section, timing of antenatal care, socio-eco-
nomic status21 and degree of remoteness.22 Medical and
obstetric complications were documented through preg-
nancy and postpartum. For Australia, socio-economic sta-
tus was classified according to residential postcode using an
index of relative socio-economic advantage and disadvan-
tage (the Socio-Economic Indexes for Areas; SEIFA).21 In
New Zealand, the New Zealand decile measures of depriva-
tion (NZDiDep2013)23 was used and transformed to match
the Australian SEIFA quintiles.
Data related to cardiac factors included the following: tim-
ing of RHD diagnosis, New York Heart Association (NYHA)
Functional Classification of heart failure,24 valve lesion(s),
atrial fibrillation, previous thromboembolic complications,
cardiac surgical intervention and use of anticoagulation.
Outcomes
Maternal outcome measures included antenatal hospital
admission, antenatal or postpartum admission to intensive

care or coronary care units (ICU/CCU), change in NYHA
status, thromboembolic events, obstetric haemorrhage,
labour outcomes and maternal death. Perinatal outcomes
included stillbirth, neonatal death, preterm birth
(<37 weeks of gestation) and admission to special care
nurseries (SCN) or neonatal intensive care units (NICU).
Statistical analysis
Descriptive statistics for nominal data are presented as
counts and percentages. Percentages were calculated based
on cases with a recorded value; where data were missing or
unknown, the denominator was altered accordingly as
shown in the data tables. Continuous measures are pre-
sented as median and interquartile range (IQR). Prevalence
estimates were calculated with 95% CI. Denominator data
were based on pro-rata A&NZ 2013/14 births.25,26 Results
were stratified into three groups: Aboriginal and Torres
Strait Islander Australians, Maori and Pasifika in New Zeal-
and (collectively referred to as First Nations people), and
Others. Differences between these three groups were
assessed. For continuous variables the Kruskal–Wallis test
was used whereas for categorical variables, the chi-square or
Fisher’s exact test was used. A P-value <0.05 was used to
infer statistical significance. Data were analysed using SPSS
software, version 24 (IBM Corporation, Somers, NY, USA).
Patient involvement
Patients were not directly involved in the design of this
study.
Core outcome set
A core outcome set was not used as none is available for
the condition of RHD in pregnancy.
Funding
National Health and Medical Research Council (Grant
#1024206), Health Research Council of New Zealand
(Grant 12/698).
Role of the funding source
The funding bodies had no role in study design, data collec-
tion, data analysis, data interpretation or manuscript writing.
Results
Prevalence
There were 311 pregnancies that met inclusion criteria with
three of these comprising twin pregnancies. One hundred
and ninety-two (62%) pregnancies were in Australia and
119 (38%) in New Zealand (see Supplementary material,
Figure S1). The prevalence of women with RHD giving
birth was 4.3/10 000 women giving birth (95% CI 3.9–4.8)
(see Supplementary material, Figure S2).
ª 2019 Royal College of Obstetricians and Gynaecologists
Rheumatic heart disease in pregnancy
One hundred and fifty (78%) Australian pregnancies
were to Aboriginal and/or Torres Strait Islander women
(60.4/10 000 Aboriginal and/or Torres Strait Islander
women giving birth, 95% CI 50.7–70.0). In New Zealand,
50 pregnancies (42%) were to Maori women (18.2/
10 000 Maori women giving birth, 95% CI 13.8–23.9) and
57 (48%) to Pasifika women (48.2/10 000 Pasifika women
giving birth, 95% CI 37.23–62.3). Nine Maori/Pasifika
women gave birth in Australia. Geographically, the highest
prevalence was observed in the NT of Australia at 74.3/
10 000 women giving birth (95% CI 55.4–93.2), corre-
sponding to 222/10 000 Aboriginal and/or Torres Strait
Islander women giving birth (95% CI 166–279) after strati-
fying by indigenous status.
Sociodemographic factors
Table 1 lists demographics and characteristics of the
cohort. Median age was 27 years (IQR 22–32 years) with
13% being teenagers. Ninety-nine (32%) women were
primipara. A significantly greater proportion of Aboriginal
and Torres Strait Islander women (79%, P < 0.001) lived
in remote locations. First Nations women exhibited greater
social disadvantage (SEIFA median 1, IQR 1–2) than Other
women (SEIFA median 3, IQR 1–4). One hundred and sev-
enteen (40%) women were obese [body mass index (BMI)
≥ 30 kg/m2] while 14 (5%) were underweight (BMI
< 18.5 kg/m2). Maori/Pasifika women had significantly
higher BMIs than other women (P < 0.001).
Antenatal care
Antenatal care and co-existing illnesses
Table 2 describes women’s antenatal care and co-existing
illnesses. One hundred and fourteen (38%) women had
their first antenatal booking visit during their first trime-
ster, and a similar proportion (38%) had their first antena-
tal booking visit at 20 weeks of gestation or later. Renal
disease was the most common comorbidity (n = 42, 14%)
with Aboriginal and Torres Strait Islander women exhibit-
ing higher prevalence than other groups (19%, P = 0.015).
Fifty-four percent smoked during their pregnancy. There
was a high level of new-onset obstetric complications,
including 42 women (14%) with gestational diabetes.
Forty-two percent (n = 129) of women were transferred
antenatally, with two-thirds of these being to access care
and pre-empt complications (n = 86, 67%). Fifty-one
(40%) of these women were transferred for management of
their RHD, while 35 (27%) were transferred due to high-
risk status of multiple comorbidities. Aboriginal and Torres
Strait Islander women were significantly more likely to be
transferred than other groups (67%, P < 0.001) with nine
women transferred interstate and seven travelling 1800 km
or more to a referral hospital. Ninety-nine (32%) women
3
 Sullivan et al.

Table 1. Demographics and characteristics of women with RHD in pregnancy

All Aboriginal Australians New Zealand Ma ori Others (A&NZ) P-value

or Torres Strait or Pasifika
Islanders

Total, n 311 150 116 45

Age, median (IQR) 27.0 (22.0–32.0) 25.5 (22.0–30.0) 26.5 (21.0–31.5) 30.0 (28.0–35.0) <0.001
Primipara, % (n) 31.9 (99/310) 28.9 (43/149) 31.9 (37/116) 42.2 (19/45) 0.242
History of caesarean section in those 27.8 (58/209) 32.1 (34/106) 26.0 (20/77) 15.4 (4/26) 0.213
with prior birth, % (n)
Remote/off-shore location, % (n) 40.8 (126/309) 78.7 (118/150) 3.5 (4/114) 8.9 (4/45) <0.001
Social disadvantage*
Quintile 1 – most disadvantaged, 66.9 (208/311) 72.0 (108/150) 74.1 (86/116) 31.1 (14/45) <0.001
% (n)
Quintiles 2–5, % (n) 33.1 (103/311) 28.0 (42/150) 25.9 (30/116) 68.9 (31/45)
BMI, median (IQR) 27.9 (22.8–34.2) 25.9 (21.8–31.2) 30.8 (25.9–37.2) 24.2 (22.8–30.8) <0.001
Underweight (< 18.5 kg/m2), % (n) 4.7 (14/296) 8.6 (12/140) 0.9 (1/111) 2.2 (1/45) 0.012
Obese (≥ 30 kg/m2), % (n) 39.5 (117/296) 30.0 (42/140) 56.8 (63/111) 26.7 (12/45) <0.001

*SEIFA quintiles (NZ deciles transformed to match SEIFA quintiles).

were admitted to higher care during pregnancy with
Maori/Pasifika women being more likely to be admitted
than other groups (45%, P < 0.001).
Cardiac history, diagnosis of RHD and complications in
pregnancy
Table 3 lists the cardiac characteristics, timing of diagnosis
and antenatal management of women with RHD in preg-
nancy. Eighty-seven percent (n = 272) of women were
diagnosed with RHD before pregnancy, 10% (n = 30) were
diagnosed during pregnancy and 3% (n = 9) postpartum.
The predominant presenting valvular lesion was mitral regur-
gitation (n = 269; 86%) while mitral stenosis was present in
116 (37%) women. Fifty (16%) women had had a prior car-
diac intervention (13 with valve replacement, 22 with valve
repair, 15 with percutaneous balloon mitral valvotomy).
Forty-five (15%) women did not have an echocardiogram
during the index pregnancy or postpartum. Of the 264 who

Table 2. Antenatal care and co-existing illnesses for women with RHD in pregnancy

All Aboriginal Australians New Zealand Ma ori Others (A&NZ) P-value

or Torres Strait Islanders or Pasifika

Timing of first antenatal visit, % (n)

1–13 weeks 37.6 (114/303) 43.9 (65/148) 30.4 (34/112) 34.9 (15/43) 0.002
14–19 weeks 24.4 (74/303) 17.6 (26/148) 25.9 (29/112) 44.2 (19/43)
≥ 20 weeks 38.0 (115/303) 38.5 (57/148) 43.8 (49/112) 20.9 (9/43)
Comorbidities, % (n)
Pre-existing diabetes 5.1 (16/311) 8.0 (12/150) 1.7 (2/116) 4.4 (2/45) 0.070
Hypertension 3.8 (9/237) 3.9 (4/103) 3.2 (3/93) 4.9 (2/41) 0.897
Renal conditions* 13.6 (42/309) 19.3 (29/150) 9.6 (11/114) 4.4 (2/45) 0.015
Smoked during pregnancy 53.8 (161/299) 75.4 (107/142) 40.9 (47/115) 16.7 (7/42) <0.001
Complications, % (n)
Gestational diabetes 13.7 (42/307) 17.3 (26/150) 8.8 (10/113) 13.6 (6/44) 0.133
Gestational hypertension 4.8 (15/306) 1.3 (2/149) 8.8 (10/113) 6.8 (3/44) 0.021
Antepartum haemorrhage 5.9 (18/307) 3.4 (5/149) 7.9 (9/114) 9.1 (4/44) 0.184
Antenatal thromboembolic event 1.6 (5/309) 1.3 (2/149) 1.7 (2/115) 2.2 (1/45) 0.884
Pre-eclampsia 2.6 (8/306) 1.3 (2/149) 4.4 (5/113) 2.3 (1/44) 0.284
Transferred antenatally for care, % (n) 41.6 (129/310) 67.3 (101/150) 16.5 (19/115) 20.0 (9/45) <0.001
Admission to HDU/ICU/CCU during 32.0 (99/309) 22.7 (34/150) 45.2 (52/115) 29.5 (13/44) <0.001
pregnancy, % (n)

*Chronic kidney disease, glomerulonephritis, pyelonephritis, recurrent urinary tract infections.

4 ª 2019 Royal College of Obstetricians and Gynaecologists

 Rheumatic heart disease in pregnancy

Table 3. Characteristics and complications of cardiac care for women with RHD in pregnancy

All Aboriginal Australians or New Zealand Ma ori or Others (A&NZ) P-value

Torres Strait Islanders Pasifika

Timing of RHD diagnosis, % (n)

Pre-pregnancy 87.5 (272/311) 88.0 (132/150) 88.8 (103/116) 82.2 (37/45) 0.019
During pregnancy 9.6 (30/311) 10.7 (16/150) 5.2 (6/116) 17.8 (8/45)
Postpartum 2.9 (9/311) 1.3 (2/150) 6.0 (7/116) 0 (0/45)
Prior cardiac intervention*, % (n) 16.1 (30/311) 11.3 (17/150) 18.1 (21/116) 26.7 (12/45) 0.037
Cardiac medication**, % (n) 17.1 (53/310) 14.7 (22/150) 18.3 (21/115) 22.2 (10/45) 0.456
Anticoagulation before, during or 25.1 (78/311) 17.3 (26/150) 30.2 (35/116) 37.8 (17/45) 0.006
after pregnancy, % (n)
Heart failure at booking, % (n)
None 56.7 (169/298) 29.4 (42/143) 92.9 (104/112) 53.5 (23/43) <0.001
NYHA class I 33.2 (99/298) 53.8 (77/143) 6.3 (7/112) 34.9 (15/43)
NYHA class II 7.7 (23/298) 14.0 (20/143) 0 (0/112) 7.0 (3/43)
NYHA class III/IV 2.3 (7/298) 2.8 (4/143) 0.9 (1/112) 4.7 (2/43)
Echocardiogram during pregnancy, 85.4 (264/309) 89.9 (133/148) 75.9 (88/116) 95.6 (43/45) 0.002
% (n)
Cardiac care visit before 20 weeks 23.3 (70/300) 31.0 (45/145) 12.3 (14/114) 26.8 (11/41) 0.002
of gestation, % (n)
Antenatal, during labour or postpartum, % (n)
Deterioration in NYHA Class
Antenatal 17.3 (51/295) 16.1 (32/143) 16.5 (18/109) 23.3 (10/43) 0.532
During labour 2.0 (6/302) 2.1 (3/144) 1.8 (2/113) 2.2 (1/45) 1.000
Postpartum 7.2 (21/293) 5.7 (8/140) 9.1 (10/110) 7.0 (3/43) 0.580
Atrial fibrillation 1.6 (5/310) 0.7 (1/150) 1.7 (2/115) 5.0 (2/45) 0.185
Endocarditis 0.3 (1/311) 0 (0/150) 0.9 (1/116) 0 (0/45) 0.518
Bleeding*** 18.6 (58/311) 10.7 (16/150) 26.7 (31/116) 24.4 (11/45) 0.002
Thromboembolism 1.9 (6/311) 1.3 (2/150) 2.6 (3/116) 2.2 (1/45) 0.632
Valve procedure 1.3 (4/300) 0.7 (1/142) 0.9 (1/115) 4.7 (2/43) 0.153

*Percutaneous balloon mitral valvuloplasty, valve repair, bioprosthetic or mechanical valve replacement.
**Diuretics, rate slowing medications, vasodilators, anti-arrhythmics.
***Antepartum and postpartum bleeding of any volume (including antepartum haemorrhage and postpartum haemorrhage).

did, 182 (69%) had the echocardiogram performed at
20 weeks of gestation or later. One hundred and three (33%)
women did not have a cardiac care consultation. Five women
experienced a thromboembolism (one with an antenatal pul-
monary embolism). Of these, two had prior mitral valve
replacements. Five women experienced atrial fibrillation and
two had cerebrovascular accidents (one of whom had a
mechanical valve replacement). Nine women were recom-
mended for valvular surgery or other cardiac-indicated inter-
ventions postpartum; three of these had been diagnosed with
RHD during pregnancy or postpartum.
Anticoagulation
Thirty-one (10%) women were prescribed anticoagulants
during pregnancy with 14 (5%) women prescribed antico-
agulants at the booking visit. Low-molecular-weight hep-
arin was most commonly prescribed (27/31). Nine women
were on a pre-pregnancy warfarin regimen. Four of these
continued warfarin into the first trimester.
Mode of birth and maternal outcomes
Table 4 details the birth event and postpartum complica-
tions. Ninety-two women (30%) had a caesarean section.
One-third of these were undertaken due to complications
relating to RHD and 20 (22%) were under general anaes-
thetic. Six (2%) women exhibited deterioration of NYHA
classification during labour and 21 (7%) postpartum
(NYHA III three women and NYHA IV eight women).
Perinatal antibiotic coverage was given to 147 (47%)
women; there was one episode of endocarditis. Thirty-two
(10%) women had a postpartum haemorrhage of
≥ 1000 ml (range 1000–4000 ml). Fifty-nine (19%) women
were admitted to higher care units postpartum including
30 (10%) women to ICU/CCU. The majority of these
admissions were for management of issues related to RHD.
There was one maternal death, giving a maternal fatality
rate of 0.3%. This woman gave birth by caesarean section
under general anaesthetic at 30 weeks of gestation and died
4 days later from causes not directly related to RHD. Two

ª 2019 Royal College of Obstetricians and Gynaecologists 5

 Sullivan et al.

Table 4. Labour and maternal outcomes for women with RHD in pregnancy and perinatal outcomes for their babies

All Aboriginal Australians or New Zealand Others A&NZ P-value

Torres Strait Islanders ori or Pasifika
Ma

Maternal outcomes, % (n)

Maternal death 0.3 (1/311) 0.7 (1/150) 0 (0/116) 0 (0/45) 1.000
Induced labour in cases that 39.8 (100/251) 40.2 (47/117) 37.0 (37/100) 47.1 (16/34) 0.582
laboured
Birth by caesarean section 29.6 (92/311) 32.0 (48/150) 24.1 (28/116) 35.6 (16/45) 0.241
General anaesthesia used 21.7 (20/92) 10.4 (5/48) 35.7 (10/28) 31.3 (5/16) 0.023
RHD indication 31.5 (29/92) 37.5 (18/48) 17.9 (5/28) 37.5 (6/16) 0.199
Urgent caesarean section* 53.7 (51/89) 53.2 (25/47) 66.7 (18/27) 53.3 (8/15) 0.499
Postpartum haemorrhage 10.4 (32/309) 4.7 (7/150) 15.7 (18/115) 15.9 (7/44) 0.006
≥ 1000 ml
Transferred during labour or 23.8 (69/290) 35.6 (48/135) 14.9 (17/114) 9.8 (4/41) <0.001
postpartum
Admitted to ICU/HDU/CCU** 19.0 (59/311) 19.3 (29/150) 14.7 (17/116) 28.9 (13/45) 0.019
postpartum
Fetal/neonatal outcomes
Mortality, % (n)
All deaths 2.9 (9/314) 3.9 (6/152) 2.6 (3/117) 0 (0/45) 0.420
Stillbirth 2.2 (7/314) 2.6 (4/152) 2.6 (3/117) 0 (0/45) 0.763
Neonatal deaths after live birth 0.7 (2/307) 1.4 (2/148) 0 (0/114) 0 (0/45) 0.641
Gestational age (weeks), median 38.0 (37.0–39.0) 38.0 (36.0–39.0) 39.0 (38.0–39.5) 39.0 (38.0–40.0) 0.023
(IQR)
Preterm birth (<37 weeks), % (n) 20.7 (65/314) 27.0 (41/152) 14.0 (17/117) 15.6 (7/45) 0.310
Live births only
Birthweight (g), median (IQR) 3110 (2660–3530) 2955 (2421–3320) 3345 (2923–3845) 3120 (2840–3373) <0.001
Low birthweight (< 2500 g), % 18.6 (57/307) 26.4 (39/148) 10.5 (12/114) 13.3 (6/45) 0.003
(n)
Apgar score < 7, % (n) 5.2 (16/306) 6.8 (10/147) 4.4 (5/114) 2.2 (1/45) 0.412
Admission to SCN/NICU***, % (n) 33.6 (103/307) 43.2 (64/148) 24.6 (28/114) 24.4 (11/45) 0.002
Required resuscitation, % (n) 22.4 (68/303) 23.0 (34/148) 21.6 (24/111) 22.7 (10/44) 0.982
Hospital outcome – transferred to 8.2 (25/305) 11.6 (17/146) 6.1 (7/114) 2.2 (1/45) 0.072
health facility, % (n)

*Royal Australian and New Zealand College of Obstetricians and Gynaecologists (RANZCOG) categories 1 or 2.
**Intensive care unit/High dependency unit/Coronary care unit.
***Special care nursery/Neonatal intensive care unit.

women experienced a thromboembolism postnatally. Four
women had cardiac surgery or interventions postpartum.
Perinatal outcomes
There were 314 babies born, with seven stillbirths (22.3/
1000 births) (see Table 4). The rate of stillbirth was higher
in women who were taking anticoagulation during preg-
nancy [3/32 (9.4%) versus 4/282 (1.4%), P = 0.025]. There
were two neonatal deaths (6.5/1000 births). Seven of the
nine women with perinatal deaths had existing maternal
comorbidities (autoimmune disease, obesity, diabetes,
hypertension and/or renal disease).
One in five babies was born preterm: nine (3%) extremely
preterm (< 28 weeks); ten (3%) very preterm (28–< 32 weeks),
46 (15%) moderately preterm (32 to < 37 weeks). Fifty-seven
(19%) live-born babies recorded low birthweights and 47
(15%) were small-for-gestational-age (birthweight < 10th cen-
tile for the gestational age of the Australian population norm).
One-third of babies were admitted to SCN/NICUs with Abo-
riginal and Torres Strait Islander babies being most likely to
receive higher care (43%, P = 0.002).
RHD diagnosis
Thirty-nine (13%) women were newly diagnosed with RHD
during pregnancy or postpartum. Late diagnosis was associated
with higher rates of deterioration in NYHA classification (31
versus 17%, P = 0.024), greater use of general anaesthesia for
caesarean births (57 versus 15%, P = 0.002), and higher rates of
maternal admission to ICU/CCU postpartum (21 versus 9%,
P = 0.039). The babies of women with late diagnosis of RHD
were more likely to have low Apgar scores than those of women
diagnosed pre-pregnancy (Apgar < 7, 13 versus 4%, P = 0.035).

6 ª 2019 Royal College of Obstetricians and Gynaecologists


Discussion
Main findings
We report, for the first time, national population-wide data
on the prevalence, management and outcomes of RHD in
pregnancy. These data reveal the high prevalence of RHD
in pregnancy, and associated morbidity, within First
Nations populations of A&NZ. However, the spectrum of
morbidity and extent of mortality associated with this con-
dition was relatively low. Access to tertiary health care,
including specialist obstetric, cardiovascular and perinatal
care, and high-care units may partly account for this.
Strengths and limitations
Our study is the first to employ a prospective population-
based design with active case finding of pregnancies compli-
cated by RHD. A robust case definition referenced echocar-
diogram reports and World Heart Federation criteria for the
diagnosis of RHD.20 One limitation of the study is that some
data were incomplete because some women accessed health
care at a number of sites and health data were not always
shared between these. A further limitation is the potential
under-reporting of Aboriginal and Maori/Pasifika status in
Australia. Women included in this study had high levels of
pre-existing illness and were therefore at risk of a spectrum
of pregnancy complications with inferior outcomes. This
may have contributed to the poorer outcomes observed in
this study for pregnant women with RHD and their babies.
Interpretation
Prevalence
Although the overall prevalence of RHD in pregnancy in
A&NZ observed in this study (0.04%) is low in comparison
to lower-income countries,1,27 rates varied substantially
according to First Nations status and geography. Aboriginal
and Torres Strait Islander women living in NT experienced a
rate of 2.22%, fifty times higher than the bi-national preva-
lence. This finding is on a par with the estimated prevalence
of RHD in all First Nations people in the NT (2.5%),28 sug-
gesting that there is no reduction in birth rates among RHD-
affected women in this population. Furthermore, while
A&NZ are high-income countries, the rates of RHD in preg-
nancy observed in Aboriginal and Torres Strait Islander and
Maori/Pasifika women are comparable to those reported
from low-income settings including Eritrea (2.3%),27 Uganda
(1.5%)15 and South Africa (0.1–0.9%).4
Antenatal care, complications and maternal and perinatal
morbidity
Antenatal care received by women in this study was subop-
timal. Only 38% received antenatal care within their first
trimester compared with 60% of all Australian women and
ª 2019 Royal College of Obstetricians and Gynaecologists
Rheumatic heart disease in pregnancy
54% of Aboriginal and Torres Strait Islander women giving
birth in 2014.25 Furthermore, the rate of women in our
cohort commencing antenatal care after 20 weeks of gesta-
tion was three times the overall Australian rate (38 versus
12%).25 In terms of cardiac care, one-third of women
received no antenatal cardiac care and almost 15% did not
have an echocardiogram during pregnancy. These findings
suggest that greater efforts are required to deliver equitable
services and to provide First Nations women with adequate
information and assistance to seek out culturally appropri-
ate early antenatal care.29 Furthermore, improved clinical
services to facilitate early case detection would enable ear-
lier diagnosis and provision of RHD care, and better plan-
ning of antenatal cardiac management and birth to
minimise potential complications.30,31
Despite significant maternal morbidity, there was only
one maternal death (0.3%). This finding is higher than
recent estimates of maternal mortality in Australia (0.007%
overall, 0.02% in First Nations Australians25), but is sub-
stantially lower than rates reported in studies of RHD in
pregnancy in low-income settings including Nepal (4%),32
India (2%)33 and Senegal (34%).17 This most likely reflects
the availability of high-quality tertiary health care, includ-
ing specialist obstetric, cardiovascular and perinatal care, in
A&NZ.
Our results indicate that RHD in pregnancy is associ-
ated with poorer perinatal outcomes as has been previ-
ously reported.4,16,34 There was a high risk of prematurity
with 21% of neonates born preterm compared with 9%
overall in Australia and 14% for babies born to Aborigi-
nal mothers.25 Similarly, one-in-three neonates was admit-
ted to an SCN/NICU compared with one-in-seven of the
total newborn population in Australia.25 The rate of still-
birth of 23/1000 births was over double the rate of still-
birth reported for Aboriginal mothers giving birth in
Australia in 2014 (9/1000 births)25 and for Maori/Pasifika
women giving birth in New Zealand (6.6–7.9/1000
births)26 but still substantially lower than reported from
low-income settings (8% mortality in Senegal,17 2% in
India33 and 16% in Nepal32).
Differences between ethnic groups
There were a number of differences in baseline characteris-
tics observed between First Nations groups that may
account for some of the differences observed in secondary
outcomes. Aboriginal and Torres Strait Islander women
had lower BMIs, smoked more and had higher rates of
renal disease, which may have resulted in the lower gesta-
tional ages and birthweights, and higher rates of admission
to SCN/NICU, observed for their babies. Maori/Pasifika
women had higher rates of prior cardiac interventions and
use of anticoagulants, which may account for their higher
rates of postpartum haemorrhage.
7
 Sullivan et al.
Public health implications
The burden of maternal comorbidities observed in our
study was significant and compounded the inherent risks
associated with RHD in pregnancy. Intensive specialist care
was required to address this and such care is particularly
difficult to provide in remote or resource-limited settings.
The prevalence of RHD in some low-income settings,
including regions of Africa, is estimated to be equivalent to
that seen in Aboriginal people in the NT,1 suggesting that
between 2 and 3% of pregnancies in such regions are com-
plicated by RHD. Results from the REMEDY study35 indi-
cate that the severity of RHD cases in Africa is greater than
in A&NZ. This, coupled with the reality that pregnant
women with RHD in developing nations may not have
access to the same level of tertiary care available in A&NZ,
suggests that these women may have poorer outcomes than
those described here. This implies that RHD poses a signif-
icant unrecognised cause of maternal mortality and mor-
bidity in such regions.
We found that the rate of stillbirth was significantly
higher in women receiving anticoagulation during preg-
nancy. This finding underlines the importance of precon-
ception care and counselling regarding pregnancy planning,
valvular interventions, and choice of anticoagulation and
its potential maternal and/or fetal risk. It also highlights
the imperative for specialist care and monitoring during
pregnancy, particularly when transitioning between differ-
ent anticoagulant therapies. Discussion with women to pro-
mote informed decision-making helps avoid the risks
associated with a lack of adherence to anticoagulation. Fur-
thermore, women with mechanical valve replacements rep-
resent a high-risk group due to the need for ongoing
anticoagulation, hence valvular repair or bioprostheses may
be a preferred option in women of child-bearing age.36–38
Almost one in seven women included in this study was
newly diagnosed with RHD with late diagnosis being asso-
ciated with a number of poorer maternal and neonatal out-
comes. These findings suggest the need for targeted
screening of nulliparous women at the first antenatal visit
in regions where RHD is endemic.
Conclusion
Rheumatic heart disease in pregnancy is a preventable pub-
lic health problem rarely encountered in high-income
countries. However, this prospective bi-national study con-
firmed high rates of RHD in pregnancy in disadvantaged
First Nations populations in A&NZ. Despite women expe-
riencing varying degrees of cardiac compromise, significant
rates of co-morbidities, and a high burden of inferior peri-
natal outcomes, maternal and baby outcomes were better
than those reported in low-income settings. The high rates
of complications observed in this cohort emphasise the
8 ª 2019 Royal College of Obstetricians and Gynaecologists
need for preconception planning and counselling regarding
fertility management, RHD screening in at-risk pregnant
women, and a trained health workforce to ensure early
diagnosis and management of complications. In addition,
continuing efforts to address the drivers of RHD, including
poverty, poor housing and health inequity, are vital to
reduce the burden of RHD. The public health paradox is
that preventing RHD appears more difficult than managing
its costly and potentially tragic sequelae.
Disclosure of interests
ES, GV, ZL, JF and MR report that their institution admin-
istered the grants detailed in the Funding section. The
remaining authors have no disclosures. Completed disclo-
sure of interest forms are available to view online as sup-
porting information.
Contribution to authorship
ES conceived and designed the study, obtained funding for
the study, led the execution of the study including data
analyses and interpretation, and co-drafted the first draft of
the manuscript and critically reviewed final drafts. GV
assisted in designing the study, assisted in acquisition of
data, analysed the data and co-drafted the first draft of the
article. MP, JC, WW, JF, BR LJP, SK, SB and CM assisted
in designing the study and critically revised the first draft
of the article for important intellectual content. ZL assisted
in designing the study, analysed and interpreted the data,
and critically revised the first draft of the article for impor-
tant intellectual content. MR analysed and interpreted the
data and prepared the final drafts of the article. All authors
approve the content of this manuscript and agree to be
accountable for all aspects of the work.
Details of ethics approval
The lead ethics approval granted for this study in Australia
was by the NSW Population and Health Services Research
Ethics Committee (2009/03/144; HREC/09/CIPHS/21; 23
April 2009) and in New Zealand through the New Zealand
Multi-region Ethics Committee (MEC/09/73/EXP; 6
November 2019).
Funding
Funding for this project in Australia, and for overall coor-
dination of the study, was provided through National
Health and Medical Research Council (NHMRC Project
Grant #1024206). Funding in New Zealand was provided
through the Health Research Council of New Zealand (Pro-
ject Grant 12/698). GV gratefully acknowledges funding by
NHMRC Postgraduate Scholarship #11332944, University
of Technology Sydney Chancellor’s Research Scholarship;
and END RHD Centre of Research Excellence (NHMRC
1080401), Telethon Kids Institute, University of Western
Australia.

Acknowledgements
The authors thank the participating maternity units and
AMOSS data collectors in Australia and New Zealand who
participated in the study, as well as the AMOSS Associate
Investigators, Project team and Advisory Group. The
authors would like to acknowledge Faith Mahony who was
the Project Coordinator for the New Zealand study and
Kylie Tune, who was the Project Coordinator for the
Northern Territory.
Supporting Information
Additional supporting information may be found online in
the Supporting Information section at the end of the arti-
cle.
Figure S1. Surveillance and confirmed cases of rheumatic
heart disease in pregnancy, 2013/14.
Figure S2. Distribution of rheumatic heart disease in
pregnancy by usual residential location, with rates per
10 000 women giving birth, 2013/14. &
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