BMJ 2018;360:k478 doi: 10.1136/bmj.

k478 (Published 9 March 2018) Page 1 of 9

Practice

PRACTICE

CLINICAL UPDATES

Pregnancy in women with congenital heart disease

1 1 2
Matthew Cauldwell honorary clinical lecturer , Francois Dos Santos research fellow , Philip J Steer
1 2
emeritus professor of obstetrics , Lorna Swan consultant cardiologist , Michael Gatzoulis consultant
2 1
cardiologist , Mark R Johnson professor of obstetrics
1
Academic Department of Obstetrics and Gynaecology, Chelsea and Westminster Hospital, London, UK; 2Department of Adult Congenital Heart
Disease, Royal Brompton Hospital, London, UK

What are the common forms of congenital

What you need to know
heart disease?
• Women with congenital heart disease have increased risk of poor
pregnancy outcomes The European Registry on Heart Disease is the largest published
• Offer counselling and specialist multidisciplinary care before conception cohort of women with pregnancy complicated by heart disease.
• Refer women with congenital heart disease to a cardiologist during In 2012, of 1321 pregnant women with heart disease, 66% had
pregnancy for clinical assessment including cardiac function, and to
review cardiac medications congenital heart disease.4 Approximately one third of those
• Offer a planned hospital birth
women had simple shunt lesions, such as ventricular or atrial
• Expert consensus suggests vaginal delivery with regional anaesthesia
septal defects (fig 1), and the rest had multiple lesions, including
is preferred mitral and pulmonary valve abnormalities, aortic coarctation,
transposition of the great arteries, and Marfan’s syndrome. Very
high risk conditions, such as univentricular circulation (Fontan),
One in 125 people is born with congenital heart disease.1 For
cyanotic heart disease, or inherited cardiomyopathies accounted
women with the condition, pregnancy induced cardiovascular
for 2% to 5% of cases.4 In a smaller Canadian study of 405
stress can cause complications such as arrhythmia, heart failure,
pregnancies in women with congenital heart disease, more than
and thromboembolism.2 The UK Confidential Enquiry into
half had shunt lesions, repaired tetralogy of Fallot, or aortic
maternal deaths found that of 910 maternal deaths between 2009
coarctation.5
and 2014,3 205 (22.5%) were caused by heart disease, and a
minority from congenital heart disease. Clinicians in primary
and emergency care increasingly encounter women with
congenital heart disease who are planning pregnancy or who
are pregnant at presentation. These women might seek
information about the risks pregnancy poses to their own health,
and to the health of the fetus. In this article, we highlight aspects
of pre-conception, antenatal, and postpartum care for women
with congenital heart disease.3

Sources and selection criteria

We searched PubMed for relevant English language publications over the
past 10 years using the search terms “pregnancy” and “preconception,”
individually combined with “heart disease” and “cardiac disease.” All abstracts
were reviewed and we selected the most relevant papers for this article. We
have drawn recommendations from the European Society of Cardiology
Pregnancy Guideline and the American Heart Association Guideline.

Correspondence to M Cauldwell matthew.cauldwell@imperial.ac.uk

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BMJ 2018;360:k478 doi: 10.1136/bmj.k478 (Published 9 March 2018)
What are the risks?
For the woman (box 1)
Box 1: Risks for women with congenital heart disease in
pregnancy
Maternal cardiac risk
Common complications of congenital heart disease?
Arrhythmias
Heart failure
Thromboembolic events
These complications can lead to need for major surgery, disability,
premature death
Maternal obstetric risk
Higher incidence of
Miscarriage
Preterm pre-labour rupture of membranes
Postpartum haemorrhage
The overall risk of maternal death in women with congenital
heart disease is approximately 1%, which is 100 times higher
than the background risk for maternal mortality in the developed
world.2 Pregnancy causes a fall in systemic vascular resistance,
leading to an increase in cardiac output and blood volume.6
These changes appear to be similar in women with heart disease.7
However, data from a large multicentre study (2966 pregnancies,
of which 56% of women had congenital heart disease, 32%
valvular heart disease, and 7% cardiomyopathy) showed that
pregnant women with heart disease are more likely to encounter
episodes of arrhythmia (overall rate 2%). This typically includes
a non-sustained tachycardia or frequent ventricular ectopic beats
in the late second or third trimester.8 In a separate study from
the same cohort (1321 women), 13% of women experienced
heart failure, most commonly at the end of the second trimester
when plasma volume expansion reaches its peak, or in the
peripartum period (typically the period immediately before
delivery and up to 48 hours after).9 A retrospective UK study
(366 pregnancies) reported that postpartum haemorrhage
occurred in 25% of women with congenital heart disease.
Women with a Fontan circulation or taking anticoagulants were
at greatest risk.10 An American study of 50 women with aortic
coarctation showed that up to 30% developed pregnancy induced
hypertension or pre-eclampsia.11
Information on the effects of pregnancy, both immediate and
throughout life, on women with congenital heart disease is
limited as most studies are retrospective,12-14 and few include
long term follow-up. Most women can be reassured that
pregnancy is not associated with any apparent decline in cardiac
function, even years later.15 16
For the fetus (box 2)
Box 2: Risks to the fetus of congenital heart disease in the
mother
Higher incidence of
Fetal growth restriction
Preterm birth
Intracranial haemorrhage
Fetal and neonatal death
Congenital heart disease in infant:
Risk 3%-50% (background risk 0.8%)
Reported miscarriage rates vary according to lesion; being
highest (up to 50%) in women with cyanotic heart disease and
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Page 2 of 9
PRACTICE
women with a univentricular (Fontan) circulation.17-19 The risk
of congenital heart disease in the children of these women is
higher than background, with 3% to 5% having the condition.20 21
A UK single centre study (331 women) showed that preterm
labour and pre-labour rupture of membranes were more common
(12% and 14%, respectively) than in those without congenital
heart disease, while the incidence of babies being small for
gestational age (less than 10th centile) was 25% and the neonatal
mortality rate was 4%.22
How can counselling before conception
help?
The UK Confidential Enquiry into maternal deaths highlighted
the importance of preconception counselling.3 Few studies
examine the effect of counselling before conception on maternal
and perinatal morbidity and mortality in the context of maternal
heart disease.4 Nevertheless, European Society of Cardiology
and American Heart Association guidance emphasises that
counselling before conception should be readily available at the
transition from paediatric to adult cardiac care,23-25 ideally by
referral to a combined cardiology-obstetric clinic.
Availability of preconception counselling depends upon
geography. In the developed world, care is commonly
undertaken by a multidisciplinary team, including an obstetrician
and a cardiologist with access to a haematologist, geneticist,
and/or anaesthetist.26 In areas where a multidisciplinary team is
not established, the primary care provider might have to provide
guidance.
Women with heart disease who require IVF or medical therapies
to become pregnant are advised to receive counselling before
undergoing fertility treatment, as pregnancy might be
contraindicated in some cases (box 1), or management of the
fertility problem might need to be modified (such as single
embryo transfer to reduce the risk of multi-fetal pregnancy).24
In some conditions, such as pulmonary hypertension, women
might be advised to avoid pregnancy.23
Women might have concerns about potential risks to themselves
and their unborn baby,27 and these can be discussed during
preconception counselling. In women not contemplating
pregnancy, ensure effective discussion on contraception and
early pregnancy termination if necessary. Preconception
counselling assessment (box 3) typically includes a clinical
evaluation, imaging (notably an echocardiogram), risk
stratification, and a review of current medications30 31 to optimise
cardiac status and/or avoid fetal exposure to known teratogens,
such as angiotensin converting enzyme inhibitors. Data from
the European Registry on Heart Disease showed that two thirds
of women took cardiac medications at some point during their
pregnancy.32Table 1 and the infographic provide information
on the use of common cardiac medications in pregnancy.
Box 3: Key considerations for counselling before conception
for women with cardiovascular disease8 9 25 28 29
• Offer woman of childbearing age with cardiovascular disease counselling
and risk stratification before conception
• Counselling is best made available within the paediatric cardiology
transition service
• Offer the woman appropriate contraceptive advice
• In women contemplating pregnancy, change cardiovascular medications
to those which can be used in pregnancy, and emphasise the importance
of close monitoring
• In women not contemplating pregnancy, ensure effective discussion on
contraception and early pregnancy termination
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BMJ 2018;360:k478 doi: 10.1136/bmj.k478 (Published 9 March 2018) Page 3 of 9

PRACTICE

What assessment is needed for pregnant
women with congenital heart disease?
Urgently refer women who become pregnant and have not had
preconception counselling, especially if they have not had a
recent cardiac review. During assessment, women who have
not previously had counselling usually undergo
echocardiography and might require additional investigations,
such as cardiac magnetic resonance imaging, for confirmation
of the defect and to assess cardiovascular function. Such tests
are best organised by a specialist able to evaluate the underlying
condition fully and identify possible surgical interventions to
improve cardiac function before pregnancy.
Several studies have evaluated risk scoring systems for women
with congenital heart disease to better predict the likelihood of
a cardiac complication.11 12 48 Most scoring systems are limited,
however, as they do not consider both fetal and maternal
risks.48 49 The modified World Health Organization (mWHO)
classification is the most widely adopted and simplest risk
categorisation system (table 2). This can assist in counselling
women with congenital heart disease, and identify women who
need referral to specialist services.
How should new cardiac symptoms be
managed?
Although the physiological burden of normal pregnancy can
cause breathlessness and palpitations, any new onset requires
careful evaluation, often with referral to the cardiac and/or
obstetric team.
Occasionally, women without a history of heart disease might
present for the first time in pregnancy with a major cardiac
event. Box 4 describes some important scenarios in women with
known and previously occult congenital heart disease. Treatment
is influenced by the gestation of the pregnancy, and usually
requires referral to a specialist centre.29
Box 4: Management of cardiac emergencies in women with
cardiac disease during pregnancy/postpartum period
Heart failure
If bed rest and medical management are effective, the pregnancy can be
managed expectantly
In refractory cases, if the fetus is viable but showing signs of hypoxia,
emergency delivery is indicated
Arrhythmia
In women with arrhythmia before pregnancy, the recurrence rate might
be as high as 50%
Ventricular arrhythmia is uncommon and management is the same as in
non-pregnant women. If needed, defibrillation is safe
Table 1, infographic lists drugs that are considered safe to use in
pregnancy
Myocardial infarction
Management is the same as in the non-pregnant
Percutaneous coronary intervention exposes the fetus to radiation, but
the benefit to mother outweighs the risk. Aspirin and nitrates are safe to
use, but glycoprotein IIb/IIIa inhibitors should be avoided because there
are limited data on their safety. If coronary artery bypass grafting is
required and the fetus is viable, the baby should be delivered before the
surgery is carried out
Aortic dissection
Women with established aortic disease are at higher risk—for example,
Marfan syndrome, Loeyz-Dietz, Ehlers-Danlos, and bicuspid aortic valve.
In such women, chest pain requires prompt imaging with echocardiography
and computed tomography scanning
Computed tomography exposes the fetus to radiation, but it is more widely
available than magnetic resonance imaging
Type A dissection warrants surgical management, which carries a high
fetal mortality. If the fetus is viable, it should be delivered before surgery.
Type B dissections should be managed conservatively with blood pressure
control using β blockers and bed rest
Mechanical valve thrombus
Reported rates of valve thrombosis range from 3.7% to 9.4%. This
diagnosis should be considered if a patient presents with dyspnoea,
fatigue, and signs of heart failure with soft or absent valve sounds
Transthoracic/transoesophageal echocardiography should be performed
to assess the size of the thrombus
If the fetus is viable, it is preferable to deliver it before surgery. In a stable
patient, management should the same as in non-pregnant women

How to deliver antenatal care

Antenatal care for women with congenital heart disease is ideally
delivered by a multidisciplinary team including an obstetrician,
cardiologist, anaesthetist, and midwife. Care is usually based
in a tertiary hospital, and is particularly recommended for
women with risk categorised as mWHO 3 or 4 (table 2). In the
UK, there are 14 centres providing specialist care for women
with congenital heart disease. Whether a woman needs antenatal
care by a specialist team or can be safely cared for by a local
hospital team with cardiology input is best discussed with the
multidisciplinary team. The European Society of Cardiology
recommends that all women with congenital heart disease should
be reviewed by a cardiologist at least once before, and once
during pregnancy, and should have a planned hospital birth. An
integrated care record can help keep track of appointments and
facilitate communication between different healthcare providers.
Box 5 lists measurements and observations undertaken at each
antenatal visit.

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BMJ 2018;360:k478 doi: 10.1136/bmj.k478 (Published 9 March 2018)
Box 5: Check and record the following at each antenatal visit
Blood pressure and heart rate
Heart rhythm
Auscultation of heart sounds and lung bases
Maternal oxygen saturations
Proteinuria
Fetal growth
Ultrasound screening of the fetus at 11-14 weeks’ gestation is
advised to detect abnormal nuchal translucency, which has a
strong association with congenital heart disease in the fetus.50
Fetal echocardiography at 18-20 weeks’ gestation can detect
major structural and functional abnormalities. The European
Society of Cardiology recommends fetal growth monitoring
using serial ultrasound biometry, particularly if a woman is
taking βblockers, as maternal heart disease is associated with
an increased risk of fetal growth restriction.22
Management of labour
Most women with congenital heart disease can expect and are
typically offered as normal a birth as possible, including
spontaneous onset of labour. However, in general women are
advised to have their birth in hospital and to attend as soon as
in labour. A small number of women might require induction
of labour for obstetric reasons (for example, fetal growth
restriction) or cardiac reasons—for example needing to stop
anticoagulants such as low molecular weight heparin before
labour.51 The usual induction methods, such as vaginal or oral
administration of prostaglandins, are safe in women with
congenital heart disease.
For pain, most experts recommend regional anaesthesia, as it
blunts the cardiovascular response to pain,23 26 thereby reducing
strain on the heart. Maternal monitoring during labour is
individualised and commonly includes continuous
electrocardiographic monitoring, pulse oximetry, and
non-invasive blood pressure measurement. Expert consensus is
to offer antibiotic prophylaxis for infective endocarditis in
women with high risk lesions, such as metallic heart valves or
a history of endocarditis.25
No trials have evaluated the preferred mode of delivery in
women with heart disease. Retrospective data from European
Registry on Heart Disease suggest that vaginal delivery should
be encouraged, as caesarean section for cardiac indications alone
does not confer any benefit to the mother or baby.52 This view
is supported by the European Society of Cardiology and the
American Heart Association.23 25 Nevertheless, a planned
caesarean section might be preferable in some settings—for
example, because access to specialist cardiac and anaesthetic
services is limited, or because of the nature of the woman’s
condition. Despite the absence of robust clinical data, both the
American Heart Association and European Society of
Cardiology23 25 recommend assisted delivery, either by vacuum
extraction or forceps, in conditions such as Marfan syndrome
or significant valvular stenosis, to minimise the duration of the
active phase of the second stage of labour. However, this might
not apply where disease is mild.23 53
After delivery, active management (cord clamping and
administration of oxytocin) is recommended for all women with
heart disease, as this reduces blood loss by up to 40%.54 Oxytocin
can cause profound hypotension and tachycardia as a bolus55 56;
hence a slow infusion of 2 IU of oxytocin over 10 minutes is
preferred.57 Ergometrine is avoided as it causes hypertension
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PRACTICE
and constriction of the coronary arteries, which can cause
myocardial infarction.58 59
What are important considerations in the
postnatal period?
The puerperium is a high risk period as many haemodynamic
changes occur concurrently. Intensity of maternal monitoring
depends upon the underlying congenital lesion, predisposition
to arrhythmia, and the presence of symptoms of heart failure.
Admission to an obstetric high dependency unit for continuous
cardiac monitoring (telemetry) might be required, with
multidisciplinary input in women at risk of arrhythmia or heart
failure.
Some babies might benefit from referral to paediatric cardiology,
as minor lesions might not have been detected earlier.
Medications are reviewed and adjusted at this time and further
review in primary or secondary care can be discussed with the
patient. Women can be reassured that breast feeding is safe with
most cardiac medications (table 1, infographic), although advice
should be individualised and the wishes of the woman
considered. Contraception can be discussed with the woman
and her partner before discharge, to assess whether they would
like to avoid or space a future pregnancy.
Additional educational resources
The Somerville Foundationhttp://www.thesf.org.uk
Free information for patients with GUCH (grown up congenital heart), including
information about pregnancy. No registration required. Includes a “Young
People Area” for those aged 16-24. Opportunity to connect with others via
social networking or through a community blog
LactMed/TOXNEThttps://toxnet.nlm.nih.gov/newtoxnet/lactmed.htm
Free advice from the National Institute of Health on the safety of drug use
when breastfeeding. No registration required
UK Teratology Information Servicehttp://rdtc.nhs.uk/services/teratology
Free advice from the Regional Drug and Therapeutic Centre. Most content is
free but registration is required to access certain content (for employees of
National Health Service organisations that commission their services)
Royal College of Obstetricians and Gynaecologistshttps://www.rcog.org.
uk/globalassets/documents/guidelines/
goodpractice13cardiacdiseaseandpregnancy.pdf
Practice guidance on the management of cardiac disease in pregnancy,
including the “typical patient journey.” Free access and no registration required
European Society of Cardiologyhttps://www.escardio.org/static_file/Escardio/
Guidelines/publications/PREGN%20Guidelines-Pregnancy-FT.pdf
Guidelines on the management of cardiovascular diseases during pregnancy.
Free access and no registration required
Education into practice
• Describe your established referral pathway for women with cardiac
disease who are pregnant or who are planning to conceive? Does this
article offer you ideas on how to improve it?
• How many women of reproductive age in your practice have cardiac
disease? Does this article offer you ideas on what standards of
pre/ante/postnatal care you could audit?
• How would you explain the risks3 associated with pregnancy and
childbirth to a woman with a cardiac condition?
How patients were involved in the creation of this article
A woman with congenital heart disease who had recently given birth kindly
reviewed this paper. Based on her experience of care under a multidisciplinary
team during her pregnancy, she emphasised the importance of the team in
caring for women with heart disease or other cardiac complications in
pregnancy. She remarked that good communication was essential between
members of the multidisciplinary team, and when discussing care plans with
the patient.
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BMJ 2018;360:k478 doi: 10.1136/bmj.k478 (Published 9 March 2018)
Competing interests: We have read and understood The BMJ policy on declaration
of interests and declare that we have no competing interests.
Provenance and peer review: Encouraged; externally peer reviewed.
1 Mandalenakis Z, Rosengren A, Skoglund K, Lappas G, Eriksson P, Dellborg M.
Survivorship in children and young adults with congenital heart disease in Sweden. JAMA
Intern Med 2017;177:224-30. 10.1001/jamainternmed.2016.7765 27992621
2 Greutmann M, Silversides CK. The ROPAC registry: a multicentre collaboration on
pregnancy outcomes in women with heart disease. Eur Heart J 2013;34:634-5.
10.1093/eurheartj/ehs335 23048193
3 Saving lives improving mothers care—surveillance of maternal deaths in the UK 2012-14
and lessons learned to inform maternity care From the UK and Ireland confidential enquiries
into maternal deaths and morbidity 2009-14. 2016. https://www.npeu.ox.ac.uk/downloads/
files/mbrrace-uk/reports/MBRRACE-UK%20Maternal%20Report%202016%20-%20website.
pdflast accessed 4 January 2017.
4 Roos-Hesselink JW, Ruys TP, Stein JI, etal. ROPAC Investigators. Outcome of pregnancy
in patients with structural or ischaemic heart disease: results of a registry of the European
Society of Cardiology. Eur Heart J 2013;34:657-65. 10.1093/eurheartj/ehs270 22968232
5 Balint OH, Siu SC, Mason J, etal . Cardiac outcomes after pregnancy in women with
congenital heart disease. Heart 2010;96:1656-61. 10.1136/hrt.2010.202838 20937754
6 Mahendru AA, Everett TR, Wilkinson IB, Lees CC, McEniery CM. A longitudinal study of
maternal cardiovascular function from preconception to the postpartum period. J Hypertens
2014;32:849-56. 10.1097/HJH.0000000000000090 24406777
7 Kampman MA, Valente MA, van Melle JP, etal. ZAHARA II investigators. Cardiac adaption
during pregnancy in women with congenital heart disease and healthy women. Heart
2016;102:1302-8. 10.1136/heartjnl-2015-308946 27048772
8 Ertekin E, van Hagen IM, Salam AM, etal . Ventricular tachyarrhythmia during pregnancy
in women with heart disease: Data from the ROPAC, a registry from the European Society
of Cardiology. Int J Cardiol 2016;220:131-6. 10.1016/j.ijcard.2016.06.061 27376569
9 Ruys TP, Roos-Hesselink JW, Hall R, etal . Heart failure in pregnant women with cardiac
disease: data from the ROPAC. Heart 2014;100:231-8.
10.1136/heartjnl-2013-304888 24293523
10 Cauldwell M, Von Klemperer K, Uebing A, etal . Why is post-partum haemorrhage more
common in women with congenital heart disease?Int J Cardiol 2016;218:285-90.
10.1016/j.ijcard.2016.05.068 27240153
11 Beauchesne LM, Connolly HM, Ammash NM, Warnes CA. Coarctation of the aorta:
outcome of pregnancy. J Am Coll Cardiol 2001;38:1728-33.
10.1016/S0735-1097(01)01617-5 11704388
12 Uebing A, Arvanitis P, Li W, etal . Effect of pregnancy on clinical status and ventricular
function in women with heart disease. Int J Cardiol 2010;139:50-9.
10.1016/j.ijcard.2008.09.001 18835051
13 Bowater SE, Selman TJ, Hudsmith LE, Clift PF, Thompson PJ, Thorne SA. Long-term
outcome following pregnancy in women with a systemic right ventricle: is the deterioration
due to pregnancy or a consequence of time?Congenit Heart Dis 2013;8:302-7.
10.1111/chd.12001 22967110
14 Kampman MA, Balci A, Groen H, etal. ZAHARA II investigators. Cardiac function and
cardiac events 1-year postpartum in women with congenital heart disease. Am Heart J
2015;169:298-304. 10.1016/j.ahj.2014.11.010 25641540
15 Egidy Assenza G, Cassater D, Landzberg M, etal . The effects of pregnancy on right
ventricular remodeling in women with repaired tetralogy of Fallot. Int J Cardiol
2013;168:1847-52. 10.1016/j.ijcard.2012.12.071 23369674
16 Cauldwell M, Steer PJ, Bonner S, etal . Retrospective UK multicentre study of the
pregnancy outcomes of women with a Fontan repair. Heart
2017.10.1093/eurheartj/ehx502.969. 28954835
17 Cauldwell M, Von Klemperer K, Uebing A, etal . A cohort study of women with a Fontan
circulation undergoing preconception counselling. Heart 2016;102:534-40.
10.1136/heartjnl-2015-308788 26786817
18 Presbitero P, Somerville J, Stone S, Aruta E, Spiegelhalter D, Rabajoli F. Pregnancy in
cyanotic congenital heart disease. Outcome of mother and fetus. Circulation
1994;89:2673-6. 10.1161/01.CIR.89.6.2673 8205680
19 Cauldwell M, Steer PJ, Bonner S, etal . Retrospective UK multicentre study of the
pregnancy outcomes of women with a Fontan repair. Heart 2018;104:401-6.
10.1136/heartjnl-2017-311763 28954835
20 Gill HK, Splitt M, Sharland GK, Simpson JM. Patterns of recurrence of congenital heart
disease: an analysis of 6,640 consecutive pregnancies evaluated by detailed fetal
echocardiography. J Am Coll Cardiol 2003;42:923-9.
10.1016/S0735-1097(03)00853-2 12957444
21 Burn J, Brennan P, Little J, etal . Recurrence risks in offspring of adults with major heart
defects: results from first cohort of British collaborative study. Lancet 1998;351:311-6.
10.1016/S0140-6736(97)06486-6 9652610
22 Gelson E, Curry R, Gatzoulis MA, etal . Effect of maternal heart disease on fetal growth.
Obstet Gynecol 2011;117:886-91. 10.1097/AOG.0b013e31820cab69 21422861
23 Regitz-Zagrosek V, Blomstrom Lundqvist C, Borghi C, etal. European Society of
Gynecology (ESG)Association for European Paediatric Cardiology (AEPC)German Society
for Gender Medicine (DGesGM)ESC Committee for Practice Guidelines. ESC Guidelines
on the management of cardiovascular diseases during pregnancy: the Task Force on the
Management of Cardiovascular Diseases during Pregnancy of the European Society of
Cardiology (ESC). Eur Heart J 2011;32:3147-97. 10.1093/eurheartj/ehr218 21873418
24 Cauldwell M, Patel RR, Steer PJ, etal . Managing subfertility in patients with heart disease:
What are the choices?Am Heart J 2017;187:29-36. 10.1016/j.ahj.2017.02.007 28454805
25 Canobbio MM, Warnes CA, Aboulhosn J, etal. American Heart Association Council on
Cardiovascular and Stroke Nursing; Council on Clinical Cardiology; Council on
Cardiovascular Disease in the Young; Council on Functional Genomics and Translational
Biology; and Council on Quality of Care and Outcomes Research. Management of
pregnancy in patients with complex congenital heart disease: a scientific statement for
healthcare professionals from the American Heart Association. Circulation
2017;135:e50-87. 10.1161/CIR.0000000000000458 28082385
26 Gynaecologists RCoOa. Good Practice Guideline Number 13—Cardiac Disease and
Pregnancy. 2011. https://www.rcog.org.uk/en/guidelines-research-services/guidelines/
good-practice-13/
27 Montgomery (Appellant) v Lanarkshire Health Board (Respondent) (Scotland) UKSC.
2015. https://www.supremecourt.uk/decided-cases/docs/UKSC_2013_0136_Judgment.
pdf.
For personal use only: See rights and reprints http://www.bmj.com/permissions
Page 5 of 9
PRACTICE
28 Cauldwell M, Steer PJ, Swan L, etal . Pre-pregnancy counseling for women with heart
disease: A prospective study. Int J Cardiol 2017;240:374-8.
10.1016/j.ijcard.2017.03.092 28377190
29 van Hagen IM, Cornette J, Johnson MR, Roos-Hesselink JW. Managing cardiac
emergencies in pregnancy. Heart 2017;103:159-73.
10.1136/heartjnl-2015-308285 27628495
30 Pieper PG. Use of medication for cardiovascular disease during pregnancy. Nat Rev
Cardiol 2015;12:718-29. 10.1038/nrcardio.2015.172 26585398
31 James PR, Nelson-Piercy C. Management of hypertension before, during, and after
pregnancy. Heart 2004;90:1499-504. 10.1136/hrt.2004.035444 15547046
32 Ruys TP, Maggioni A, Johnson MR, etal . Cardiac medication during pregnancy, data
from the ROPAC. Int J Cardiol 2014;177:124-8. 10.1016/j.ijcard.2014.09.013 25499355
33 Yakoob MY, Bateman BT, Ho E, etal . The risk of congenital malformations associated
with exposure to β-blockers early in pregnancy: a meta-analysis. Hypertension
2013;62:375-81. 10.1161/HYPERTENSIONAHA.111.00833 23753416
34 Nakhai-Pour HR, Rey E, Bérard A. Antihypertensive medication use during pregnancy
and the risk of major congenital malformations or small-for-gestational-age newborns.
Birth Defects Res B Dev Reprod Toxicol 2010;89:147-54.20437474
35 Gladstone GR, Hordof A, Gersony WM. Propranolol administration during pregnancy:
effects on the fetus. J Pediatr 1975;86:962-4. 10.1016/S0022-3476(75)80236-8 1168703
36 Chow T, Galvin J, McGovern B. Antiarrhythmic drug therapy in pregnancy and lactation.
Am J Cardiol 1998;82(4A):58I-62I. 10.1016/S0002-9149(98)00473-1 9737655
37 Weber-Schoendorfer C, Hannemann D, Meister R, etal . The safety of calcium channel
blockers during pregnancy: a prospective, multicenter, observational study. Reprod Toxicol
2008;26:24-30. 10.1016/j.reprotox.2008.05.065 18585452
38 Wagner X, Jouglard J, Moulin M, Miller AM, Petitjean J, Pisapia A. Coadministration of
flecainide acetate and sotalol during pregnancy: lack of teratogenic effects, passage
across the placenta, and excretion in human breast milk. Am Heart J 1990;119:700-2.
10.1016/S0002-8703(05)80306-0 1689933
39 Bartalena L, Bogazzi F, Braverman LE, Martino E. Effects of amiodarone administration
during pregnancy on neonatal thyroid function and subsequent neurodevelopment. J
Endocrinol Invest 2001;24:116-30. 10.1007/BF03343825 11263469
40 Widerhorn J, Bhandari AK, Bughi S, Rahimtoola SH, Elkayam U. Fetal and neonatal
adverse effects profile of amiodarone treatment during pregnancy. Am Heart J
1991;122:1162-6. 10.1016/0002-8703(91)90489-5 1927869
41 Perkin RM, Levin DL, Clark R. Serum salicylate levels and right-to-left ductus shunts in
newborn infants with persistent pulmonary hypertension. J Pediatr 1980;96:721-6.
10.1016/S0022-3476(80)80753-0 7359281
42 Nørgård B, Puhó E, Czeizel AE, Skriver MV, Sørensen HT. Aspirin use during early
pregnancy and the risk of congenital abnormalities: a population-based case-control study.
Am J Obstet Gynecol 2005;192:922-3. 10.1016/j.ajog.2004.10.598 15746692
43 Vitale N, De Feo M, De Santo LS, Pollice A, Tedesco N, Cotrufo M. Dose-dependent fetal
complications of warfarin in pregnant women with mechanical heart valves. J Am Coll
Cardiol 1999;33:1637-41. 10.1016/S0735-1097(99)00044-3 10334435
44 Hassouna A, Allam H. Limited dose warfarin throughout pregnancy in patients with
mechanical heart valve prosthesis: a meta-analysis. Interact Cardiovasc Thorac Surg
2014;18:797-806. 10.1093/icvts/ivu009 24595247
45 Regitz-Zagrosek V, Blomstrom Lundqvist C, Borghi C, etal. European Society of
Gynecology (ESG)Association for European Paediatric Cardiology (AEPC)German Society
for Gender Medicine (DGesGM)ESC Committee for Practice Guidelines. ESC Guidelines
on the management of cardiovascular diseases during pregnancy: the Task Force on the
Management of Cardiovascular Diseases during Pregnancy of the European Society of
Cardiology (ESC). Eur Heart J 2011;32:3147-97. 10.1093/eurheartj/ehr218 21873418
46 Bullo M, Tschumi S, Bucher BS, Bianchetti MG, Simonetti GD. Pregnancy outcome
following exposure to angiotensin-converting enzyme inhibitors or angiotensin receptor
antagonists: a systematic review. Hypertension 2012;60:444-50.
10.1161/HYPERTENSIONAHA.112.196352 22753220
47 Hecker A, Hasan SH, Neumann F. Disturbances in sexual differentiation of rat foetuses
following spironolactone treatment. Acta Endocrinol (Copenh) 1980;95:540-5.7456979
48 Knight M, Callaghan WM, Berg C, etal . Trends in postpartum hemorrhage in high resource
countries: a review and recommendations from the International Postpartum Hemorrhage
Collaborative Group. BMC Pregnancy Childbirth 2009;9:55.
10.1186/1471-2393-9-55 19943928
49 van Hagen IM, Roos-Hesselink JW, Donvito V, etal . Incidence and predictors of obstetric
and fetal complications in women with structural heart disease. Heart 2017;103:1610-8.
10.1136/heartjnl-2016-310644 28377476
50 Atzei A, Gajewska K, Huggon IC, Allan L, Nicolaides KH. Relationship between nuchal
translucency thickness and prevalence of major cardiac defects in fetuses with normal
karyotype. Ultrasound Obstet Gynecol 2005;26:154-7. 10.1002/uog.1936 15977311
51 D’Souza R, Silversides CK, McLintock C. Optimal anticoagulation for pregnant women
with mechanical heart valves. Semin Thromb Hemost 2016;42:798-804.
10.1055/s-0036-1593418 27706532
52 Ruys TP, Roos-Hesselink JW, Pijuan-Domènech A, etal. ROPAC investigators. Is a
planned caesarean section in women with cardiac disease beneficial?Heart
2015;101:530-6. 10.1136/heartjnl-2014-306497 25539946
53 Cauldwell M, Cox M, Gatzoulis M, etal . The management of labour in women with cardiac
disease: need for more evidence?BJOG 2017;124:1307-9.
10.1111/1471-0528.14547. 28218452
54 Prendiville WJ, Elbourne D, McDonald S. Active versus expectant management in the
third stage of labour. Cochrane Database Syst Rev 2000;Cd000007.
55 Bhattacharya S, Ghosh S, Ray D, Mallik S, Laha A. Oxytocin administration during
cesarean delivery: Randomized controlled trial to compare intravenous bolus with
intravenous infusion regimen. J Anaesthesiol Clin Pharmacol 2013;29:32-5.
10.4103/0970-9185.105790 23493050
56 Dyer RA, Butwick AJ, Carvalho B. Oxytocin for labour and caesarean delivery: implications
for the anaesthesiologist. Curr Opin Anaesthesiol 2011;24:255-61.
10.1097/ACO.0b013e328345331c 21415725
57 Cauldwell M, Steer PJ, Swan L, Uebing A, Gatzoulis MA, Johnson MR. The management
of the third stage of labour in women with heart disease. Heart 2017;103:945-51.
10.1136/heartjnl-2016-310607 27993911
58 Svanström MC, Biber B, Hanes M, Johansson G, Näslund U, Bålfors EM. Signs of
myocardial ischaemia after injection of oxytocin: a randomized double-blind comparison
of oxytocin and methylergometrine during Caesarean section. Br J Anaesth
2008;100:683-9. 10.1093/bja/aen071 18385263
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BMJ 2018;360:k478 doi: 10.1136/bmj.k478 (Published 9 March 2018) Page 6 of 9

PRACTICE

59 Ramzy J, New G, Cheong A, Roberts L, Teh AW. Iatrogenic anterior myocardial infarction
secondary to ergometrine-induced coronary artery spasm during dilation and curettage
for an incomplete miscarriage. Int J Cardiol 2015;198:154-6.
10.1016/j.ijcard.2015.06.106 26163906
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Tables

Table 1| Drugs used to treat cardiovascular disease during pregnancy and breast feeding

Drugs Adverse effects in pregnancy Contraindications in pregnancy and Considered

postpartum safe while breast
feeding
Βeta blockers Commonly used. None. Can be used throughout pregnancy
No increased risk of major congenital abnormalities.
Organ specific malformations (cardiovascular defects, cleft palate/lip,
and neural tube defects), more prevalent in the offspring of women
treated with β blockers, although more likely explained by maternal
cardiac disease than by the lesion itself.33
Fetal growth restriction reported, although this might relate to
underlying maternal pathophysiology
Labetalol Fetal growth restriction (second and third trimester), neonatal None. Can be used throughout pregnancy and Yes
bradycardia and hypotension (when used near term) postpartum period 34
Bisoprolol Bradycardia and hypoglycaemia in the fetus None. Can be used throughout pregnancy and Yes
postpartum period 35 36
Atenolol Low birth weight, bradycardia, and hypoglycaemia in fetus (second Avoid during pregnancy. Can be used Yes
and third trimester) postpartum
Methyldopa Mild neonatal hypotension. Avoid postpartum because of the risk of Avoid postpartum No
postnatal depression
Digoxin Serum levels are unreliable. Safe in breast feeding None. Can be used throughout pregnancy Yes
Calcium-channel blockers Not associated with an increased incidence of congenital anomalies None. Can be used throughout pregnancy37
in humans
Nifedipine Potential synergism with magnesium sulphate can induce hypotension None. Can be used throughout pregnancy Yes
in mother and fetal hypoxia
Verapamil Well tolerated (limited evidence) None. Can be used throughout pregnancy Yes
Antiarrhythmic drugs
Adenosine No fetal adverse effects reported (limited human data) None Yes
Procainamide Unknown (limited evidence). Appears to be safe None Yes
38
Flecainide Unknown (limited evidence). Appears to be safe None Yes
Amiodarone Might be used in special circumstances. Risk of hypothyroidism Best to avoid in pregnancy unless the patient Yes
(goitre, bradycardia), fetal growth restriction, and preterm birth39 40 fails to respond to all other anti-arrhythmic
agents
Platelet aggregation inhibitors
Acetylsalicylic acid Low dose aspirin seems to be safe throughout pregnancy, though None. Can be used throughout pregnancy, Yes
usually stopped at 34-36 weeks. No teratogenic effects reported though usually stopped at 34-36 weeks.
(large datasets)41 42
Clopidogrel Safe during pregnancy in animal studies, but experience in humans Unclear. Current data are limited—no large Unknown
is limited and caution with this drug is advised. Available data suggest studies have shown harm
safe to continue but stop one week before anticipated delivery
Anticoagulants
Warfarin Can safely be used in the second and third trimesters. Risk of skeletal Contraindicated in the first trimester if dose Yes
defects, abnormalities of the central nervous system, and intracranial >5 mg/day.44
haemorrhage if used in the first trimester43 Most safely used in second and third
trimesters
Heparin (low molecular Low molecular weight heparin is safe throughout pregnancy and the None. Can be used throughout pregnancy Yes
weight) postnatal period
Diuretics and aldosterone Bumetanide, furosemide, and hydrochlorothiazide not teratogenic. Can be used in pregnancy and breast feeding
antagonists Risk of oligohydramnios and of electrolyte imbalance in the fetus45
Drugs contraindicated in pregnancy
Angiotensin converting Risk of neonatal renal failure and hypotension, renal tubular Avoid during pregnancy Yes46
enzyme inhibitors and dysgenesis, intrauterine growth restriction, decreased skull
angiotensin receptor ossification46
blockers
Spironolactone Possible risk of anomalies of the external genitalia (animal studies Avoid during pregnancy, although can be used Yes
only).47 If potassium sparing diuretics are needed, amiloride is postpartum
preferable

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Table 2| Modified World Health Organization classification of maternal cardiovascular riskAdapted from Thorne et al, 2006 and Regitz-Zagrosek
et al, 2011

Class Conditions Risk in pregnancy

I Uncomplicated, small, or mild: No detectable increased risk of maternal mortality
pulmonary stenosis, ventricular septal defect, patent ductus arteriosus, mitral valve prolapse with no and no/mild increase in morbidity
more than trivial mitral regurgitation
Successfully repaired simple lesions:
ostium secundum atrial septal defect, ventricular septal defect, patent ductus arteriosus, total anomalous
pulmonary venous drainage
Isolated ventricular extrasystoles and atrial ectopic beats
II Unoperated atrial septal defect, repaired tetralogy of Fallot, most arrhythmias Small increased risk of maternal mortality or
moderate increase in morbidity
II-III Mild left ventricular impairment, hypertrophic
Depending on individual cardiomyopathy, native or tissue valvular heart disease
not considered WHO IV, Marfan syndrome without
aortic dilatation, heart transplantation
III Mechanical valve, systemic right ventricle (eg, congenitally corrected transposition, simple transposition Substantially increased risk of maternal mortality
post Mustard or Senning repair), post Fontan operation, cyanotic heart disease, other complex congenital or severe morbidity. Expert counselling required.
heart disease If pregnancy is decided upon, intensive specialist
cardiac and obstetric monitoring needed throughout
pregnancy, childbirth, and the puerperium
IV Pulmonary arterial hypertension of any cause, severe systemic ventricular dysfunction, previous Extremely high risk of maternal mortality or severe
peripartum cardiomyopathy with any residual impairment of left ventricular function, severe left heart morbidity; pregnancy is contraindicated and
obstruction, Marfan syndrome with aorta dilated >0.40 mm termination should be discussed. If pregnancy
continues, care as for class III

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Figure

Fig 1 Common heart lesions

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