Thứ Bảy, 8 tháng 3, 2014

Tài liệu ESC Guidelines on the management of cardiovascular diseases during pregnancy pdf

programmes are needed because it has been shown that the
outcome of disease may be favourably influenced by the thorough
application of clinical recommendations.
Surveys and registries are needed to verify that real-life daily
practice is in keeping with what is recommended in the guidelines,
thus completing the loop between clinical research, writing of
guidelines, and implementing them into clinical practice.
The guidelines do not, however, override the individual respon-
sibility of health professionals to make appropriate decisions in the
circumstances of the individual patients, in consultation with that
patient, and, where appropriate and necessary, the patient’s guar-
dian or carer. It is also the health professional’s responsibility to
verify the rules and regulations applicable to drugs and devices at
the time of prescription.
2. General considerations
2.1 Introduction
At present, 0.2–4% of all pregnancies in western industrialized
countries are complicated by cardiovascular diseases (CVD),
1
and the number of the patients who develop cardiac problems
during pregnancy is increasing. Nevertheless, the number of such
patients presenting to the individual physician is small. However,
knowledge of the risks associated with CVD during pregnancy
and their management are of pivotal importance for advising
patients before pregnancy. Therefore, guidelines on disease man-
agement in pregnancy are of great relevance. Such guidelines
have to give special consideration to the fact that all measures
concern not only the mother, but the fetus as well. Therefore,
the optimum treatment of both must be targeted. A therapy
favourable for the mother can be associated with an impairment
of the child, and in extreme cases treatment measures which
protect the survival of the mother can cause the death of the
fetus. On the other hand, therapies to protect the child may
lead to a suboptimal outcome for the mother. Because prospective
or randomized studies are lacking, with a few exceptions, rec-
ommendations in this guideline mostly correspond to the evidence
level C.
Some general conclusions have arisen from these guidelines:
counselling and management of women of childbearing age with
suspected cardiac disease should start before pregnancy occurs;
they should be managed by interdisciplinary teams; high risk
patients should be treated in specialized centres; and diagnostic
procedures and interventions should be performed by specialists
with great expertise in the individual techniques and experience
in treating pregnant patients. Registries and prospective studies
are urgently needed to improve the state of knowledge.
2.2 Methods
The Guidelines are based on a systematic search of the literature
of the last 20 years in the National Institutes of Health database
(PubMed). The publications and recommendations of the Euro-
pean and American cardiological societies are also considered:
American Heart Association/American College of Cardiology
(AHA/ACC),
2
the ESC in 2003,
3
the Working Group Valvular
Heart Disease of the ESC,
4
the guidelines of the German Society
of Cardiology (German Society of Cardiology),
5,6
and the ESC
Task Force on the Management of Valvular Heart Disease 2007.
7
2.3 Epidemiology
The spectrum of CVD in pregnancy is changing and differs
between countries. In the western world, the risk of CVD in preg-
nancy has increased due to increasing age at first pregnancy and
increasing prevalence of cardiovascular risk factors—diabetes,
hypertension, and obesity. Also the treatment of congenital heart
disease has improved, resulting in an increased number of
women with heart disease reaching childbearing age.
8
In western
countries maternal heart disease is now the major cause of
maternal death during pregnancy.
9
Hypertensive disorders are the most frequent cardiovascular
events during pregnancy, occurring in 6–8% of all pregnancies.
10
In the western world, congenital heart disease is the most frequent
cardiovascular disease present during pregnancy (75– 82%), with
shunt lesions predominating (20–65%).
11,12
Congenital heart
disease represents just 9– 19% outside Europe and North
America. Rheumatic valvular disease dominates in non-western
countries, comprising 56– 89% of all cardiovascular diseases in
pregnancy.
11,12
Cardiomyopathies are rare, but represent severe causes of car-
diovascular complications in pregnancy. Peripartum cardiomyopa-
thy (PPCM) is the most common cause of severe complications.
13
2.4 Haemodynamic, haemostatic, and
metabolic alterations during pregnancy
Pregnancy induces changes in the cardiovascular system to meet
the increased metabolic demands of the mother and fetus. They
include increases in blood volume and cardiac output (CO), and
reductions in systemic vascular resistance and blood pressure (BP).
Plasma volume reaches a maximum of 40% above baseline at 24
weeks gestation. A 30–50% increase in CO occurs in normal preg-
nancy. In early pregnancy increased CO is primarily related to the
rise in stroke volume; however, in late pregnancy, heart rate is the
major factor. Heart rate starts to rise at 20 weeks and increases
until 32 weeks. It remains high 2 –5 days after delivery. Systemic
BP (SBP) typically falls early in gestation and diastolic BP (DBP)
is usually 10 mmHg below baseline in the second trimester. This
decrease in BP is caused by active vasodilatation, achieved
Table 2 Levels of evidence
Level of
Evidence A
Data derived from multiple randomized
clinical trials
or meta-analyses.
Level of
Evidence B
Data derived from a single randomized
clinical trial
or large non-randomized studies.
Level of
Evidence C
Consensus of opinion of the experts and/
or small studies, retrospective studies,
registries.
ESC Guidelines 3151
through the action of local mediators such as prostacyclin and
nitric oxide. In the third trimester, the DBP gradually increases
and may normalize to non-pregnant values by term.
The heart can increase its size by up to 30%, which is partially
due to dilatation. Data regarding systolic and diastolic function in
pregnancy are scarce. Systolic function increases first but may
decrease in the last trimester. Reports on diastolic function are
conflicting.
Pregnancy induces a series of haemostatic changes, with an
increase in concentration of coagulation factors, fibrinogen, and
platelet adhesiveness, as well as diminished fibrinolysis, which lead
to hypercoagulability and an increased risk of thrombo-embolic
events. In addition, obstruction to venous return by the enlarging
uterus causes stasis and a further rise in risk of thrombo-embolism.
Maternal glucose homeostasis may change and cholesterol levels
increase in adaptation to fetal–maternal needs.
Physiological changes that occur during pregnancy can affect
absorption, excretion, and bioavailability of all drugs.
14
The
increased intravascular blood volume partly explains the higher
dosages of drugs required to achieve therapeutic plasma concen-
trations, and the dose adaptations needed during treatment. More-
over, the raised renal perfusion and the higher hepatic metabolism
increase drug clearance. The altered pharmacokinetics of drugs
vary in magnitude during different stages of pregnancy, making
careful monitoring of the patient and dose adjustments necessary.
Uterine contractions, positioning (left lateral vs. supine), pain,
anxiety, exertion, bleeding, and uterine involution cause significant
haemodynamic changes during labour and post-partum. Anaesthe-
sia, analgesia, haemorrhage, and infection may induce additional
cardiovascular stress. SBP and DBP increase 15–25% and 10–
15%, respectively, during uterine contractions. Such increases are
associated with a rise in pressure in the amniotic fluid, and in the
intrathoracic venous, cerebrospinal, and extradural fluids. CO
increases by 15% in early labour, by 25% during stage 1, and by
50% during expulsive efforts.
15
It reaches an increase of 80%
early post-partum due to autotransfusion associated with uterine
involution and resorption of leg oedema.
In conclusion, the physiological adaptations to pregnancy influ-
ence the evaluation and interpretation of cardiac function and clini-
cal status.
2.5 Genetic testing and counselling
An important aspect concerning the care of young women with
CVD is the consultation about the risk of inheritance of cardiac
defects for their descendants. The risk is raised significantly in com-
parison with parents without CVD where the risk is 1%. In
addition, there are large differences between each of the heredi-
tary heart disease conditions, and the risk for descendants is
dependent on whether only the mother, only the father, or both
parents suffer from hereditary cardiac defects.
16
In general, the
risk is higher when the mother is affected rather than the
father.
16
The recurrence risk varies between 3% and 50% depend-
ing on the type of maternal heart disease.
Children of parents with a cardiovascular condition inherited in
an autosomal dominant manner (e.g. Marfan syndrome, hyper-
trophic cardiomyopathy, or long QT syndrome) have an inheri-
tance risk of 50%, regardless of gender of the affected parent.
The final phenotype will also be determined by incomplete pene-
trance and pleiotropic effects, and may vary significantly. For
defects that are inherited in a polygenic manner, recurrence risk
is less clearly defined. Autosomal recessive and X-chromosomal
recessive inheritance are rare.
Genetic testing may be useful:
† in cardiomyopathies and channelopathies, such as long QT
syndromes
17
† when other family members are affected
† when the patient has dysmorphic features, developmental delay/
mental retardation, or when other non-cardiac congenital
abnormalities are present, in syndromes such as in Marfan,
22q11 deletion, Williams–Beuren, Alagille, Noonan, and
Holt–Oram syndrome.
For a steadily increasing number of genetic defects, genetic screen-
ing by chorionic villous biopsy can be offered in the 12th week of
pregnancy. All women with congenital heart disease should be
offered fetal echocardiography in the 19th to 22nd week of preg-
nancy. Measurement of nuchal fold thickness in the 12th to 13th
week of pregnancy is an early screening test for women over 35
years of age. The sensitivity for the presence of a significant
heart defect is 40%, while the specificity of the method is 99%.
The incidence of congenital heart disease with normal nuchal
fold thickness is 1/1000.
18
The inheritance pattern differs among the diseases, and there-
fore genetic counselling by a geneticist is highly recommended
for patients and their family members.
17
Genetic testing after
careful counselling has the rationale of identifying at-risk asympto-
matic or disease-free relatives and to guide clinical surveillance for
disease onset, thereby enhancing preventive and treatment inter-
ventions. It is advocated in patients with known genetic disorders
and is more advisable if treatment options are available.
17
2.6 Cardiovascular diagnosis
in pregnancy
The following procedures are of relevance for the diagnosis and
management of CVD in pregnancy.
History and clinical investigation
Many disorders can be identified by taking a careful personal and
family history, particularly cardiomyopathies, the Marfan syn-
drome, congenital heart disease, juvenile sudden death, long
QT syndrome, and catecholaminergic ventricular tachycardia
(VT) or Brugada syndrome. It is important to ask specifically
about possible sudden deaths in the family. The assessment of
dyspnoea is important for diagnosis and prognosis of valve
lesions and for heart failure. A thorough physical examination
considering the physiological changes that occur during preg-
nancy (Section 2.4) is mandatory, including auscultation for
new murmurs, changes in murmurs, and looking for signs of
heart failure. When dyspnoea occurs during pregnancy or
when a new pathological murmer is heard, echocardiography is
indicated. It is crucial to measure the BP, in left lateral recum-
bency (see Section 9) using a standardized method, and to
look for proteinuria, especially with a history or family history
ESC Guidelines3152
of hypertension or pre-eclampsia. Oximetry should be per-
formed in patients with congenital heart disease.
Electrocardiography
The great majority of pregnant patients have a normal electrocar-
diogram (ECG). The heart is rotated towards the left and on the
surface ECG there is a 15–20 left axis deviation. Common findings
include transient ST segment and T wave changes, the presence of
a Q wave and inverted T waves in lead III, an attenuated Q wave in
lead AVF, and inverted T waves in leads V1, V2, and, occasionally,
V3. ECG changes can be related to a gradual change in the position
of the heart and may mimic left ventricular (LV) hypertrophy and
other structural heart diseases.
Holter monitoring should be performed in patients with known
previous paroxysmal or persistent documented arrhythmia [VT,
atrial fibrillation (AF), or atrial flutter] or those reporting symp-
toms of palpitations.
Echocardiography
Because echocardiography does not involve exposure to radiation,
is easy to perform, and can be repeated as often as needed, it has
become an important tool during pregnancy and is the preferred
screening method to assess cardiac function.
Transoesophageal echocardiography
Multiplane transducers have made transoesophageal echocardio-
graphy a very useful echocardiographic method in the assessment
of adults with, for example, complex congenital heart disease.
Transoesophageal echocardiography, although rarely required, is
relatively safe during pregnancy. The presence of stomach con-
tents, risk of vomiting and aspiration, and sudden increases in
intra-abdominal pressure should be taken into account, and fetal
monitoring performed if sedation is used.
Exercise testing
Exercise testing is useful to assess objectively the functional
capacity, chronotropic and BP response, as well as
exercise-induced arrhythmias. It has become an integral part of
the follow-up of grown up congenital heart disease patients as
well as patients with asymptomatic valvular heart disease.
19,20
It
should be performed in patients with known heart disease, prefer-
ably prior to pregnancy to assist in risk assessment.
This Committee recommends performing submaximal exercise
tests to reach 80% of predicted maximal heart rate in asympto-
matic pregnant patients with suspected CVD. There is no evidence
that it increases the risk of spontaneous abortion.
21
Semi-
recumbent cycle ergometry appears to be the most comfortable
modality, but treadmill walking or upright cycle ergometry may
also be used. Dobutamine stress should be avoided. If respiratory
gas analysis is used, the limit is a respiratory exchange ratio of 1.0.
Stress echocardiography using bicycle ergometry may add to the
diagnostic specificity in detecting the presence and extent of
ischaemia in high risk patients with possible coronary artery
disease. This can also be useful prior to conception to assess myo-
cardial reserve in patients with prior PPCM and recovered LV func-
tion [left ventricular ejection fraction (LVEF)], and also in patients
with other cardiomyopathies, with valvular or congenital heart
disease with borderline or mildly reduced LVEF. Nuclear scintigra-
phy should be avoided during pregnancy because of radiation
exposure.
Radiation exposure
The effects of radiation on the fetus depend on the radiation dose
and the gestational age at which exposure occurs. If possible, pro-
cedures should be delayed until at least the completion of the
period of major organogenesis (. 12 weeks after menses). There
is no evidence of an increased fetal risk of congenital malformations,
intellectual disability, growth restriction, or pregnancy loss at doses
of radiation to the pregnant woman of , 50 mGy
22,23
(www.bt.cdc.
gov/radiation/prenatalphysician.asp; accessed 31 October 2007).
There may be a small increase in risk (1:2000 vs. 1:3000) of childhood
cancer. The threshold at which an increased risk of congenital mal-
formations occurs has not been definitely determined. Some evi-
dence suggests that risk of malformations is increased at doses
.100 mGy, whereas the risk between 50 and 100 mGy is less
clear. During the first 14 days after fertilization, intact survival
without fetal abnormality or death are the most likely outcomes of
radiation exposure .50 mGy. After the first 14 days, radiation
exposure .50 mGy may be associated with an increased risk of con-
genital malformations, growth restriction, and intellectual disability.
Most medical procedures do not expose the fetus to such high
levels of radiation (Table 3). For the majority of diagnostic medical
procedures, involving doses to the fetus of up to 1 mGy, the
associated risks of childhood cancer are very low. (Documents
of the Health Protection Agency. Radiation, Chemical and Environ-
mental Hazards March 2009. RSE-9 Protection of pregnant patients
during diagnostic medical exposures to ionising radiation. Advice
from the Health Protection Agency, The Royal College of Radiol-
ogists, and the College of Radiographers.)
Table 3 Estimated fetal and maternal effective doses
for various diagnostic and interventional radiology
procedures
Procedure Fetal exposure
Maternal
exposure
Chest radiograph
(PA and lateral)
<0.01 mGy <0.01 mSv 0.1 mGy 0.1 mSv
CT chest 0.3 mGy 0.3 mSv 7 mGy 7 mSv
Coronary
angiography
a
1.5 mGy 1.5 mSv 7 mGy 7 mSv
PCI or
radiofrequency
catheter ablation
a
3 mGy 3 mSv 15 mGy 15 mSv
a
Exposure depends on the number of projections or views.
CT ¼ computed tomography; PA ¼ postero-anterior; PCI ¼ percutaneous
coronary intervention.
ESC Guidelines 3153
As a general rule, according to the principle ‘as low as reason-
ably achievable’ (ALARA), all radiation doses due to medical
exposures must be kept as low as reasonably achievable.
24
Chest radiograph
The fetal dose from a chest radiograph is ,0.01 mGy.
25
Neverthe-
less, a chest radiograph should only be obtained if other methods
fail to clarify the cause of dyspnoea, cough, or other symptoms.
23
If the required diagnostic information can be obtained with an
imaging modality that does not use ionizing radiation, it should
be used as a first-line test. If a study that uses ionizing radiation
has to be performed, the radiation dose to the fetus should be
kept as low as possible (preferably ,50 mGy). The risks and
benefits of performing or not performing the examination should
be communicated. Documentation of the radiation dose to the
mother in the medical records, particularly if the fetus is in the
field of view, is highly recommended.
26,27
Magnetic resonance imaging and computed tomography
Magnetic resonance imaging (MRI) may be useful in diagnosing
complex heart disease or pathology of the aorta.
28
It should only
be performed if other diagnostic measures, including transthoracic
and transoesophageal echocardiography, are not sufficient for
complete diagnosis. Limited data during organogenesis are avail-
able, but MRI is probably safe, especially after the first trimester.
29
Gadolinium can be assumed to cross the fetal blood– placental
barrier, but data are limited. The long-term risks of exposure of
the developing fetus to free gadolinium ions
30
are not known,
and therefore gadolinium should be avoided.
Computed tomography (CT)
31
is usually not necessary to diag-
nose CVD during pregnancy and, because of the radiation dose
involved, is therefore not recommended. One exception is that
it may be required for the accurate diagnosis or definite exclusion
of pulmonary embolism. For this indication it is recommended if
other diagnostic tools are not sufficient (see Section 10). Low radi-
ation CT 1 –3 mSv can be used in these situations.
Cardiac catheterization
During coronary angiography the mean radiation exposure to the
unshielded abdomen is 1.5 mGy, and ,20% of this reaches the
fetus because of tissue attenuation. Shielding the gravid uterus
from direct radiation and especially shortening fluoroscopic time
will minimize radiation exposure. The radial approach is preferable
and should be undertaken by an experienced operator. Most elec-
trophysiological studies aiming for ablation should only be per-
formed if arrhythmias are intractable to medical treatment and
cause haemodynamic compromise. If undertaken, electroanatomi-
cal mapping systems should be used to reduce the radiation
dose.
32
General recommendations for diagnostic and therapeutic man-
agement during pregnancy are listed in Table 9.
2.7 Fetal assessment
First trimester ultrasound allows accurate measurement of gesta-
tional age and early detection of multiple pregnancy and of malfor-
mations. Diagnosis of congenital cardiac malformations can be
made as early as 13 weeks, and, in families with heart disease,
this timing is appropriate to start screening for congential heart
disease. A review of the accuracy of first-trimester ultrasounds
for detecting major congenital heart disease showed a sensitivity
and specificity of 85% [95% confidence interval (CI) 78– 90%]
and 99% (95% CI 98–100%), respectively. Early examination in
pregnancy allows parents to consider all options, including termin-
ation of pregnancy, if there are major malformations.
33
The optimum time for screening of normal pregnancies for con-
genital heart diseases
34
is 18–22 weeks of gestation when visual-
ization of the heart and outflow tracts is optimal. It becomes
more difficult after 30 weeks since the fetus is more crowded
within the amniotic cavity. Second-trimester screening (18– 22
weeks) for detection of fetal anomalies should be performed by
experienced specialists, particularly in pregnancies with risk
factors for congenital heart anomalies.
35
Cardiac anatomy and function, arterial and venous flow, and
rhythm should be evaluated. When a fetal cardiac anomaly is sus-
pected, it is mandatory to obtain the following.
(1) A full fetal echocardiography to evaluate cardiac structure and
function, arterial and venous flow, and rhythm.
(2) Detailed scanning of the fetal anatomy to look for associated
anomalies (particularly the digits and bones).
(3) Family history to search for familial syndromes.
(4) Maternal medical history to identify chronic medical disorders,
viral illnesses, or teratogenic medications.
(5) Fetal karyotype (with screening for deletion in 22q11.2 when
conotruncal anomalies are present).
(6) Referral to a maternal –fetal medicine specialist, paediatric car-
diologist, geneticist, and/or neonatologist to discuss prognosis,
obstetric, and neonatal management, and options.
(7) Delivery at an institution that can provide neonatal cardiac
care, if needed.
Doppler velocimetry (uterine, umbilical, fetal renal, and cerebral
arteries, and descending aorta) provides a non-invasive measure
of the fetoplacental haemodynamic state. Abnormality of the
Doppler index in the umbilical artery correlates to fetoplacental
vascular maldevelopment, fetal hypoxia, acidosis, and adverse peri-
natal outcome. The most ominous pre-terminal findings of the
umbilical artery Doppler waveform are absent end-diastolic vel-
ocity and reversed end-diastolic velocity. Reversed end-diastolic
velocity beyond 28 weeks should prompt immediate delivery by
caesarean delivery. Absent end-diastolic velocity should prompt
immediate consideration of delivery beyond 32 completed
weeks.
36
Fetal biophysical profile testing is indicated in pregnancies at risk
of fetal compromise. Testing should be performed one or more
times per week, depending upon the clinical situation. Four echo-
graphic biophysical variables (fetal movement, tone, breathing, and
amniotic fluid volume) and results of non-stress testing are used
for scoring. Their presence implies absence of significant
central nervous system hypoxaemia/acidaemia. A compromised
fetus exhibits loss of accelerations of the fetal heart rate, decreased
body movement and breathing, hypotonia, and, less acutely,
decreased amniotic fluid volume. From 70% to 90% of late fetal
deaths display evidence of chronic and/or acute compromise.
Sonographic detection of signs of fetal compromise can allow
ESC Guidelines3154
appropriate intervention that ideally will prevent adverse fetal
sequelae.
37,38
2.8 Interventions in the mother during
pregnancy
2.8.1 Percutaneous therapy
The same restrictions which apply for diagnostic coronary angio-
graphy (see Section 2.6) are relevant. If an intervention is absol-
utely necessary, the best time to intervene is considered to be
after the fourth month in the second trimester. By this time orga-
nogenesis is complete, the fetal thyroid is still inactive, and the
volume of the uterus is still small, so there is a greater distance
between the fetus and the chest than in later months. Fluoroscopy
and cineangiography times should be as brief as possible and the
gravid uterus should be shielded from direct radiation. Heparin
has to be given at 40 –70 U/kg, targeting an activated clotting
time of at least 200 s, but not exceeding 300 s.
2.8.2 Cardiac surgery with cardiopulmonary bypass
Maternal mortality during cardiopulmonary bypass is now similar
to that in non-pregnant women who undergo comparable
cardiac procedures.
1
However, there is significant morbidity
including late neurological impairment in 3–6% of children, and
fetal mortality remains high.
39
For this reason cardiac surgery is
recommended only when medical therapy or interventional pro-
cedures fail and the mother’s life is threatened. The best period
for surgery is between the 13th and 28th week.
40,41
Surgery
during the first trimester carries a higher risk of fetal malfor-
mations, and during the third trimester there is a higher inci-
dence of pre-term delivery and maternal complications. We
know from previous studies that gestational age has a large
impact on neonatal outcome.
42
Recent improvement in neonatal
care has further improved survival of premature infants. At 26
weeks, survival is generally 80%, with 20% having serious
neurological impairment. For this reason, caesarean delivery
may be considered before cardiopulmonary bypass if gestational
age is .26 weeks.
43
Whether or not delivery is advantageous
for the baby at this gestational age depends on several factors:
gender, estimated weight, prior administration of corticosteroids
before delivery, and the outcome statistics of the neonatal unit
concerned. When gestational age is 28 weeks or more, delivery
before surgery should be considered. Before surgery a full
course (at least 24 h) of corticosteroids should be administered
to the mother, whenever possible. During cardiopulmonary
bypass, fetal heart rate and uterine tone should be monitored
in addition to standard patient monitoring. Pump flow .2.5 L/
min/m
2
and perfusion pressure .70 mmHg are mandatory to
maintain adequate utero-placental blood flow; pulsatile flow,
although controversial, seems more effective for preserving uter-
oplacental blood flow. Maternal haematocrit .28% is rec-
ommended to optimize the oxygen delivery. Normothermic
perfusion, when feasible, is advocated, and state of the art pH
management is preferred to avoid hypocapnia responsible for
uteroplacental vasoconstriction and fetal hypoxia. Cardiopulmon-
ary bypass time should be minimized.
44
2.9 Timing and mode of delivery: risk for
mother and child
High risk delivery
Induction, management of labour, delivery, and post-partum sur-
veillance require specific expertise and collaborative management
by skilled cardiologists, obstetricians, and anaesthesiologists, in
experienced maternal –fetal medicine units.
45,46
Timing of delivery
Spontaneous onset of labour is appropriate for women with
normal cardiac function and is preferable to induced labour for
the majority of women with heart disease. Timing is individualized,
according to the gravida’s cardiac status, Bishop score (a score
based upon the station of the presenting part and four character-
istics of the cervix: dilatation, effacement, consistency, and pos-
ition), fetal well-being, and lung maturity. Due to a lack of
prospective data and the influence of individual patient character-
istics, standard guidelines do not exist, and management should
therefore be individualized. In women with mild unrepaired conge-
nital heart disease and in those who have undergone successful
cardiac surgical repair with minimal residua, the management of
labour and delivery is the same as for normal pregnant women.
Labour induction
Oxytocin and artificial rupture of the membranes are indicated
when the Bishop score is favourable. A long induction time
should be avoided if the cervix is unfavourable. While there is
no absolute contraindication to misoprostol or dinoprostone,
there is a theoretical risk of coronary vasospasm and a low risk
of arrhythmias. Dinoprostone also has more profound effects on
BP than prostaglandin E
1
and is therefore contraindicated in
active CVD. Mechanical methods such as a Foley catheter would
be preferable to pharmacological agents, particularly in the
patient with cyanosis where a drop in systemic vascular resistance
and/or BP would be detrimental.
47
Vaginal or caesarean delivery
The preferred mode of delivery is vaginal, with an individualized
delivery plan which informs the team of timing of delivery (spon-
taneous/induced), method of induction, analgesia/regional anaes-
thesia, and level of monitoring required. In high risk lesions,
delivery should take place in a tertiary centre with specialist
multidisciplinary team care. Vaginal delivery is associated with
less blood loss and infection risk compared with caesarean deliv-
ery, which also increases the risk of venous thrombosis and
thrombo-embolism.
48
In general, caesarean delivery is reserved
for obstetric indications. There is no consensus regarding absolute
contraindications to vaginal delivery as this is very much dependent
on maternal status at the time of delivery and the anticipated
cardiopulmonary tolerance of the patient. Caesarean delivery
should be considered for the patient on oral anticoagulants
(OACs) in pre-term labour, patients with Marfan syndrome and
an aortic diameter .45 mm, patients with acute or chronic
aortic dissection, and those in acute intractable heart failure.
Cesarean delivery may be considered in Marfan patients with an
aortic diameter 40–45 mm.
7,49,50
(see also Section 4.3).
ESC Guidelines 3155
In some centres, caesarean delivery is advocated for women with
severe aortic stenosis (AS) and in patients with severe forms of pul-
monary hypertension (including Eisenmenger syndrome), or acute
heart failure.
7,46
(see specific sections). Caesarean delivery may be
considered in patients with mechanical heart valve prostheses to
prevent problems with planned vaginal delivery. In such patients, a
prolonged switch to heparin/low molecular weight heparin
(LMWH) may indeed be required for a long time before vaginal
birth, particularly, when the obstetrical situation is unfavourable.
This would increase the maternal risk (see also Sections 5.5 and 5.6).
Haemodynamic monitoring
Systemic arterial pressure and maternal heart rate are monitored,
because lumbar epidural anaesthesia may cause hypotension. Pulse
oximetry and continuous ECG monitoring are utilized as required.
A Swan–Ganz catheter for haemodynamic monitoring is rarely if
ever indicated due to the risk of arrhythmia provocation, bleeding,
and thrombo-embolic complications on removal.
51
Anaesthesia/analgesia
Lumbar epidural analgesia is often recommendable because it
reduces pain-related elevations of sympathetic activity, reduces
the urge to push, and provides anaesthesia for surgery. Continuous
lumbar epidural analgesia with local anaesthetics or opiates, or
continuous opioid spinal anaesthesia can be safely administered.
Regional anaesthesia can, however, cause systemic hypotension
and must be used with caution in patients with obstructive valve
lesions. Intravenous (i.v.) perfusion must be monitored carefully.
52
Labour
Once in labour, the woman should be placed in a lateral decubitus
position to attenuate the haemodynamic impact of uterine con-
tractions.
53
The uterine contractions should descend the fetal
head to the perineum, without maternal pushing, to avoid the
unwanted effects of the Valsalva manoeuvre.
54,55
Delivery may be assisted by low forceps or vacuum extraction.
Routine antibiotic prophylaxis is not recommended. Continuous
electronic fetal heart rate monitoring is recommended.
Delivery in anticoagulated women with prosthetic valves
OACs should be switched to LMWH or unfractionated heparin
(UFH) from the 36th week. Women treated with LMWH should
be switched to i.v. UFH, at least 36 h before the induction of
labour or caesarean delivery. UFH should be discontinued 4–6 h
before planned delivery, and restarted 4– 6 h after delivery if
there are no bleeding complications (see also Section 5.5).
Urgent delivery in a patient with a mechanical valve taking thera-
peutic anticoagulation may be necessary, and there is a high risk
of severe maternal haemorrhage. If emergent delivery is necessary
while the patient is still on UFH or LMWH, protamine should be
considered. Protamine will only partially reverse the anticoagulant
effect of LMWH. In the event of urgent delivery in a patient on
therapeutic OACs, caesarean delivery is preferred to reduce the
risk of intracranial haemorrhage in the fully anticoagulated fetus.
If emergent delivery is necessary, fresh frozen plasma should be
given prior to caesarean delivery to achieve a target international
normalized ratio (INR) of ≤2.
4
Oral vitamin K (0.5–1 mg) may
also be given, but it takes 4–6 h to influence the INR. If the
mother was on OACs at the time of delivery, the anticoagulated
newborn may be given fresh frozen plasma and should receive
vitamin K. The fetus may remain anticoagulated for 8–10 days
after discontinuation of maternal OACs.
Ventricular arrhythmias during pregnancy and labour
Arrhythmias are the most common cardiac complication during preg-
nancy in women with and without structural heart disease.
12,56,57
They may manifest for the first time during pregnancy, or pregnancy
may exacerbate pre-existing arrhythmias.
58–60
The 2006 ACC/AHA/
ESC guidelines for management of patients with ventricular arrhyth-
mias and the prevention of sudden cardiac death recommend that
pregnant women with prolonged QT syndrome who have had symp-
toms benefit from continued b-blocker therapy throughout preg-
nancy, during delivery, and post-partum unless there are definite
contraindications. Use of b-blockers during labour does not
prevent uterine contractions and vaginal delivery.
61
Post-partum care
A slow i.v. infusion of oxytocin (,2 U/min), which avoids systemic
hypotension, is administered after placental delivery to prevent
maternal haemorrhage. Prostaglandin F analogues are useful to
treat post-partum haemorrhage, unless an increase in pulmonary
artery pressure (PAP) is undesirable. Methylergonovine is contra-
indicated because of the risk (. 10%) of vasoconstriction and
hypertension.
62,63
Meticulous leg care, elastic support stockings,
and early ambulation are important to reduce the risk of
thrombo-embolism. Delivery is associated with important haemo-
dynamic changes and fluid shifts, particularly in the first 12–24 h,
which may precipitate heart failure in women with structural
heart disease. Haemodynamic monitoring should therefore be
continued for at least 24 h after delivery.
64
Breastfeeding
Lactation is associated with a low risk of bacteraemia secondary to
mastitis. In highly symptomatic/unwell patients, bottle-feeding
should be considered.
2.10 Infective endocarditis
Infective endocarditis during pregnancy is rare, with an estimated
overall incidence of 0.006% (1 per 100 000 pregnancies)
65
and
an incidence of 0.5% in patients with known valvular or congenital
heart disease.
66
The incidence is higher in drug addicts. Patients
with the highest risk for infective endocarditis are those with a
prosthetic valve or prosthetic material used for cardiac valve
repair, a history of previous infective endocarditis, and some
special patients with congenital heart disease.
2.10.1 Prophylaxis
The same measures as in non-pregnant patients with recent modi-
fications of guidelines apply.
67
Endocarditis prophylaxis is now only
recommended for patients at highest risk of aquiring endocarditis
during high risk procedures, e.g. dental procedures. During delivery
the indication for prophylaxis has been controversial and, given the
lack of convincing evidence that infective endocarditis is related to
either vaginal or caesarean delivery, antibiotic prophylaxis is not
recommended during vaginal or caesarean delivery.
67,68
ESC Guidelines3156
2.10.2 Diagnosis and risk assessment
The diagnosis of infective endocarditis during pregnancy involves
the same criteria as in the non-pregnant patient.
67
In spite of pro-
gress in the diagnosis and treatment of infective endocarditis,
maternal morbidity and mortality remain high, reportedly 33% in
one study (mainly due to heart failure and thrombo-embolic com-
plications).
69
Fetal mortality is also high at 29%. Heart failure due
to acute valve regurgitation is the most common complication,
requiring urgent surgery when medical treatment cannot stabilize
the patient.
67
Cerebral and peripheral embolizations are also fre-
quent complications.
2.10.3 Treatment
Infective endocarditis should be treated the same way as in the
non-pregnant patient, bearing in mind the fetotoxic effects of anti-
biotics (see Section 11). If infective endocarditis is diagnosed, anti-
biotics should be given guided by culture and antibiotic sensitivity
results and local treatment protocols. Antibiotics that can be given
during all trimesters of pregnancy are penicillin, ampicillin, amoxi-
cillin, erythromycin, mezlocillin, and cephalosporins.
70
All of
them are included in group B of the Food and Drug Administration
(FDA) classification. Vancomycin, imipenem, rifampicin, and teico-
planin are all group C, which means risk cannot be excluded and
their risk –benefit ratio must be carefully considered. There is a
definite risk to the fetus in all trimesters of pregnancy with
group D drugs (aminoglycosides, quinolones, and tetracyclines)
and they should therefore only be used for vital indications.
71
Valve surgery during pregnancy should be reserved for cases
where medical therapy has failed as per guidelines in non-pregnant
patients.
67
A viable fetus should be delivered prior to surgery
where possible (see Section 2.8.2).
2.11 Risk estimation: contraindications
for pregnancy
2.11.1 Pre-pregnancy counselling
The risk of pregnancy depends on the specific heart disease and
clinical status of the patient. Individual counselling by experts is rec-
ommended. Adolescents should be given advice on contraception,
and pregnancy issues should be discussed as soon as they become
sexually active. A risk assessment should be performed prior to
pregnancy and drugs reviewed so that those which are contraindi-
cated in pregnancy can be stopped or changed to alternatives
where possible (see Section 11.2, Table 21). The follow-up plan
should be discussed with the patient and, if possible, her partner.
Women with significant heart disease should be managed jointly
by an obstetrician and a cardiologist with experience in treating
pregnant patients with heart disease from an early stage. High
risk patients should be managed by an expert multidisciplinary
team in a specialist centre. All women with heart disease should
be assessed at least once before pregnancy and during pregnancy,
and hospital delivery should be advised.
2.11.2 Risk assessment: estimation of maternal and
offspring risk
To estimate the risk of maternal cardiovascular complications,
several approaches are available. Disease-specific risk can be
assessed, and is described in these guidelines in the respective
sections dealing with specific diseases. In general, the risk of com-
plications increases with increasing disease complexity.
56,72
Disease-specific series are usually retrospective and too small to
identify predictors of poor outcome. Therefore, risk estimation can
be further refined by taking into account predictors that have been
identified in studies that included larger populations with various dis-
eases. Severalrisk scores have been developed based onthese predic-
tors, of which the CARPREG risk score is most widely known and
used. This risk score has been validated in several studies and
Table 4 Predictors of maternal cardiovascular events
and risk score from the CARPREG study
12
Prior cardiac event (heart failure, transient ischaemic attack, stroke
before pregnancy or arrhythmia).
Baseline NYHA functional class >II or cyanosis.
Left heart obstruction (mitral valve area <2 cm
2
, aortic valve area
<1.5 cm
2
, peak LV outflow tract gradient >30 mmHg by
echocardiography).
Reduced systemic ventricular systolic function (ejection fraction
<40%).
CARPREG risk score: for each CARPREG predictor that is present a point is
assigned. Risk estimation of cardiovascular maternal complications
0 point 5%
1 point 27%
.1 point 75%
LV ¼ left ventricular; NYHA ¼ New York Heart Association.
Table 5 Predictors of maternal cardiovascular events
identified in congential heart diseases in the ZAHARA
and Khairy study
ZAHARA predictors
57
History of arrhythmia event.
Baseline NYHA functional class >II.
Left heart obstruction (aortic valve peak gradient >50 mm Hg).
Mechanical valve prosthesis.
Moderate/severe systemic atrioventricular valve regurgitation (possibly
related to ventricular dysfunction).
Moderate/severe sub-pulmonary atrioventricular valve regurgitation
(possibly related to ventricular dysfunction).
Use of cardiac medication pre-pregnancy.
Repaired or unrepaired cyanotic heart disease.
Predictors from Khairy
76
Smoking history.
Reduced subpulmonary ventricular function and/or severe pulmonary
regurgitation.
NYHA ¼ New York Heart Association.
ESC Guidelines 3157
appears valuable to predict maternal risk, although overestimation
can occur.
57,73
The CARPREG risk score is described in Table 4.In
women with congenital heart disease, the CARPREG score
12
may
also be associated with a higher risk of late cardiovascularevents post-
pregnancy.
74
The predictors from the ZAHARA study
57
(Table 5)
have not yet been validated in other studies. It should be noted that
predictors and risk scores from the CARPREG and ZAHARA
studies are highly population dependent. Important risk factors
including pulmonary arterial hypertension (PAH) and dilated aorta
were not identified because they were under-represented in these
studies. The CARPREG study included acquired and congenital
heart disease, while the ZAHARA study investigated a population
with congenital heart disease only.
The Task Force recommends that maternal risk assessment is
carried out according to the modified World Health Organization
(WHO) risk classification.
72
This risk classification integrates all
known maternal cardiovascular risk factors including the underlying
heart disease and any other co-morbidity. It includes contraindica-
tions for pregnancy that are not incorporated in the CARPREG
and ZAHARA risk scores/predictors. The general principles of
this classification are depicted in Table 6. A practical application
is given in Table 7. In women in WHO class I, risk is very low,
and cardiology follow-up during pregnancy may be limited to
one or two visits. Those in WHO II are at low or moderate risk,
and follow-up every trimester is recommended. For women in
WHO class III, there is a high risk of complications, and frequent
(monthly or bimonthly) cardiology and obstetric review during
pregnancy is recommended. Women in WHO class IV should be
advised against pregnancy but, if they become pregnant and will
not consider termination, monthly or bimonthly review is needed.
Neonatal complications occur in 20– 28% of patients with heart
disease
12,56,57,75,76
with a neonatal mortality between 1% and
4%.
12,56,57
Maternal and neonatal events are highly correlated.
57
Predictors of neonatal complications are listed in Table 8.
Table 7 Modified WHO classification of maternal
cardiovascular risk: application
Conditions in which pregnancy risk is WHO I
• Uncomplicated, small or mild
- pulmonary stenosis
- patent ductus arteriosus
- mitral valve prolapse
• Successfully repaired simple lesions (atrial or ventricular septal
defect, patent ductus arteriosus, anomalous pulmonary venous
drainage).
• Atrial or ventricular ectopic beats, isolated
Conditions in which pregnancy risk is WHO II or III
WHO II (if otherwise well and uncomplicated)
• Unoperated atrial or ventricular septal defect
• Repaired tetralogy of Fallot
• Most arrhythmias
WHO II–III (depending on individual)
• Mild left ventricular impairment
• Hypertrophic cardiomyopathy
• Native or tissue valvular heart disease not considered WHO I or IV
• Marfan syndrome without aortic dilatation

Aorta <45 mm in aortic disease associated with bicuspid aortic valve
• Repaired coarctation
WHO III
• Mechanical valve
• Systemic right ventricle
• Fontan circulation
• Cyanotic heart disease (unrepaired)
• Other complex congenital heart disease
• Aortic dilatation 40–45 mm in Marfan syndrome
• Aortic dilatation 45–50 mm in aortic disease associated with bicuspid
aortic valve
Conditions in which pregnancy risk is WHO IV
(pregnancy contraindicated)
• Pulmonary arterial hypertension of any cause
• Severe systemic ventricular dysfunction (LVEF <30%, NYHA III–IV)
• Previous peripartum cardiomyopathy with any residual impairment of
left ventricular function
• Severe mitral stenosis, severe symptomatic aortic stenosis
• Marfan syndrome with aorta dilated >45 mm
• Aortic dilatation >50 mm in aortic disease associated with bicuspid
aortic valve
• Native severe coarctation
Adapted from Thorne et al.
73
LVEF ¼ left ventricular ejection fraction; NYHA ¼ New York Heart Association;
WHO ¼ World Health Organization.
Table 6 Modified WHO classification of maternal
cardiovascular risk: principles
Risk class Risk of pregnancy by medical condition
I
No detectable increased risk of maternal mortality and
no/mild increase in morbidity.
II
Small increased risk of maternal mortality or moderate
increase in morbidity.
III
Significantly increased risk of maternal mortality
or severe morbidity. Expert counselling required.
If pregnancy is decided upon, intensive specialist
cardiac and obstetric monitoring needed throughout
pregnancy, childbirth, and the puerperium.
IV
Extremely high risk of maternal mortality or severe
morbidity; pregnancy contraindicated. If pregnancy
occurs termination should be discussed. If pregnancy
continues, care as for class III.
Modified from Thorne et al.
72
WHO ¼ World Health Organization
ESC Guidelines3158
2.12 Methods of contraception and
termination of pregnancy, and in vitro
fertilization
2.12.1 Methods of contraception
Contraceptive methods include combined hormonal contracep-
tives (oestrogen/progestin), progestogen-only methods, intrauter-
ine devices, and emergency contraception. Their use needs to be
balanced against the risk of pregnancy.
In 2010, the Centers for Disease Control (CDC) modified the
WHO suggestions for medical eligibility criteria for contraceptive
use in women with CVD. [http://www.cdc.gov/Mmwr/preview/
mmwrhtml/rr59e0528a13.htm]. Monthly injectables that contain
medroxyprogesterone acetate are inappropriate for patients with
heart failure because of the tendency for fluid retention. Low
dose oral contraceptives containing 20 mg of ethinyl estradiol are
safe in women with a low thrombogenic potential, but not in
women with complex valvular disease.
77,78
Apart from barrier methods (condom), the levonorgestrel-
releasing intrauterine device is the safest and most effective contra-
ceptive that can be used in women with cyanotic congenital heart
disease and pulmonary vascular disease. It reduces menstrual
blood loss by 40 –50% and induces amenorrhoea in a significant pro-
portion of users.
79
It should be borne in mind that 5% of patients
experience vasovagal reactions at the time of implant; therefore, for
those with highly complex heart disease (e.g. Fontan, Eisenmenger)
intrauterine implants are indicated only when progesterone-only
pills or dermal implants have proved unacceptable and, if used,
they should only be implanted in a hospital environment. A copper
intrauterine device is acceptable in non-cyanotic or mildly cyanotic
women. Antibiotic prophylaxis is not recommended at the time of
insertion or removal since the risk of pelvic infection is not increased.
If excessive bleeding occurs at the time of menses, the device should
be removed. It is contraindicated in cyanotic women with haemato-
crit levels .55% because intrinsic haemostatic defects increase the
risk of excessive menstrual bleeding.
2.12.2 Sterilization
Tubal ligation is usually accomplished safely, even in relatively high
risk women. Because of the associated anaesthesia and abdominal
inflation, it is, however, not without risk in patients with PAH, cya-
nosis, and Fontan circulation. The risk may be lower with the mini-
mally invasive hysteroscopic techniques such as the Essure device.
Hysteroscopic sterilization is performed by inserting a metal
micro-insert or polymer matrix into the interstitial portion of
each fallopian tube. Three months after placement, correct
device placement and bilateral tubal occlusion are confirmed
with pelvic imaging. Advantages of hysteroscopic sterilization
include the ability to perform the procedure in an outpatient
setting and without an incision. A disadvantage is the 3 month
waiting period until tubal occlusion is confirmed.
80
Vasectomy
for the male partner is another efficacious option, but the long-
term prognosis of the female partner must be taken into
account as the male partner may outlive her for many years.
Given the lack of published data about contraception in heart
disease, advice should be provided by physicians or gynaecologists
with appropriate training.
2.12.3 Methods of termination of pregnancy
Pregnancy termination should be discussed with women in whom
gestation represents a major maternal or fetal risk. The first trime-
ster is the safest time for elective pregnancy termination, which
should be performed in hospital, rather than in an outpatient facil-
ity, so that all emergency support services are available. The
method, including the need for anaesthesia, should be considered
on an individual basis. High risk patients should be managed in an
experienced centre with on-site cardiac surgery. Endocarditis pro-
phylaxis is not consistently recommended by cardiologists,
81
but
treatment should be individualized. Gynaecologists routinely
advise antibiotic prophylaxis to prevent post-abortal endometritis,
which occurs in 5 –20% of women not given antibiotics.
82,83
Dilatation and evacuation is the safest procedure in both the
first and second trimesters. If surgical evacuation is not feasible
in the second trimester, prostaglandins E
1
or E
2
, or misoprostol,
a synthetic prostaglandin structurally related to prostaglandin E
1
,
can be administered to evacuate the uterus.
84
These drugs are
absorbed into the systemic circulation and can lower systemic vas-
cular resistance and BP, and increase heart rate, effects that are
greater with E
2
than with E
1
.
85
Up to 7 weeks gestation, mifepristone is an alternative to
surgery. When prostaglandin E compounds are given, systemic
arterial oxygen saturation should be monitored with a transcu-
taneous pulse oximeter and norepinephrine infused at a rate that
supports the DBP, which reflects systemic vascular resistance.
Prostaglandin F compounds should be avoided because they can
significantly increase PAP and may decrease coronary perfusion.
85
Saline abortion should be avoided because saline absorption can
cause expansion of the intravascular volume, heart failure, and clot-
ting abnormalities.
2.12.4 In vitro fertilization
In vitro fertilization may be considered where the risk of the pro-
cedure itself, including hormonal stimulation and pregnancy, is low.
Thrombo-embolism may complicate in vitro fertilization when high
oestradiol levels may precipitate a prothrombotic state.
86
Table 8 Maternal predictors of neonatal events in
women with heart disease
1. Baseline NYHA class >II or cyanosis
12
2. Maternal left heart obstruction
12,76
3. Smoking during pregnancy
12,57
4. Multiple gestation
12,57
5. Use of oral anticoagulants during pregnancy
12
6. Mechanical valve prosthesis
57
Modified from Siu et al.
12
(CARPREG investigators); Khairy et al.
76
; Drenthen/
Pieper et al.
57
(ZAHARA investigators).
NYHA ¼ New York Heart Association.
ESC Guidelines 3159
2.13 General recommendations
3. Congenital heart disease and
pulmonary hypertension
In many women with congenital heart disease, pregnancy is well
tolerated. The risk of pregnancy depends on the underlying
heart disease as well as on additional factors such as ventricular
and valvular function, functional class, and cyanosis. The miscar-
riage rate is higher in more complex disease (Figure 1).
56
Maternal
cardiac complications are present in 12% of completed pregnan-
cies and are again more frequent as the disease becomes more
complex. Patients who experience complications during pregnancy
may also be at higher risk of late cardiac events after pregnancy.
74
Offspring complications, including offspring mortality (4%), are
more frequent than in the general population.
Diagnosis
Usually, congenital heart diseases will be known and diagnosed
before pregnancy. Pre-pregnancy assessment including medical
history, echocardiography, and exercise testing is indicated in all
patients, with other diagnostic tests indicated on an individual
patient basis. Functional status before pregnancy and history of
previous cardiac events are of particular prognostic value (see
Tables 4 and 5). Also B-type natriuretic peptide (BNP)/N-terminal
pro B-type natriuretic peptide (NT-pro-BNP) assessment may be
helpful in risk stratification. An exercise test before pregnancy
achieving ,70% of expected workload, showing a drop in arterial
pressure or a drop in oxygen saturation may identify women at
risk of developing symptoms or complications during pregnancy.
Diagnostic procedures that can be used during pregnancy are
outlined in Section 2.6.
21
For further risk assessment see
Section 2.11.
3.1 Maternal high risk conditions [World
Health Organization (III)–IV; see also
Section 2.11]
Patients in NYHA class III/IV or with severely reduced function of
the systemic ventricle are at high risk during pregnancy, along with
other specific conditions discussed below. In addition, some
specific conditions are at particular high risk during pregnancy.
3.1.1 Pulmonary hypertension
Maternal risk
Pulmonary hypertension encompasses a group of diseases with
different pathophysiologies which include PAH, pulmonary hyper-
tension related to left heart disease, pulmonary hypertension
related to lung disease and/or hypoxia, chronic thrombo-embolic
pulmonary hypertension, and pulmonary hypertension with
unclear and or multifactorial mechanisms. PAH includes the idio-
pathic and heritable forms of the disease as well as pulmonary
hypertension associated with congenital heart disease, with or
without previous corrective surgery. A mean PAP ≥25 mmHg at
rest is indicative of pulmonary hypertension.
87
A high maternal
mortality risk is reported (30–50% in older series and 17–33%
Table 9 General recommendations
Recommendations Class
a
Level
b
Pre-pregnancy risk assessment and counselling
is indicated in all women with known or
suspected congenital or acquired cardiovascular
and aortic disease.
I
C
Risk assessment should be performed in all
women with cardiac diseases of childbearing
age and after conception.
I
C
High risk patients should be treated in
specialized centres by a multidisciplinary team.
I
C
Genetic counselling should be offered to women
with congenital heart disease or congenital
arrhythmia, cardiomyopathies, aortic disease or
genetic malformations associated with CVD.
I C
Echocardiography should be performed in
any pregnant patient with unexplained or new
cardiovascular signs or symptoms.
I
C
Before cardiac surgery a full course of
corticosteroids should be administered to the
mother whenever possible.
I
C
For the prevention of infective endocarditis
in pregnancy the same measures as in non-
pregnant patients should be used.
I
C
Vaginal delivery is recommended as first choice
in most patients.
I
C
MRI (without gadolinium) should be considered
if echocardiography is insufficient for diagnosis.
IIa
C
In patients with severe hypertension, vaginal
delivery with epidural analgesia and elective
instrumental delivery should be considered.
IIa
C
When gestational age is at least 28 weeks,
delivery before necessary surgery should be
considered.
IIa
C
Caesarean delivery should be considered for
obstetric indications or for patients with
dilatation of the ascending aorta >45 mm,
severe aortic stenosis, pre-term labour while on
oral anticoagulants, Eisenmenger syndrome, or
severe heart failure.
IIa
C
Caesarean delivery may be considered in Marfan
patients with an aortic diameter 40–45mm.
IIb
C
A chest radiograph, with shielding of the fetus,
may be considered if other methods are not
successful in clarifying the cause of dyspnoea.
IIb
C
Cardiac catheterization may be considered with
very strict indications, timing, and shielding of
the fetus.
IIb
C
CT and electrophysiological studies, with
shielding of the fetus, may be considered in
selected patients for vital indications.
IIb
C
Coronary bypass surgery or valvular surgery
may be considered when conservative and
medical therapy has failed, in situations that
threaten the mother’s life and that are not
amenable to percutaneous treatment.
IIb
C
Prophylactic antibiotic therapy during delivery is
not recommended.
III
C
a
Class of recommendation.
b
Level of evidence.
CT ¼ computed tomography; CVD ¼ cardiovascular disease; MRI ¼ magnetic
resonance imaging
ESC Guidelines3160

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