REVIEW
Cardiovascular disease
in pregnancy
Williams Roberts
Dawn Adamson
Abstract
Cardiac disease is the leading cause of maternal death in the UK with a
growing proportion of women being affected by ischaemic heart disease.
In this paper we describe the changes in cardiac physiology encountered
in pregnancy that may unmask pre-existing cardiac disease and the ef-
fects of individual cardiac diseases in this context. Key cardiac investiga-
tions and their safety, application and interpretation in pregnancy are
discussed. We outline the main cardiac pathology that can affect
women during this period and provide a summary of the available evi-
dence to guide management. This can be informed by the Toronto risk
score which highlights four key factors linked to maternal outcomes:
Prior cardiac events, baseline symptoms or cyanosis, left heart obstruc-
tion or left ventricular ejection fraction <40%. As cardiac disease
increases; the importance of pre-pregnancy diagnosis, effective pre-
pregnancy counselling and contraception are essential to safely manage
planned pregnancies in the presence of significant cardiac morbidity. As-
pects of obstetric care that require adjustment in the presence of cardiac
disease are highlighted.
Keywords heart disease; maternal mortality; obstetric cardiology;
pregnancy; pre-pregnancy counselling
Introduction
Cardiac disease is the leading cause of maternal death in the UK
and continues to rise. In the latest triennial report from CMACE,
53 women died from heart disease associated with, or aggra-
vated by, pregnancy. This gives a maternal mortality rate for
cardiac disease for 2006e08 of 2.31 per 100 000 maternities
(95% CI 1.77e3.03) (Figure 1). Acquired heart disease was the
cause of the majority of deaths, with ischaemic heart disease
becoming a common cause of death in pregnancy. Whilst
women with congenital heart disease are often complex, they
only accounted for 6% of the deaths.
In this article we illustrate the interaction between cardio-
vascular physiology and pregnancy and outline the assessment of
cardiac diseases and highlight key points in their management in
pregnancy and the puerperium.
Williams Roberts MBChB MRCP MD is a Cardiology Specialist Registrar at
University Hospital of Coventry and Warwickshire, UK. Conflicts of
interest: none declared.
Dawn Adamson BSc(Hons) MB BS MRCP PhD is a Consultant Cardiologist at
University Hospital of Coventry and Warwickshire, UK. Conflicts of
interest: none declared.
OBSTETRICS, GYNAECOLOGY AND REPRODUCTIVE MEDICINE 23:7
Cardiovascular physiology
During pregnancy, a woman’s body undergoes many adaptations
to meet the needs of a growing fetus. It is the extra demands on
cardiac output and the fluid shifts that occur during pregnancy
and childbirth that can expose limitations in cardiac function.
Increased circulating vasodilators increase the capacitance of
the venous and arterial bed and the development of the low
resistance uteroplacental circulation leads to a reduction in the
systemic vascular resistance (SVR); the SVR falls from week 5 to
its lowest between week 20e32 and subsequently rises to exceed
pre-pregnancy levels at term. As the SVR falls, the afterload that
the heart must pump against also falls.
The circulating vasodilators increase flow to the kidneys,
increasing GFR and thus decreasing serum creatinine levels.
Blood flow is also increased to the extremities, breast and nasal
mucous causing the warm hands and feet, nasal congestion and
breast engorgement often noted by pregnant women.
As the systemic resistance decreases, the circulating blood
volume increases by 50e70% during pregnancy; red cell mass
increases to a lesser extent, resulting in relative haemodilution
and thus the physiological anaemia of pregnancy. As the blood
volume increases, left ventricular end diastolic pressures and
atrial and right ventricular volumes increase. This volume in-
crease leads to atrial and ventricular stretch and combined with
increased catecholamines and adrenergic receptor sensitivity
may increase arrhythmogenicity. There is increased atrial and
ventricular ectopic activity in pregnancy which may also initiate
arrhythmias where the underlying substrate is present.
Overall, cardiac output must increase by 30e50% during
pregnancy which can unmask previously asymptomatic cardiac
disease. A combination of the physiological, hormonal and
emotional changes and the heightened visceral awareness
experienced in pregnancy can lead to the development of new
symptoms and may also lead a woman to seek advice on
symptoms that are within the normal range and may otherwise
have been ignored.
Normal cardiovascular examination
 Breathlessness and even orthopnoea are common in
pregnancy but dyspnoea leading to severe restriction of
activities is abnormal.
 Peripheral oedema and varicose veins are very common in
pregnancy and do not necessarily indicate heart failure. In
the presence of generalized oedema, if hypertension and
proteinuria are present, pre-eclampsia should be
considered.
 Heart rate is generally increased in pregnancy but rates
over 100 bpm require further evaluation for an underlying
cause.
 Bradycardia is extremely rare and a heart rate <50 bpm
requires ECG and further evaluation.
 Ventricular ectopics are common in pregnancy and can
lead to an irregular pulse which may be mistaken for atrial
fibrillation; an ECG can differentiate.
 The pulse often has a bounding character due to the
reduced SVR and increased cardiac output and the apex
often feels prominent and thrusting but not displaced. Fine
tremor and eye signs may be evident in thyroid disease.
195 Ó 2013 Elsevier Ltd. All rights reserved.
 REVIEW

under local anaesthesia. Although pregnancy is not a contrain-

Cardiac causes of maternal mortality 1952–2008 dication, it is unlikely that this will be required in pregnant
(per million maternities) women but as they have a battery life of about 2e3 years they
may have previously inserted and pose no problems in
60 pregnancy.
Acquired
50 Congenital Exercise test
Outside of pregnancy the use of the exercise ECG is declining due
40 to poor incremental diagnostic utility, it may, however, be useful
in the context of early pregnancy to establish functional capacity.
30 If hypotension develops the procedure must be stopped (Box 1).

Transthoracic echocardiography
20
This non-invasive test uses ultrasound to provide valuable in-
formation about cardiac structure and function. In particular
10
echocardiography is useful for identifying impairment of left
ventricular (LV) function, valvular heart disease and congenital
0
abnormalities. Measurements require adjustment for pregnancy,
19 4

19 4
19 7
19 0
19 3
19 6
19 9
19 2
19 5
19 8
19 1

19 7
19 0
19 3
19 6
20 9
20 2
20 5
08
5
5
6
6
6
6
7
7
7
8
8
8
9
9
9
9
0
0
in particular for chamber dimensions, and in quantifying veloc-
19

ities across valves. If a CT pulmonary angiogram (CTPA) is not

Figure 1
 When measuring blood pressure, be sure to use a correctly
sized cuff. Blood pressure decreases early in pregnancy but
from around week 23 rises to term.
 JVP may be raised late in pregnancy due to increased blood
volume and inferior vena cava compression.
 Loud heart sounds and the presence of a third heart sound
are normal in pregnancy. Ejection systolic murmurs are
heard in over 90% of women. Very loud murmurs or a
palpable thrill suggest underlying pathology. It is very
important to time murmurs with the carotid pulse as dia-
stolic murmurs are always pathological.
Cardiac investigations in pregnancy
ECG
The ECG often shows these differences in pregnancy:
 Sinus tachycardia
 15 left axis deviation due to diaphragmatic elevation
 T-wave changes e commonly t wave inversion in leads III
and aVF
 Non-specific ST changes e.g. depression
 Supra-ventricular and ventricular ectopic beats
 Small Q waves
available, echocardiography may aid in the diagnosis of acute
pulmonary embolism by identifying raised pulmonary artery
pressures and impairment and dilatation of the right ventricle.
However, CTPA is considerably more specific and should be
performed if clinically indicated with shielding to protect the
fetus.
Stress echocardiography
This procedure is performed with exercise or dobutamine infu-
sion to provide pharmacological stress whilst echocardiographic
pictures of LV contraction are obtained. Stress echocardiography
is used to assess for ischaemia and can be useful in some forms
of valvular heart disease. There is limited data on safety in
pregnancy.
Transoesophageal echocardiography
This requires placement of an ultrasound tube in the oesophagus
and stomach to allow more detailed examination of cardiac
structures, particularly in the setting of endocarditis where it has
a greater sensitivity than transthoracic echocardiography. The
main risk is of aspiration which is greater in pregnancy and thus
the assistance of an anaesthetist may be required to perform the
procedure under general anaesthesia. The procedure is usually
performed in the lateral position and this is important to reduce
inferior vena cava compression.

Holter monitors Contraindications to exercise testing in the pregnant

Ambulatory ECG recordings are used to capture the heart rhythm woman
at time of palpitations, pre-syncope and syncope. They are sim-
ple, non-invasive and safe to use in pregnancy. It is important, C Labour (pre-term or at term)
however, to correlate symptoms with any abnormalities found C Incompetent cervix with bulging membranes and not in labour
and not to treat asymptomatic arrhythmias unless absolutely C Recent vaginal bleeding with placenta praevia or suspected
necessary. placental abruption
C Pre-eclampsia
Implantable loop recorders C Symphyseal-pubic dysfunction which limits movement
Infrequent symptoms of palpitations or syncope can be difficult
to capture by non-invasive means and this allows the implant of
a small ECG recording device in a simple procedure performed Box 1

OBSTETRICS, GYNAECOLOGY AND REPRODUCTIVE MEDICINE 23:7 196 Ó 2013 Elsevier Ltd. All rights reserved.

Tilt testing
Used in the diagnosis of syncope, it is usually possible to delay
this investigation until after pregnancy as women are required to
lie flat on their backs which impedes inferior vena cava flow later
in pregnancy. There is also concern that the normal physiology of
pregnancy may lead to a false positive result.
Ionizing radiation in pregnancy
The general principle to ordering radiological tests in pregnancy is
that if a test is truly required to inform management it should be
performed. Where equivalent information can be obtained from
other modalities without ionizing radiation this should be
considered and precautions taken to reduce fetal dose with lead
shielding where possible. The radiation dose required to harm the
fetus or cause miscarriage is far in excess of doses used in medical
diagnostic procedures.
Chest X-ray
The normal CXR in pregnancy can have:
 Prominent vascular markings
 Horizontal position of the heart
 Flattened left heart border
 Raised diaphragm due to gravid uterus
 Small pleural effusion seen postpartum
In the assessment of cardiac disease the presence of pulmonary
oedema, a widened mediastinum occasionally seen in aortic
dissection and rib notching in coarctation are important to consider.
CT pulmonary angiogram (CTPA)
The CTPA is the gold standard test for suspected acute pulmonary
emboli; the risk to fetus is small, outweighed by the danger of
missed pulmonary emboli and can be minimized by lead shielding.
V/Q scan
Signs of mismatch in perfusion compared with ventilation in the
lungs are used in diagnosis of pulmonary emboli. The test uses
radioisotopes and the risks to fetus are negligible but can be reduced
by omitting the ventilation part of the scan if perfusion is normal.
Myocardial perfusion scan
This nuclear medicine scan is used to evaluate for signs of
myocardial ischaemia by examining myocardial blood supply at
rest and whilst under the effects of exercise or pharmacologically
induced stress. It is likely that vascular breast tissue will impair
analysis in pregnant women and as alternatives such as stress
echo are available is rarely used.
Cardiac magnetic resonance imaging
This provides information on cardiac anatomy and function but it
is generally only used if other investigations such as echocardi-
ography cannot provide the relevant information. MRI does not
use ionizing radiation but the safety of MRI in the early stages of
pregnancy is not yet determined although it has been used in the
second and third trimesters to assess fetal anatomy. It should be
used in preference to CT if it can provide equivalent information.
OBSTETRICS, GYNAECOLOGY AND REPRODUCTIVE MEDICINE 23:7
REVIEW
Electrophysiological studies and radio-frequency ablation
These invasive procedures to diagnose and treat arrhythmias are
rarely required in pregnancy and, due to the high radiation doses
used, should be postponed till after delivery with the exception of
severe refractory arrhythmias. Radiation can be minimized using
non-fluoroscopic catheter location systems as well as lead shielding.
Cardiac catheterization
This invasive procedure allows visualization of coronary arteries
and measurement of intracardiac pressures. They give high radia-
tion exposure to the fetus and should be used with caution but, this
is not contra-indicated if clinically required and is the treatment of
choice for ST segment elevation myocardial infarction ST segment
elevation myocardial infarction (STEMI) in pregnancy. Lead
shielding should be used to reduce fetal dose.
Pre-pregnancy counselling and contraception
All women with existing cardiac disorders need to be made
aware of the risks of pregnancy to their health with regard to
their individual diagnosis and the risks of passing on genetic
abnormalities to their child in advance so they can make
informed decisions and pregnancies can be planned and moni-
tored in an optimal manner to reduce maternal mortality.
The Toronto group risk score, validated in a large prospective
observational study identified four factors that were important
for maternal outcomes:
 A prior event (heart failure, transient ischaemic attack or
cerebrovascular accident) or arrhythmia;
 Baseline NYHA* class >II or cyanosis;
 Left heart obstruction (with aortic valve area [AVA] <1.5
cm2, mitral valve area [MVA] <2 cm2 or left ventricular
outflow tract [LVOT] gradient more than 30 mmHg);
 Left ventricular ejection fraction (LVEF) <40%.
*NYHA represents the New York Heart Association functional
class with NYHA Class II corresponding to mild symptoms (mild
shortness of breath and/or angina) and slight limitation during
ordinary activity.
For the presence of each factor a score of 1 was given. A total
score of 0, 1 and greater than 1 was predictive of a 5%, 27% and
75% event rate respectively (see Figure 2). This was true of
either primary (acute pulmonary oedema, symptomatic
arrhythmia, stroke or cardiac arrest) or secondary cardiac events
(which included significant deterioration in NYHA class or need
for future cardiac intervention).
The study was also able to identify five factors that predicted
neonatal outcomes; NYHA class >II or cyanosis, anticoagulation
during pregnancy, smoking, multiple gestation, and left heart
obstruction. The rate of fetal death doubled to 4% if at least one
of these factors was present.
Risk assessment has been discussed, but one of the benefits of
preconceptual counselling is to ensure all possible measures to
reduce risk have been taken. Please discuss.
Effective contraception is essential for patients with pre-
existing cardiac disease who are not seeking to conceive; this
needs to be discussed with all such patients in advance. The
combined oral contraceptive pill is contraindicated in most car-
diac diseases due to the effects of oestrogen. There are however
197 Ó 2013 Elsevier Ltd. All rights reserved.

Frequency of maternal primary cardiac events, as
predicted by the risk index and observed in the
derivation and validation groups, expressed as a
function of the number of cardiac predictors or points
80
Predicted
Cardiac event rate (% pregnancies)
70 Derivation
Validation
60
50
40
30
20
10
0
0 1
Number of predictors
Figure 2
progesterone only based products that are as effective as sterili-
zation such as Implenon implant and Mirena intrauterine device
plus a newer more effective progesterone only pill, Cerazette.
Ischaemic heart disease
Outside of pregnancy, women are recognized to present later
than men, and with atypical features of ischaemic heart disease,
making the diagnosis harder. Risk factors for ischaemic heart
disease are the same as outside of pregnancy; obesity, smoking,
hypertension, diabetes, family history and age. As the age of
pregnant women rises in the UK and the prevalence of risk fac-
tors such as diabetes rises in the population, ischaemic heart
disease in pregnancy is becoming an increasing problem.
Whilst there are some changes to the ECG in pregnancy dis-
cussed above, the recognition of significant changes such as ST
elevation and depression and T wave changes remains the same.
In patients with chest pain and ST elevation on ECG, immediate
action with involvement of cardiology specialists is required to
identify and treat ST elevation myocardial infarction due to an
occluded coronary artery.
ST elevation MI (STEMI) is now widely treated with primary
angioplasty whereby occluded arteries are opened mechanically,
and this is treatment of choice in pregnancy as it not only allows
treatment of STEMI, it can prevent inappropriate treatment when
the absence of occluded coronary arteries is identified. Primary
angioplasty is not universally available and thrombolysis may
still be given in some areas. Standard measures for nursing sick
pregnant women such as nursing in left lateral position should be
initiated. 300 mg Aspirin can be administered safely, oxygen,
opiate analgesia and anti emetics should be given as required.
Clopidogrel, a second antiplatelet agent that has been shown to
reduce mortality and reinfarction in STEMI has been used with
increasing frequency and appears to be safe.
OBSTETRICS, GYNAECOLOGY AND REPRODUCTIVE MEDICINE 23:7
REVIEW
In the absence of ST elevation on ECG, the patient may be
suffering from an acute coronary syndrome. Measurement of
cardiac troponins has become pivotal in the diagnosis of acute
coronary syndromes, but the history is paramount and where
suspected, patients should be reviewed by a cardiologist. Whilst
pre-eclampsia can cause minor changes, troponin levels other-
wise remain normal in pregnancy, therefore a raised troponin
should be taken seriously.
Whilst STEMI requires immediate treatment to restore coronary
flow, acute coronary syndromes require treatment depending on
risk stratification, with subsequent medical, percutaneous or sur-
gical treatment. Cardiac catheterization should be performed when
clinically indicated as risk to the fetus is low.
Aortic disease
Loey-Dietz, Marfan’s and Ehlers Danlos (type IV) syndromes are
inherited genetic disorders that have increased risk of aortic
dissection in pregnancy. These patients require regular moni-
>1 toring during pregnancy to identify further increase in aortic
diameter. Beta blockers, avoidance of isometric exercise and
maintenance of normotension are important in reducing risk but
surgical correction should be considered in Marfan’s patients
with aorta >40 mm diameter prior to pregnancy, or increasing
diameter during pregnancy. There are other non-cardiac com-
plications related to these diseases in pregnancy which must also
be considered as obstetric complications are also common.
Caesarian section, ideally under epidural is recommended.
Aortic dissection can occur outside of these diseases and thus
women presenting with chest pain sufficient to require opiate
analgesia require senior review and consideration of aortic dissec-
tion, pulmonary embolism and myocardial infarction. Where fa-
cilities and expertise are available, MRI should be used to confirm
diagnosis of dissection but if this is not available, contrast enhanced
CT should be performed; the risk to the fetus from the ionizing ra-
diation is minimal, particularly if lead shielding is used to reduce
exposure. Dissections involving the aortic root require urgent sur-
gical correction whereas those that only involve the descending
aorta generally require treatment with medication such as beta
blockers and calcium channel blockers to maintain strict blood
pressure control with a mean arterial pressure (MAP) of <75 mmHg.
Where possible, the fetus should be delivered as soon as possible.
Cardiomyopathy
Dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy
and restrictive cardiomyopathy usually pre-date pregnancy,
although these conditions may be first diagnosed in pregnancy
due to the increased cardiac output requirements. Peripartum
cardiomyopathy develops during or shortly after pregnancy.
Cardiomyopathy should be suspected in patients complaining
of limiting breathlessness, particularly if other signs or symptoms
of heart failure such as orthopnoea, Paroxysmal Nocturnal
Dyspnoea (PND) and significant peripheral oedema are present.
The diagnosis can usually be accurately made by transthoracic
echocardiography. B Type natriuretic peptide (BNP) is released
from the heart in response to stretch and can be measured from a
peripheral venous blood sample in the diagnosis of heart failure.
Although BNP levels may rise in pre-eclampsia, a BNP level
198 Ó 2013 Elsevier Ltd. All rights reserved.

100 pg/ml had a 100% negative predictive value for cardiac
events in a group of patients with structural heart disease.
DCM can result from infectious, autoimmune or genetic pre-
disposition although in a large number of cases an identifiable
cause is not found. In women with severe LV impairment,
pregnancy is not advised and termination should be considered
due to the high risk or maternal mortality. Those with particu-
larly poor prognosis are women with left ventricular ejection
fraction <30% or marked limitation in activity due to symptoms.
Peripartum Cardiomyopathy (PPCM) is an idiopathic cardio-
myopathy that presents with heart failure secondary to left
ventricular systolic dysfunction towards the end of pregnancy or
in the months after delivery, in the absence of any other cause of
heart failure. PPCM is a diagnosis of exclusion. Although the left
ventricle may not be dilated, the ejection fraction is nearly al-
ways reduced below 45%. It is rare with an incidence of 1:5000
to 1:10 000. Risk factors for the development of peripartum
cardiomyopathy are: increased maternal age, afrocaribbean race,
multiparity, multiple pregnancy, hypertension (pre-existing,
pregnancy induced or pre-eclampsia).
DCM and PPCM are managed in similar ways with the
involvement of cardiologist, obstetrician and obstetric and cardiac
anaesthetists. Cardiac decompensation in an otherwise stable pa-
tient may occur with fluid overload which can be caused by iatro-
genic fluid infusions, syntocinon infusions, b-agonists for tocolysis
or steroids for fetal lung maturity. Pulmonary oedema should be
treated promptly with oxygen and IV diuretics e.g. furosemide.
Subsequently afterload reducing agents such as: nifedipine,
hydralazine or isosorbide mononitrate used antenatally whilst the
teratogenic ACE inhibitors are preferably only used postnatally
(though in severe LV impairment they may be used and the risk to
fetus accepted). Arrhythmias should be managed as described later.
Patients who fail to respond to therapy may require intubation
and ventilation and transfer to specialist centres where intra aortic
balloon pumps or left ventricular assist devices are available.
Delivery should be expedited if PPCM is diagnosed antenatally.
This may result in iatrogenic preterm delivery. Early evidence
suggests we may see a role for Bromocriptine in helping to improve
Subsequent pregnancy after PPCM
Persistent LV dysfunction or dilatation 6 months after initial
diagnosis of PPCM: pregnancy not advised due to 25% risk of
maternal death, 50% risk of worsening cardiac failure.
If LV function returns to normal the risk is lower but there remains
a 26% risk of worsening cardiac failure.
Pre-pregnancy counselling is imperative. In an unplanned preg-
nancy consider termination of pregnancy if there is persistent LV
dysfunction. If, after pre-pregnancy counselling, the woman de-
cides to go ahead with a subsequent pregnancy, consider careful
withdrawal of teratogenic medications i.e. ACE inhibitors prior to
conception, baseline echocardiogram and repeat echocardiogram
at regular intervals and full hospital combined obstetric and car-
diology care. If there is clinical or echocardiographic deterioration
in pregnancy, serious consideration should be given to discon-
tinuation of the pregnancy either as a termination of pregnancy or
preterm delivery.1,2
OBSTETRICS, GYNAECOLOGY AND REPRODUCTIVE MEDICINE 23:7
REVIEW
recovery of LV function with PPCM when given postpartum.
Insertion of an implantable cardio-defibrillator should be consid-
ered in women with severe LV impairment prior to discharge.
Valvular heart disease
As with all cardiac disease, patients with valve disease should be
assessed prior to conception in order to consider whether treat-
ment such as valve repair/replacement or valvuloplasty should
be performed pre pregnancy. Stenotic valvular lesions are less
well tolerated in pregnancy than regurgitant lesions with severe
aortic or mitral stenosis having mortality greater than 10%.
Mitral stenosis:
 Mitral stenosis is predominantly due to prior rheumatic
fever and can present for the first time in pregnancy.
 Patients usually present with breathlessness, fatigue or
acute pulmonary oedema.
 Due to dilation of the left atrium, they are often in atrial
fibrillation. They have a tapping apex beat, a loud first
heart sound and a mid diastolic murmur.
 Poor prognostic signs are presence of symptoms pre-
pregnancy and severe stenosis (valve area <1 cm2).
 Standard heart failure management with diuretics. To in-
crease the time for the ventricle to fill in diastole through
the stenosed valve, beta blockers or digoxin are used to
reduce heart rate; this is vital in patients with atrial
fibrillation (AF). Patients in AF require therapeutic heparin
(or warfarin after 12 weeks) and prophylactic heparin
should be considered if the left atrium is dilated.
 Balloon mitral valvuloplasty is a percutaneous procedure
performed via the femoral vein to stretch the valve to
reduce the degree of stenosis, but is not appropriate in
heavily calcified valves. Surgery is not usually required
during pregnancy unless there is severe decompensation.
Aortic stenosis:
 In childbearing women this is most commonly due to a con-
genitally bicuspid valve which can be associated with aortic
coarctation, the presence of which should also be established.
 This is often asymptomatic but can present with dyspnoea,
chest pain and pre-syncope or syncope.
 Patients can have an ejection systolic murmur which ra-
diates to the carotids. The murmur lengthens with severity
and obscures the second heart sound. The carotid pulse
has a slow rising character in severe aortic stenosis.
 ECG will often demonstrate left ventricular hypertrophy
(LVH), but transthoracic echocardiography can confirm the
diagnosis, valve morphology, left ventricular (LV) function
and presence of coarctation or Patent Ductus Arteriosus
(PDA) which can be associated. MRI should be performed
where the aorta is dilated or coarctation is suspected.
 Cardiologist supervised exercise tolerance test (ETT) is
sometimes performed, particularly if early in pregnancy to
predict ability to cope with haemodynamic changes in
pregnancy. Poor prognosis is indicated by ST changes or
failure to increase blood pressure when exercising.
 Severe lesions require treatment; either with aortic valve
replacement or occasionally as a temporary measure with
aortic balloon valvuloplasty though this is much less
199 Ó 2013 Elsevier Ltd. All rights reserved.
 REVIEW
performed than in mitral stenosis. Admission for bedrest
may be required throughout pregnancy and close moni-
toring for symptoms or change in gradient or LV function
with serial echocardiography is also required.
 Beta blockers are beneficial, particularly in hypertension and
if aorta is dilated. Vasodilators (i.e. nitrates) should be
avoided.
Aortic and mitral regurgitation
 These can present with breathlessness or heart failure
symptoms.
 Aortic regurgitation (AR): wide pulse pressure, collapsing
pulse and diastolic murmur.
 Mitral regurgitation (MR): pansystolic murmur radiating to
apex.
 Usually well tolerated, but patients who are symptomatic
pre-pregnancy should be considered for valve replace-
ment. If severe AR or MR identified in pregnancy, close
monitoring for development of heart failure is necessary;
heart failure should be treated with diuretics and vasodi-
lators such as hydralazine and nitrates.
Prosthetic heart valves
 The most important issue in patients who have previously
undergone valve replacement with mechanical prostheses
is that of anticoagulation which is not usually required
post bioprosthetic valve replacement. The highest risk of
valve thrombosis is in the mitral valve, particularly with
single tilting disc or ball cage valves, though these should
be decreasingly prevalent in this relatively young group.
 The vast majority of these patients will be on warfarin
which is teratogenic so pre-pregnancy counselling is vital.
 There are several approaches to anticoagulation in this group
and each case requires careful consideration of the risk of
embryopathy, valve thrombosis and critically, patient
compliance. Teratogenicity from warfarin mostly occurs be-
tween weeks 6 and 12 and is more common in women with
doses greater than 5 mg od. Warfarin does however give the
best protection from valve thrombosis. Low Molecular
Weight Heparin (LMWH) with dose adjustments with anti
factor Xa levels is probably the best alternative to warfarin,
avoiding the need for continuous infusion and dose adjust-
ments in unfractionated heparin. In patients with high risk
mitral valve replacements on warfarin doses <5 mg od,
warfarin can be used for the majority of pregnancy e avoiding
the times with greatest fetal risk; LMWH should be used in
early pregnancy and near term, with anti factor Xa monitoring
to ensure adequate dosing. A woman with a bitilting aortic
valve replacement who requires high warfarin doses could be
managed with LMWH with dose monitoring throughout
pregnancy.
Arrhythmias
The vast majority of palpitations identified in pregnancy are not
life threatening, and generally conservative management is un-
dertaken. A careful history-taking to identify relatives who have
died suddenly or unexpectedly, and assessment to identify un-
derlying cardiac disease, are the key to identifying the small
OBSTETRICS, GYNAECOLOGY AND REPRODUCTIVE MEDICINE 23:7
number of women with malignant arrhythmias. The resting ECG
can sometimes reveal pre-excitation indicating the substrate for a
re-entry tachycardia, prolonged QT with risk of Ventricular
Tachycardia or rarely Brugada syndrome (a rare genetic syn-
drome that can lead to cardiac arrest).
Identifying the actual rhythm responsible for the symptoms
requires an ECG during the episode. If symptoms are short-lived,
this usually requires prolonged Holter recordings and rarely
reveal device implantation.
For women presenting compromised with hypotension or heart
failure, any tachycardia either broad or narrow should be treated
with synchronized DC cardioversion as per Advanced Life Support
(ALS) protocols. Direct current cardioversion (DCCV) in preg-
nancy should be performed with general anaesthesia and intuba-
tion to avoid aspiration; patients should be nursed on the left
lateral to avoid vena caval compression, with the addition of close
fetal monitoring (if necessary the priority should be to stabilize the
mother rather than wait for fetal monitoring to be available). In
patients with regular narrow complex tachycardias who do not
respond to vagal manoeuvres, an attempt to terminate a supra-
ventricular tachycardia (SVT) with increasing boluses of adeno-
sine is a safe option but should be performed with a defibrillator
attached, both to record the response to adenosine and also in the
rare event that treatment accelerates the arrhythmia.
Bradycardia in this group is very rare and may represent
congenital complete heart block or underlying cardiomyo-pathy.
The decision whether to treat paroxysmal arrhythmias in
pregnancy requires consideration of the frequency, length and
severity of symptoms balanced with the potential drug side ef-
fects. The data available for making such decisions is limited but
the following drugs are probably the better options.
 SVT e verapamil e but must be given slowly if given
intravenously.
 Wolf Parkinson White e beta blockers (bisoprolol
preferred as less Intrauterine growth retardation (IUGR)
recorded).
 AF e sotalol but early cardioversion to avoid anti-
coagulation best. Procainamide and flecainide also have
some utility.
 VT e DCCV and lignocaine or if required amiodarone for
short periods in emergencies only.
 Bradycardia e atropine in resuscitation
Implantable cardioverter-defibrillators (ICD)
Generally these have been inserted prior to pregnancy, but may be
used in patients with malignant arrhythmias. Women with pre-
vious ICDs can have a good outcome in pregnancy, although de-
vice complications are not uncommon. It is important not to forget
management of the underlying pathology that lead to the implant.
After each shock, the device must be interrogated, the patient
reviewed by a cardiologist and the fetus monitored for signs of
distress; these are more likely related to transient hypotension
from the arrhythmia than the effects of the electrical shock.
Pulmonary hypertension
Pulmonary hypertension has an extremely high mortality in preg-
nancy and if pregnancy is to be continued with must be managed in
a specialist unit with an extensive multi-disciplinary team.
200 Ó 2013 Elsevier Ltd. All rights reserved.
 REVIEW
Congenital heart disease
Although the cohort of patients with congenital heart disease
surviving to childbearing age is increasing, the mortality has
been falling in recent years. The worst prognosis is in those with
cyanotic heart disease, Fontan circulation (a surgically created
circulatory system to palliate congenital heart disease, relying on
one functioning ventricular chamber that feeds the systemic
circulation) or palliative shunts. Again, the emphasis is on pre-
pregnancy counselling and reliable contraception. Pregnancy
must be managed in a specialist tertiary unit with appropriate
care from cardiologists, anaesthetists and obstetricians.
Obstetric care in cardiac patients at delivery
In the majority of cardiac patients a normal vaginal delivery is
appropriate, with a Caesarean section reserved for obstetric in-
dications. Outcome is improved if a delivery plan is drawn up using
a, multi-disciplinary team prior to delivery. Specific conditions
such as patients with dilated aortas should be delivered by planned
Caesarean section to avoid large intrathoracic pressure changes.
High-risk patients should be considered for delivery in the
tertiary unit with the assistance or advice of cardiac anaesthe-
tists, with techniques to minimize changes in afterload and
monitoring of preload i.e. central venous pressure monitoring for
higher risk cases.
In patients with Marfan syndrome, dural ectasia is often
present which, unless mild, presents an increased risk of a spinal
CSF leak in the case of accidental dural puncture during epidural;
epidural anaesthesia is thus generally not recommended.
Oxytocin can still be given in the majority of cardiac disease,
as the risk of haemorrhage to these patients is high. It can lead to
increased autotransfusion which may be poorly tolerated in some
patients, particularly those with stenotic lesions or pulmonary
hypertension and cause rapid fall in afterload and thus tachy-
cardia and demands on cardiac output. If used, bolus doses
should be replaced by slow infusion to minimize these effects.
Ergometrine, however, should generally be avoided. The pros-
taglandin carboprost, used in postpartum haemorrhage due to
uterine atony, causes bronchospasm, hypertension and myocar-
dial ischaemia; carboprost should thus be reserved for patients
with life threatening haemorrhage.
Summary
As ischaemic heart disease increases in pregnancy, greater
attention needs to be paid to risk factors in this group. Pre-
pregnancy counselling and effective contraceptive advice re-
mains of paramount importance in patients with known cardiac
disease to avoid unplanned pregnancies. High-risk pregnancies
should be managed in specialist centres by an experienced multi-
disciplinary team. A
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Practice points
C Heart disease is the leading cause of maternal death and
continues to rise.
C Women with pre-existing cardiac disease should receive pre-
pregnancy counselling to reduce morbidity and mortality.
C If an investigation involving ionizing radiation is required to
manage a pregnant woman it should not be with held for fear
of fetal exposure.
C Clinicians should have a low threshold for investigating any
women complaining of chest pain and in particular if requiring
significant analgesia.
C The Toronto risk score is a useful tool to assess risk of women
with heart disease in pregnancy.
201 Ó 2013 Elsevier Ltd. All rights reserved.