Pregnancy in women with congenital heart disease: General principles

Authors
Carol A Waksmonski, MD
Michael R Foley, MD
Section Editors
Charles J Lockwood, MD, MHCM
Heidi M Connolly, MD
Deputy Editor
Susan B Yeon, MD, JD, FACC
Disclosures: Carol A Waksmonski, MD Nothing to disclose. Michael R Foley, MD Nothing to
disclose. Charles J Lockwood, MD, MHCM Consultant/Advisory Boards: Celula [Aneuploidy
screening (Prenatal and cancer DNA screening tests in development)]. Equity Ownership/Stock
Options: Celula [Aneuploidy screening (Prenatal and cancer DNA screening tests in
development)]. Heidi M Connolly, MD Nothing to disclose. Susan B Yeon, MD, JD, FACC
Employee of UpToDate, Inc.
Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found,
these are addressed by vetting through a multi-level review process, and through requirements
for references to be provided to support the content. Appropriately referenced content is required
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All topics are updated as new evidence becomes available and our peer review process is
complete.
Literature review current through: Jan 2015. | This topic last updated: Jan 08, 2015.
INTRODUCTION Progress in surgical treatment has resulted in larger numbers of women
with congenital heart disease surviving to proceeding with pregnancy [1].
The general principles of management of pregnancy and contraception in women who have
unrepaired or repaired congenital malformations of the heart or great vessels will be reviewed
here. Pregnancy in women with specific congenital cardiac anomalies, the management of
valvular heart disease during pregnancy, and the management of heart failure and arrhythmias
during pregnancy are discussed separately. (See "Pregnancy in women with congenital heart
disease: Specific lesions" and "Pregnancy and valve disease" and "Management of heart failure
during pregnancy" and "Supraventricular arrhythmias during pregnancy" and "Ventricular
arrhythmias during pregnancy".)
EPIDEMIOLOGY Successful cardiac surgery improves fertility and reduces the maternal and
fetal risk of pregnancy in women with congenital heart disease [2,3]. Accordingly, women are
now presenting for obstetric and cardiologic care after reparative cardiac surgery [1]. In a
registry of the European Society of Cardiology, congenital heart disease was the most prevalent
form of structural heart disease (66 percent) affecting pregnancy outcomes worldwide [4].

Congenital heart disease remains an important cause of maternal mortality and morbidity during
pregnancy. The magnitude of risk is illustrated by the following observations:
In a report of maternal deaths from 2000 to 2002 in the United Kingdom, cardiac disease was
the second most common cause, with congenital heart disease accounting for 20 percent of
cardiac deaths [5].
Maternal morbidity was evaluated in a review of mostly retrospective reports published from
1985 to 2006 that described the outcomes of 2491 pregnancies in women with structural
congenital heart disease [6]. The following findings were noted:
Cardiac complications were documented in 11 percent of completed pregnancies, with heart
failure (5 percent) and arrhythmias (4.5 percent) the most common.
Cardiovascular events such as myocardial infarction, stroke, and cardiovascular mortality were
reported primarily in Eisenmenger patients and in those with palliated or unrepaired cyanotic
heart disease. (See "Medical management of Eisenmenger syndrome".)
Hypertensive disorders related to pregnancy were reported in 9 percent of pregnancies, which is
comparable to the rate expected in the general population [7], but were more frequent in patients
with transposition of the great arteries, aortic coarctation, pulmonic valve stenosis, or aortic
stenosis.
Gravidas with congenital heart disease may be at higher risk during an individual pregnancy, but
if they survive, the risk of pregnancy is generally not cumulative. Thus, successive pregnancies
generally entail the same but not greater risk.
CARDIOVASCULAR CHALLENGES DURING PREGNANCY
Hemodynamic changes Normal alterations in circulatory and respiratory physiology during
pregnancy can have deleterious effects on the mother with congenital heart disease and on her
developing fetus. There are two major hemodynamic changes: fall in systemic blood pressure
and increase in cardiac output. (See "Maternal cardiovascular and hemodynamic adaptations to
pregnancy".)
Systemic blood pressure typically falls early in gestation and is usually 10 mmHg below baseline
in the second trimester, declining to a mean of 105/60 mmHg. This response reflects a reduction
in systemic vascular resistance that serves to increase flow across right-to-left shunts.
A 30 to 50 percent increase in intravascular volume and cardiac output occurs in normal
pregnancy by the early to mid third trimester. In patients whose cardiac output is limited by
myocardial dysfunction or valvular lesions (eg, mitral stenosis), volume overload is poorly
tolerated and may result in heart failure.
Marked fluctuations in cardiac output occur during normal labor and delivery. Cardiac output
increases progressively from the first stage of labor, sometimes reaching an additional 50 percent

by the late second stage. The potential for dramatic volume shifts is heightened at the time of
delivery in response to the physiologic transfusion, which occurs with release of vena caval
obstruction and blood from the now contracted uterus; postpartum hemorrhage may exacerbate
these volume shifts, which are tolerated poorly by women whose cardiac output is highly
dependent upon adequate preload.
Risk of thromboembolism In addition to the normal hemodynamic changes of pregnancy,
cardiac reserve can be impaired by thromboembolism. Pregnancy is associated with an increased
thromboembolic risk due to lower extremity venous stasis resulting from inferior vena caval
compression by the gravid uterus, and to a hypercoagulable state due to an increase in vitamin K
dependent clotting factors and a reduction in free protein S. (See "Deep vein thrombosis and
pulmonary embolism in pregnancy: Prevention".)
A review cited above found a 2 percent incidence of thromboembolic events in 688 completed
pregnancies in women with congenital heart disease [6] compared to an expected rate of 0.05 to
0.10 percent during uncomplicated completed pregnancies [8]. Potential risk factors for
thromboembolism were not evaluated.
The issue of hypercoagulability is of particular relevance in women at risk for thrombosis related
to prosthetic heart valves, atrial fibrillation, or previous thromboembolic events. (See 'Prenatal
care' below.)
MATERNAL RISK STRATIFICATION
Maternal cardiovascular risk assessment
Overview We agree with the 2011 European Society of Cardiology task force
recommendation to use the modified World Health Organization (WHO) risk classification for
maternal risk assessment [9]. The modified WHO classification provided the best risk assessment
in the prospective study by the ZAHARA investigators described below [10]. The modified
WHO risk classification includes contraindications for pregnancy not included in the two
frequently used indices for material cardiovascular risk with congenital heart disease: the
CARPREG (Cardiac Disease in Pregnancy) and the ZAHARA (Zwangerschap bij vrouwen met
een Aangeboren HARtAfwijking-II, translated as Pregnancy in women with congenital heart
disease II) scoring systems.
A prospective study examined outcomes in 213 pregnancies in 203 women with congenital heart
disease and compared the performance of the modified WHO classification, CARPREG and
ZAHARA risk scores, as well as total numbers of cardiovascular predictors and offspring risk
predictors:
Maternal cardiovascular events occurred during 22 pregnancies (10 percent)
The highest area under the curve (AUC) for maternal cardiovascular risk was achieved by the
modified WHO class (AUC: 0.77). The AUC for the ZAHARA risk score was 0.71 and the
CARPREG risk score was 0.57; the latter was not significantly different from random guess.

Modified WHO classification The Working Group on Pregnancy and Contraception classified
pregnancy risk in women with heart disease using modified World Health Organization
(WHO) classification categories [11]. We agree with the classification adopted in the 2011
European Society of Cardiology guidelines for management of cardiovascular disease during
pregnancy [12]:
Class I conditions are associated with no detectable increased risk of maternal mortality and
no/mild increase in morbidity.
Conditions in this category include uncomplicated, small, or mild pulmonic stenosis, patent
ductus arteriosus, or mitral valve prolapse; successfully repaired simple lesions (atrial or
ventricular septal defect, patent ductus arteriosus, or anomalous pulmonary venous drainage);
and isolated atrial or ventricular ectopic beats
Class II conditions are associated with small increased risk of maternal mortality or moderate
increase in morbidity.
Conditions in risk class II include unrepaired atrial or ventricular septal defect, repaired
tetralogy of Fallot, most arrhythmias.
Conditions in risk class II to III (depending on the individual) include mild left ventricular
impairment, hypertrophic cardiomyopathy, native or tissue valvular heart disease not considered
WHO I or IV, repaired coarctation, Marfan syndrome without aortic dissection, and bicuspid
aortic valve with ascending aorta diameter <45 mm.
Class III conditions are associated with significantly increased risk of maternal mortality or
severe morbidity.
Conditions in this category include a mechanical valve and bicuspid aortic valve with ascending
aortic diameter of 45 to 50 mm.
Class IV conditions are associated with extremely high risk of maternal mortality or severe
morbidity; pregnancy is contraindicated. (See 'Prenatal care' below.)
Conditions in this category include severe mitral stenosis, symptomatic severe aortic stenosis,
bicuspid aortic valve with ascending aorta diameter >50 mm, Marfan syndrome with aorta
dilated >45 mm, severe systemic ventricular systolic dysfunction (left ventricular ejection
fraction <30 percent, New York Heart Association [NYHA] III to IV), native severe coarctation,
and significant pulmonary arterial hypertension of any cause (ie, pulmonary artery systolic
pressure >25 mmHg at rest or >30 mmHg with exercise).
Recommendations for follow-up for patients with conditions in each of these categories is
discussed below. (See 'Prenatal care' below.)
ZAHARA score The ZAHARA score is based upon a retrospective observational cohort study
of 1302 completed pregnancies in 714 women with exclusively congenital heart disease,

including predominantly complicated lesions [13]. The ZAHARA risk score is derived from a
weighted scoring system to predict adverse maternal cardiac events and includes the following
factors:
Mechanical heart valve (4.25 points)
Severe left heart obstruction (mean pressure gradient >50 mmHg or aortic valve area <1.0 cm2)
(2.50 points)
History of arrhythmias (1.50 points)
History of cardiac medication use before pregnancy (1.50 points)
History of cyanotic heart disease (uncorrected or corrected) (1.00 points)
Moderate-to-severe pulmonary or systemic atrioventricular valve regurgitation (0.75)
Symptomatic heart failure before pregnancy (NYHA class II) (0.75 points)
The score is divided into five categories of risk based on accrued points: 0 to 0.5 points, 2.9
percent; 0.51 to 1.50 points, 7.5 percent; 1.51 to 2.50, 17.5 percent; 2.51 to 3.50, 43.1 percent;
and >3.51, 70.0 percent risk, respectively. However, the ZAHARA score has not been validated
in other studies. (See 'Overview' above.)
CARPREG risk score The CARPREG risk score is based upon a retrospective study that
examined the risks and predictors of pregnancy-related cardiac complications in women with
heart disease [14]. The findings were then applied by the Cardiac Disease in Pregnancy
(CARPREG) investigators in a prospective study of 562 women with congenital or acquired
cardiac disease or arrhythmias who had endured 617 pregnancies. Seventy-four percent of the
pregnancies occurred in women with congenital heart disease [15].
Four predictors of cardiac events were identified:
Poor functional class (NYHA class III or IV) (table 1) or cyanosis
Previous cardiovascular events including heart failure, a transient ischemic attack, stroke, or
arrhythmia
Left heart obstruction (mitral valve area of <2 cm2, aortic valve area of <1.5 cm2, or peak left
ventricular outflow gradient >30 mmHg).
Left ventricular systolic dysfunction (ejection fraction <40 percent)
One point was assigned to each finding. The overall rate of cardiac events (pulmonary edema,
arrhythmia requiring treatment, stroke, cardiac arrest, or death) was 13 percent, with 55 percent
occurring antepartum. There was high agreement between the rates that were observed in the two

studies and those predicted by the CARPREG risk index: 0 points (4 versus 5 percent); 1 point
(26 versus 27 percent); and 2 points (62 versus 75 percent).
The predictive value of the CARPREG risk index in women with congenital heart disease was
subsequently evaluated in 53 such women who had 90 pregnancies [16]. Adverse cardiac events
occurred in 25 percent of the pregnancies; the events included heart failure with pulmonary
edema, symptomatic arrhythmias, and the need for urgent invasive intervention. There were no
significant differences between the rates of adverse cardiac events and those predicted by the
CARPREG risk index: 0 points (12 versus 5 percent); 1 point (30 versus 27 percent); and 2
points (100 versus 75 percent). Overall risk assessment was improved, primarily in patients with
1 point, by incorporating the presence (high risk) or absence (low risk) of subpulmonary
ventricular dysfunction and/or severe pulmonary regurgitation.
Neither of these studies included patients with the Eisenmenger syndrome which, as noted
below, is associated with a high maternal mortality. (See 'Pulmonary hypertension' below.)
Individual risk factors The following risks and predictors for maternal or fetal complications
in women with congenital heart disease during pregnancy have been identified [2,3,13,14,17]:
Pulmonary hypertension (pulmonary vascular disease)
Maternal cyanosis
Poor maternal functional class
History of arrhythmia
Maternal anticoagulants
Pulmonary hypertension The most serious risk to the mother is pulmonary hypertension,
particularly Eisenmenger syndrome, which includes the additional risk of maternal cyanosis [1].
Pulmonary hypertension limits appropriate adaptive responses to the circulatory changes of
pregnancy and to the volatile changes during labor, delivery, and the postpartum period. Women
with Eisenmenger syndrome can develop potentially fatal hypoxemia during pregnancy or the
postpartum period.
The maternal mortality in women with Eisenmenger syndrome is as high as 50 percent [18]. A
study evaluating whether new advanced therapies had an impact on outcomes suggested that
there was a reduction in overall mortality from 38 to 25 percent compared to historical controls
but that the risk of maternal mortality remains prohibitively high [19]. In addition to appreciable
fetal morbidity and mortality, Eisenmenger patients poorly tolerate the hemodynamic changes
associated with pregnancy and delivery, and are particularly susceptible to complications such as
pre-eclampsia and postpartum hemorrhage. Preterm delivery and fetal growth restriction occur in
at least 50 percent of cases, with only 15 to 25 percent of pregnancies progressing to term. (See
"Medical management of Eisenmenger syndrome".)

The majority of maternal deaths occur during delivery or in the first week postpartum. A
gestational fall in systemic vascular resistance augments the right-to-left shunt through a
nonrestrictive ventricular septal defect. A sudden fall in systemic resistance can precipitate
intense cyanosis, and a sudden rise in resistance with bearing down during labor can abruptly
depress cardiac output and provoke fatal syncope.
Women with Eisenmenger syndrome should be counseled to avoid pregnancy because of high
maternal mortality, appreciable fetal risk, and increased risk of thromboembolism. When
pregnancy occurs, and termination is declined, heparin is recommended beginning at 20 weeks
by some groups. (See "Medical management of Eisenmenger syndrome".)
Cyanosis The arterial oxygen saturation before pregnancy is one of the most important
predictors of fetal and maternal outcomes [3,14,20,21]. The impact of maternal oxygen
saturation was illustrated in a series of 96 pregnancies in 44 women with cyanotic congenital
heart disease: only 43 percent of the pregnancies resulted in a live birth, 15 of which were
premature [2]. The likelihood of a live birth was much lower in women when their resting
arterial oxygen saturation was below 85 percent.
Adverse maternal outcomes were also reported in a series of 104 pregnancies in 74 women: 90
percent of mothers with cyanotic lesions had significant postpartum cardiac complications
compared to 19 percent with acyanotic lesions [3]. Arterial oxygen saturation above 80 percent
reduces this risk [21].
A related problem in patients with cyanotic congenital heart disease is erythrocytosis.
Phlebotomy is not indicated except in women with hematocrits 65 percent and hyperviscosity
symptoms such as headache, loss of concentration, fatigue, and myalgias. (See "Medical
management of cyanotic congenital heart disease in adults", section on 'Erythrocytosis and
anemia'.)
Maternal functional class Maternal morbidity and mortality vary directly with NYHA
functional classification (table 1) [14,22,23]. In 405 women with heart disease who experienced
519 pregnancies, 60 percent had rheumatic heart disease (which is now much less common in
developed countries), 31 percent had congenital heart disease, and the remaining patients had
arrhythmias, cardiomyopathy, or ischemic heart disease [22]. Eighty-six percent were NYHA
class I or II. The three maternal deaths occurred in women who were NYHA class III or IV.
Application of the NYHA classification is problematic in women with cyanotic malformations
and pulmonary vascular disease. Cardiac dyspnea, culminating in pulmonary edema, is a major
maternal risk but is more common in acquired heart disease involving the left ventricle or mitral
valve.
Aortic disease The hypervolemic and hyperdynamic circulatory state in pregnancy and/or
hormonal effects of pregnancy may contribute to risk of ascending aortic aneurysm or dissection
in patients with an anatomic predisposition (eg, Marfan syndrome, coarctation of the aorta, or
bicuspid aortic valve). These issues are discussed separately. (See "Pregnancy and Marfan
syndrome" and "Pregnancy in women with a bicuspid aortic valve".)

Natriuretic peptide levels An elevated N-terminal pro-B-type natriuretic peptide (NTproBNP) level (>128 pg/mL) at 20 weeks of gestation may be an independent risk factor for
cardiovascular events during pregnancy in women with congenital heart disease [24]. The
predictive value of NT-proBNP was evaluated in the ZAHARA II prospective multicenter
observational study, which included 213 pregnancies in 203 women with congenital heart
disease. The majority of the patients had mildly or moderately increased cardiovascular risk as
indicated by modified WHO class.
Adverse cardiovascular events occurred in 10.3 percent of 213 pregnancies
Independent predictors of adverse events included NT-proBNP level >128 pg/mL at 20 weeks
gestation, the presence of a mechanical valve, and subpulmonary ventricular dysfunction before
conception (odd ratios 10.6, 12.0, and 4.2, respectively).
The negative predictive value of NT-proBNP levels <128 pg/mL was 96.9 percent. The positive
predictive value of NT-proBNP levels >128 pg/mL was 18.3 percent.
Addition of NT-proBNP level >128 pg/mL at 20 weeks gestation to the two preconception
independent predictors significantly improved the area under the receiver operating curve (from
0.78 to 0.90).
Less evidence is available on the utility of B-type natriuretic peptide (BNP) levels for risk
stratification in pregnant women with congenital heart disease. Median plasma BNP levels
during normal pregnancy are approximately twofold those in nonpregnant controls [25], while
median BNP levels in a series of 66 pregnant women with congenital and acquired heart disease
were over twofold those in 12 pregnant women without heart disease [26]. Elevated BNP
(>100pg/mL) was found in all eight women who developed adverse cardiac events (defined as
arrhythmia requiring treatment, stroke, cardiac arrest or cardiac death, pulmonary edema, decline
in NYHA function class by at least two classes, or need for urgent invasive cardiac procedures
during pregnancy or within six months after delivery). Elevated BNP level detection preceded
the adverse event in four women. One-third of women with BNP >100pg/mL had an adverse
cardiac event.
FETAL RISK
Risk assessment The functional class of the mother, maternal cyanosis, and other factors such
as maternal medications expose the fetus to risks that threaten normal intrauterine growth and
development. (See "Use of anticoagulants during pregnancy and postpartum" and
"Supraventricular arrhythmias during pregnancy", section on 'Safety during pregnancy' and
"Management of heart failure during pregnancy" and "Management of heart failure during
pregnancy", section on 'Drugs'.)
A fetal risk assessment score has not been established. A registry study including 1321 pregnant
women with structural or ischemic heart disease (66 percent with congenital heart disease) found
strong associations between modified World Health Organization (WHO) class and offspring
outcome, especially preterm birth and birth weight [4]. A prospective study of 213 pregnancies in

203 women with congenital heart disease found that risk assessment using modified WHO
classification, ZAHARA offspring risk score, CARPREG offspring risk score, and number of
offspring predictors showed increases in class or risk score with increased offspring risk [10].
However none of these methods adequately predicted offspring events (area under the curve
[AUC] 0.6 for all).
Maternal functional class Maternal functional class (table 1) is a major determinant of fetal
mortality, with risk ranging from not raised above baseline risk for gravidas who are
asymptomatic to about 30 percent for gravidas with severe symptoms [1]. In pregnant women
with the Eisenmenger syndrome, for example, only 15 to 25 percent of pregnancies progress to
term. Spontaneous abortion is common, and there is appreciable perinatal mortality associated
with fetal growth restriction and preterm delivery [18]. (See "Medical management of
Eisenmenger syndrome".)
Maternal cyanosis Women with cyanotic congenital heart disease but no pulmonary
hypertension can go through pregnancy with a relatively low maternal risk, although fetal risk is
increased.
Maternal cyanosis compromises fetal growth and increases prematurity and fetal loss [27,28].
Fetal outcomes were reported in a review of 96 pregnancies in women with cyanotic congenital
heart disease [20]. The following findings were commented on:
Only 43 percent of pregnancies resulted in a live birth, 37 percent of which were premature
The rate of spontaneous abortion increased in parallel with maternal hypoxemia
The mean birth weight of full-term infants was 2575 grams compared to a normal term birth
weight of 3500 grams.
Even when pre-gestational cyanosis is mild, the incidence of fetal loss is not insignificant
because right-to-left shunts tend to increase during the course of pregnancy in response to the fall
in systemic vascular resistance.
Antepartum fetal monitoring should begin as soon as an increased risk of fetal demise is
identified and delivery for perinatal benefit would be considered if test results are abnormal. This
is generally between 26 and 32 weeks of gestation, with the specific time based on patient
specific factors. (See "Nonstress test and contraction stress test", section on 'Antepartum fetal
heart rate testing' and "Fetal growth restriction: Evaluation and management".)
Outcomes Overall fetal outcomes in mothers with congenital heart disease have been assessed
in a number of studies. The following observations illustrate the range of findings:
In retrospective reports of 2491 pregnancies in women with structural congenital heart disease,
miscarriage occurred in 15 percent, and 5 percent of women chose to terminate their pregnancies
[6]. Fetal mortality was 1.7 percent and perinatal mortality was 2.3 percent (compared to less
than 0.5 percent in the general population). The relatively high rate of premature births (16

percent) and the occurrence of congenital heart disease in the offspring are important variables.
(See 'Inheritance' below.)
The prospective Cardiac Disease in Pregnancy (CARPREG) study evaluated 562 women with
congenital or acquired cardiac disease or arrhythmias who had 617 pregnancies; 74 percent of
the pregnancies occurred in women with congenital heart disease [15]. Neonatal complications
occurred in 122 pregnancies (20 percent).
The major complications were premature birth in 105 pregnancies (17 percent), one-half of
which were due to preterm labor, and small for gestational age in 22 pregnancies (4 percent).
Less common complications included respiratory distress syndrome or intraventricular (cerebral)
hemorrhage as complications of premature birth in 17 pregnancies (3 percent overall, but 16
percent of premature births) and fetal or neonatal death (1 percent each). (See "Clinical
manifestations and diagnosis of intraventricular hemorrhage in the newborn" and "Management
and complications of intraventricular hemorrhage in the newborn".)
Risk factors for adverse outcomes included New York Heart Association class III or IV (table 1)
or cyanosis at the baseline prenatal visit, left heart obstruction (aortic and/or mitral stenosis),
smoking, multiple gestations, and the use of anticoagulants throughout pregnancy. (See "Use of
anticoagulants during pregnancy and postpartum".)
In the series of 90 pregnancies in women with congenital heart disease, spontaneous abortion
occurred in 12.2 percent, a rate that was not different from the 12 to 15 percent rate in women
without heart disease [16]. There were adverse neonatal outcomes in 28 percent of pregnancies,
including preterm delivery (21 percent), small for gestational age (8 percent), intrauterine fetal
demise (3 percent), intraventricular hemorrhage, and neonatal death (1.4 percent each). An
adverse neonatal outcome was independently predicted by a basal left ventricular outflow
gradient >30 mmHg (odds ratio 7.5).
Issues related to survival of premature infants are discussed separately. (See "Incidence and
mortality of the premature infant".)
Inheritance Offspring of women with congenital heart disease are at increased risk of
congenital heart defects. The risk of recurrent congenital heart disease varies with the specific
parental defect [6,29]. The data according to defect are presented separately. (See "Pregnancy in
women with congenital heart disease: Specific lesions".)
The largest experience is from a series of 6640 pregnancies in which one parent or sibling had
congenital heart disease. Recurrence in the fetus was detected by echocardiography [30] in 178
(2.7 percent), with recurrence of the same parental or sibling defect in approximately one-third.
The incidence was similar whether the affected index case was the father, the mother, or a
sibling.
These results are qualified because only pregnant women referred for fetal echocardiography
were included. Thus, the overall recurrence estimates may not be the same as those obtained
from population-based studies:

In a survey of 427 probands with congenital heart disease and their 837 children, the incidence
of a cardiac anomaly in the offspring was 10.7 percent [31].
In a collaborative study from Britain that evaluated 393 children of 727 parents, recurrence
occurred in 4.1 percent of offspring and 2.1 percent of siblings [29]. The risk of recurrence was
greater if the mother rather than the father had congenital heart disease (5.7 versus 2.2 percent).
Similar findings were found in the prospective CARPREG report cited above [15]. There were
432 live births in mothers with congenital heart disease but no recognized genetic syndromes.
Congenital heart disease was present in 7 percent.
There also appears to be variation among lesions in the rate of recurrence of the same lesion in
the parent or sibling, depending upon the specific defect. In the referral series cited above, the
following were the three most frequent recurrent lesions [30]:
Ventricular septal defect 55 percent concordance (17 of 31 recurrences)
Coarctation of the aorta 13 percent (two of 15 recurrences)
Hypoplastic left heart syndrome 33 percent (four of 12 recurrences)
There are familial syndromes associated with specific disorders, such as atrial septal defect in the
Holt-Oram syndrome, and a high rate of heritability with bicuspid aortic valve. (See
"Classification of atrial septal defects (ASDs), and clinical features and diagnosis of isolated
ASDs in children", section on 'Genetic disorders' and "Clinical manifestations and diagnosis of
bicuspid aortic valve in adults", section on 'Genetics'.)
PRECONCEPTION AND PRENATAL CARE
Preconception or initial evaluation When possible, women should receive preconception
assessment and counseling so that they are able to make informed pregnancy decisions. For
women who have not had preconception counseling, a complete risk evaluation should occur at
the first prenatal visit. Women with congenital heart disease should receive a preconception
evaluation by a cardiologist with expertise in pregnancy and congenital heart disease. Risk
assessment should involve a focused evaluation of the risk of pregnancy for the mother and baby.
Many women with heart disease are unaware of the risks of pregnancy, and patient education is
an important aspect of the preconception assessment [32]. (See "The preconception office visit"
and "Initial prenatal assessment and first trimester prenatal care".)
Preconception (or initial prenatal if the patient presents during pregnancy) evaluation should
include a detailed history, information on prior interventions (surgical and percutaneous),
symptom status, a complete physical exam, a 12-lead electrocardiogram, a transthoracic
echocardiogram, and an assessment of functional status (which may include exercise testing).
A transthoracic echocardiogram is important to determine the type and severity of cardiac
lesions, ventricular size and function, assessment of valve function, and pulmonary pressures.

Valvular or vascular gradients may increase during pregnancy and should be interpreted
accordingly. (See "Pregnancy and valve disease".)
Intervention prior to pregnancy One of the best ways to simplify medical management during
pregnancy is to perform indicated cardiac intervention (surgical or percutaneous) before
conception. Successful cardiac intervention may improve fertility, may enable the mother to
better tolerate the physiologic changes of pregnancy, can eliminate the fetal risk from maternal
cyanosis, and benefits the subsequent health of mother and child. Women with congenital heart
disease who have indications for cardiac intervention are generally advised to proceed with
intervention prior to pregnancy (eg, percutaneous aortic balloon valvuloplasty for asymptomatic
severe aortic stenosis to reduce maternal and fetal risks). However, the decision is much more
complex when a valve replacement is required and a detailed discussion on the risk and benefits
of bioprosthetic and mechanical valve options must be discussed with the patient. (See
"Pregnancy and valve disease", section on 'Interventions prior to pregnancy'.)
In contrast, cardiac surgery during pregnancy should be minimized or avoided [33-36]. The
maternal risks are about the same as those in nonpregnant women [33,34], but cardiopulmonary
bypass during pregnancy incurs risk for the fetus [33-37]. (See "Pregnancy and valve disease".)
Prenatal care Prenatal care should include patient education concerning the patients
responsibilities and the expected course of pregnancy and delivery. General recommendations for
initial prenatal assessment and for prenatal care are discussed separately. (See "Initial prenatal
assessment and first trimester prenatal care" and "Prenatal care (second and third trimesters)".)
The frequency of prenatal follow-up depends upon the severity of heart disease (see 'Modified
WHO classification' above):
For class I conditions, cardiology follow-up during pregnancy may be limited to one or two
visits
For class II conditions, follow-up every trimester is recommended
For class II to III conditions (depending on the individual), cardiology follow-up ranging from
every trimester to monthly is recommended.
For class III conditions, at least monthly or bimonthly cardiology follow-up during pregnancy
are recommended. Expert counseling is required and this may include consideration of
alternatives to pregnancy. Intensive specialist cardiac and obstetric monitoring are needed
throughout pregnancy, childbirth, and the puerperium.
For class IV conditions, pregnancy is contraindicated. If a woman presents with a lesion in this
class early in pregnancy, termination should be discussed. If pregnancy is terminated, reparative
surgery for high risk valve and/or aortic disease should be performed before another attempt at
pregnancy. If pregnancy continues, care as for class III with monthly or bimonthly cardiology
follow-up at a minimum.

Pulmonary edema and marked peripheral edema should be distinguished from the normal
physiologic edema of pregnancy. The peripheral edema typically seen during pregnancy does not
connote excess risk and is due to an increase in total exchangeable sodium and water and inferior
vena caval compression. Diuretics are not indicated and sodium restriction is not helpful for
physiologic edema. (See "Renal and urinary tract physiology in normal pregnancy".)
The value of antepartum oxygen in cyanotic women is questionable. There is little evidence that
oxygen benefits the mother, and there is no evidence that a favorable effect is exerted on a
growth-restricted fetus, even though administration of high levels of inspired oxygen may raise
the arterial oxygen saturation [38]. Antepartum oxygen administration is not recommended.
Other aspects of management of cyanotic congenital heart disease are discussed separately. (See
"Medical management of cyanotic congenital heart disease in adults".)
Hemoglobin levels decline modestly during healthy pregnancy so lower hemoglobin levels are
used to identify anemia during pregnancy. (See "Hematologic changes in pregnancy", section on
'Anemia'.) Routine administration of iron supplements beyond standard prenatal multivitamins
should be avoided, particularly in cyanotic patients. Patients with right-to-left shunts are
erythrocytotic because of a hypoxia-driven increase in erythropoietin production. (See "Medical
management of cyanotic congenital heart disease in adults", section on 'Erythrocytosis and
anemia'.)
Given the risk of thromboembolism during pregnancy, clinical follow-up should include careful
surveillance for signs and symptoms of venous thromboembolic disease. Hypercoagulability is of
particular concern for women at risk for thrombosis related to prosthetic heart valves, atrial
fibrillation, or previous thromboembolic events. Such patients are candidates for anticoagulation.
Considerations in choosing an anticoagulation regimen should include adverse fetal effects (eg,
warfarin embryopathy) in the first trimester, bleeding risk, and the risk of thrombosis on the
prosthetic valve. (See "Deep vein thrombosis and pulmonary embolism in pregnancy:
Prevention" and "Use of anticoagulants during pregnancy and postpartum" and "Management of
pregnant women with prosthetic heart valves".)
Exercise encroaches upon the limited reserve of the pregnant woman with heart disease.
Strenuous physical activity should therefore be avoided. (See "Exercise during pregnancy and
the postpartum period: Practical recommendations".)
Management of arrhythmias during pregnancy is discussed separately. (See "Supraventricular
arrhythmias during pregnancy" and "Ventricular arrhythmias during pregnancy".)
Fetal evaluation Women with congenital heart disease should be offered fetal
echocardiography in the 19th to 22nd week of pregnancy [12]. (See "Fetal cardiac abnormalities:
Screening, evaluation, and pregnancy management".)
PREGNANCY TERMINATION The option of pregnancy termination should be discussed
with women in whom gestation represents a major maternal or fetal risk. The first trimester is the
safest time for elective pregnancy termination, which should be performed in-hospital, rather

than in an outpatient facility, so that all emergency support services are available. The general
principles to consider when a termination procedure is planned include:
The patient's cardiologist, an anesthesiologist, and the obstetrician/gynecologist who will
perform the procedure should confer prior to the termination.
Endocarditis prophylaxis is not consistently recommended [39], but treatment should be
individualized. (See "Antimicrobial prophylaxis for bacterial endocarditis".) Gynecologists
routinely advise antibiotic prophylaxis to prevent postabortal endometritis, which occurs in 5 to
20 percent of women not given antibiotics [39-41]. (See "Overview of pregnancy termination",
section on 'Antibiotic prophylaxis'.)
Dilatation and evacuation can be performed safely in both the first and second trimesters. (See
"Overview of pregnancy termination", section on 'Choice of technique'.)
If surgical evacuation is not feasible in the second trimester, protocols for second trimester
induction abortion typically involve a prostaglandin (PG), usually misoprostol (PGE1), and may
utilize mifepristone (an antiprogestin). Misoprostol and mifepristone appear to have minimal
cardiovascular effects [42-45]. (See "Overview of pregnancy termination".)
Older pregnancy termination techniques (PGE2, PGF, saline), which are no longer used, have
been associated with serious adverse cardiac effects.
MANAGEMENT OF LABOR, DELIVERY, AND THE POSTPARTUM PERIOD
Endocarditis The rate of endocarditis was evaluated in retrospective review published from
1985 to 2006 that described the outcomes of 2491 pregnancies in women with structural
congenital heart disease [6]. The rate of endocarditis was 0.5 percent during 1372 completed
pregnancies among women with congenital heart disease [6]. However, other puerperal
infections and antibiotic prophylaxis for this population were not disclosed.
High rates of both maternal and fetal mortality (22 and 15 percent, respectively, in a review of 67
cases) have been reported for endocarditis during pregnancy [46]. However, most of the data
came from individual case reports and are subject to selection bias.
Guidelines for endocarditis prophylaxis are discussed separately. (See "Antimicrobial
prophylaxis for bacterial endocarditis", section on 'Vaginal or cesarean delivery'.)
Labor In women with functionally mild unrepaired congenital heart disease, and in women
who have undergone successful cardiac surgery without major residua, the management of labor
and delivery is the same as for normal pregnant women, except for potential increase in risk of
infective endocarditis.
Pregnant women with unrepaired or postoperative congenital heart disease who are considered
functionally normal are allowed to go into spontaneous labor. However, when there are concerns
about the functional adequacy of the heart and circulation, labor should be induced under

controlled conditions if there are no obstetrical contraindications to vaginal delivery. The timing
of induction is individualized, taking into account the gravida's cardiac status, inducibility of
the cervix, and probability fetal lung maturity as determined by gestational age and/or
amniocentesis. Long inductions in women with an unfavorable cervix should be avoided.
Induction of labor in gravidas with a favorable cervix usually requires only oxytocin
administration and artificial rupture of the membranes. An unfavorable cervix can be ripened by
a variety of methods. The softened, dilated cervix is more responsive to the subsequent induction
of labor. (See "Techniques for ripening the unfavorable cervix prior to induction" and "Induction
of labor".)
The method of choice for cervical ripening may vary with the clinical setting. Mechanical
methods are preferable in the patient with cyanosis where a drop in systemic vascular resistance
and/or blood pressure would be detrimental. If a mechanical method is not possible, we favor
misoprostol.
A mechanical method, such as a Foley catheter, is favored due to concerns of potential adverse
effects from pharmacologic agents for cervical ripening. However, there is a theoretical risk of
infection from introduction of a foreign body. While there is no categorical contraindication to
misoprostol or dinoprostone, there is a theoretical risk of coronary vasospasm and a low risk of
arrhythmias. Dinoprostone appears to have more profound effects on blood pressure and is
contraindicated in the presence of cardiovascular disease.
During labor, the gravida should be in a lateral decubitus position to minimize uterine
compression of the abdominal aorta and inferior vena cava, and thus to attenuate the
hemodynamic fluctuations associated with major uterine contractions in the supine position. The
fetal head should be allowed to descend to the perineum in response to the forces of labor,
unassisted by maternal pushing, in order to avoid the undesirable circulatory effects of the
Valsalva maneuver. Delivery can then be assisted by low forceps or vacuum extraction. (See
"Maternal cardiovascular and hemodynamic adaptations to pregnancy".)
Anesthesia and hemodynamic monitoring Anesthesia and hemodynamic monitoring for labor
and delivery for high-risk cardiovascular disease is discussed separately. (See "Anesthesia for
labor and delivery in high-risk cardiovascular disease: General considerations" and "Anesthesia
for labor and delivery in high-risk cardiovascular disease: Specific lesions".)
Fetal monitoring Continuous electronic fetal heart rate monitoring is recommended during
labor. (See "Intrapartum fetal heart rate assessment".) Reductions in uterine blood flow and
placental oxygen delivery typically occur during uterine contractions, but the fetus usually
extracts enough oxygen to meet its needs. Fetal hypoxemia may occur with complications such
as abruptio placentae, cord compression, maternal hemodynamic instability, or fetal growth
restriction.
Oxygen therapy Oxygen is often administered during labor, especially in cyanotic women.
However, maternal benefit has not been demonstrated, and it is not clear whether and to what

extent maternal oxygen administration increases fetal PaO2. Transcutaneous fingertip oximetry
is sufficient for monitoring maternal oxygenation.
Preterm labor Preterm labor is a major concern, especially in cyanotic pregnant women whose
fetuses are likely to be immature. Pharmacologic inhibition of uterine contractions (tocolytic
therapy) may involve indomethacin, nifedipine, a beta adrenergic agonist, or atosiban, an
oxytocin receptor antagonist. Potential complications of beta adrenergic agonist therapy include
volume expansion and increased maternal heart rate, which can result in heart failure [47].
Nifedipine or indomethacin are generally the preferred agents. Nifedipine may be harmful in
patients with significant aortic stenosis or cyanotic congenital heart disease. (See "Inhibition of
acute preterm labor".)
Role of cesarean delivery For the pregnant woman with a functionally significant congenital
cardiac malformation, regardless of whether or not there has been surgical repair, the anticipation
and management of labor, delivery, and the puerperium are crucial if risk is to be minimized.
There is consensus that cesarean delivery should be reserved for obstetrical indications, such as
breech presentation, failure to progress, placenta previa, or some abnormal fetal heart rate
patterns. (See "Cesarean delivery: Preoperative issues", section on 'Indications and
contraindications'.)
The risks of cesarean delivery in such women include:
General anesthesia that incurs the risk of hemodynamic instability associated with intubation
and the anesthetic agent.
Blood loss of at least twice as with vaginal delivery
Increased risks of wound and uterine infections and postoperative thrombophlebitis
Incisional bleeding in patients on anticoagulants and in cyanotic gravidas who have inherent
coagulation defects
Endocarditis Routine antimicrobial prophylaxis for bacterial endocarditis is not recommended
in most women with congenital heart disease during pregnancy and delivery [48,49]. However,
we agree with the suggestion in the 2008 American College of Cardiology/American Heart
Association guidelines for the management of adults with congenital heart disease that in select
high-risk patients (such as those with completely repaired congenital heart defects with
prosthetic material or device during the first six months after the procedure, unrepaired cyanotic
congenital heart disease [including those with palliative shunts and conduits], repaired congenital
heart disease with residual defects at the site or adjacent to the site of the prosthetic device, or
prosthetic heart valves); it is reasonable to consider antibiotic prophylaxis before vaginal
delivery at the time of membrane rupture [50]. (See "Antimicrobial prophylaxis for bacterial
endocarditis", section on 'Vaginal or cesarean delivery'.)
The rate of endocarditis was evaluated in a retrospective review published from 1985 to 2006
that described the outcomes of 2491 pregnancies in women with structural congenital heart

disease [6]. The rate of endocarditis was 0.5 percent during 1372 completed pregnancies among
women with congenital heart disease [6]. However, other puerperal infections and antibiotic
prophylaxis for this population were not disclosed.
High rates of both maternal and fetal mortality (22 and 15 percent, respectively, in a review of 67
cases) have been reported for endocarditis during pregnancy [46]. However, most of the data
came from individual case reports and are subject to selection bias.
Postpartum care After expulsion of the placenta, bleeding is reduced by uterine massage and
administration of intravenous oxytocin, which should be infused slowly (less than 2 U/min) to
avoid hypotension. Meticulous leg care, elastic support stockings, and early ambulation are
important preventive measures that reduce the risk of postpartum thromboembolism.
In cyanotic gravidas, heparin reinforces the intrinsic hemostatic defect(s) and may result in
dangerous and even fatal hemorrhage [1]. Accordingly, heparin should be used with caution and
restricted to patients identified to be high risk for venous thromboembolism. This does not apply
to patients with prosthetic heart valves in whom anticoagulation is required. (See "Management
of pregnant women with prosthetic heart valves", section on 'Anticoagulation during pregnancy
for women with mechanical heart valves'.)
Breastfeeding Breastfeeding a newborn is fatiguing and is associated with a low risk of
mastitis with bacteremia. As a result, some women with symptomatic congenital heart disease
who might otherwise prefer breastfeeding choose to bottle-feed.
FERTILITY There are two main fertility issues in women with congenital heart disease:
menstrual patterns and contraception.
Ovarian function Ovarian function in women with congenital heart disease is an important
concern. Whether or not ovarian function varies with the type of heart defect, and how reparative
surgery affects ovarian function, remain largely unknown. In a report of 98 women (mean age
33), those who were cyanotic had a delay in onset of menstruation (by about one year) and an
increased incidence of abnormally short or long cycle lengths. Women with acyanotic congenital
heart disease had menstrual patterns similar to normal controls [51].
Abnormal menstrual patterns in cyanotic women are believed to represent a chronic anovulatory
state related to dysfunction of the hypopituitary-ovarian axis or to abnormal uterine hemostasis
in response to chronic hypoxemia and erythrocytosis. It is not known if there is an age beyond
which reparative cardiac surgery is unlikely to be followed by normal ovarian function,
especially in cyanotic women. For women with subfertility due to anovulation, standard
treatment such as clomophine is not contraindicated.
Chronic anovulation predisposes to endometrial hyperplasia and carcinoma. (See "Endometrial
carcinoma: Epidemiology and risk factors", section on 'Chronic anovulation'.)
Contraception Women with congenital heart disease should be given information about
contraception and the potential risks associated with pregnancy [32]. The same contraceptive

methods available to normal healthy women are generally applicable to those with congenital
heart disease [52]. However, women with congenital heart disease often do not know the most
appropriate method of contraception or are given incorrect advice [53]. Factors to consider are
whether a reversible contraceptive method or sterilization is preferable, the efficacy of various
methods, patient-specific factors that affect compliance (table 2), and medical issues that affect
the risk-benefit profile of various methods. The Centers for Disease Control have published
guidelines for estimating the risk versus benefit of use of contraception in women with medical
disorders (table 3). (See "Overview of contraception".)
Briefly:
Barrier methods include condoms for males or the diaphragm with spermicide for females.
Although these methods are generally less effective than other techniques, they pose virtually no
risk of complications. In women needing to avoid pregnancy due to their cardiac status, these
methods should be avoided due to their high failure rate (18 percent for male condom).
An intrauterine device (IUD) is an option for acyanotic or mildly cyanotic women who want a
reversible method of contraception and are at low risk of acquiring a sexually transmitted
infection. (See "Intrauterine contraception (IUD): Overview".)
The higher dose levonorgestrel-releasing intrauterine device (Mirena) has the advantage of
reducing menstrual blood loss by 40 to 50 percent but may induce amenorrhea. It is effective for
five years. A lower dose device is also available and is effective for three years. Systemic
hormonal effects are minimal.
A copper containing IUD has the advantage of lasting for at least 10 years, hence, less need to
change the IUD and thus minimizing risk of infection. It will not interfere with medication
metabolism, it causes no hormonal side effects, and has few contraindications related to medical
conditions. It is not recommended in women who are anemic or cyanotic with hematocrit levels
above 55 percent because intrinsic hemostatic defects increase the risk of excessive menstrual
bleeding, which is more common with the copper containing IUD than the levonorgestrelreleasing IUD.
Combination estrogen-progestin contraceptives (pill, patch, ring) can be used in women at low
risk for thromboembolic complications, ie, no pulmonary hypertension and no surgical baffles,
such as those with uncomplicated valvular disease (table 3). The usual ethinyl estradiol dose is
20 to 35 mcg. The 20 mcg dose may have fewer thromboembolic complications. (See "Overview
of the use of estrogen-progestin contraceptives" and "Risks and side effects associated with
estrogen-progestin contraceptives".)
Progestin-only contraception can be given, using the levonorgestrel-releasing IUD, injections
of depot medroxyprogesterone (eg, Depo-Provera), pills (eg, Micronor tablets, Nor-QD, or
generics), or the etonogestrel implant (Nexplanon). Depo-Provera is inappropriate for patients
with heart failure because of its tendency to cause fluid retention. (See "Overview of
contraception", section on 'Issues to consider when beginning hormonal contraception'.)

Tubal occlusion is not reversible but can be accomplished safely, even in relatively high-risk
women. The risk may be even lower with the minimally invasive hysteroscopic technique [54].
(See "Hysteroscopic sterilization" and "Surgical sterilization of women".) Vasectomy for the
male is an equally efficacious option that incurs no maternal risk.
Recommendations for appropriate contraception for women with heart disease, including
congenital heart disease, are reviewed in an American Heart Disease guideline statement on best
practices in managing the transition to adulthood for adolescents with congenital heart disease
[55].
Recommendations For women with cyanotic congenital heart disease and pulmonary vascular
disease, tubal ligation, etonogesterel implant (Implanon), or an intrauterine device are the safest
and most effect options for preventing pregnancy. (See "Etonogestrel contraceptive implant" and
"Surgical sterilization of women" and "Intrauterine contraception (IUD): Overview" and
"Overview of contraception".)
Depo-Provera is inappropriate for patients with heart failure because of its tendency to cause
fluid retention. Oral contraceptives containing 20 to 35 mcg of ethinyl estradiol are considered
safe in patients with a low thrombogenic potential and a low failure rate, provided that no dose is
missed and provided the patient does not smoke [56].
SUMMARY
Improved surgical repair options have resulted in most women with congenital heart disease
surviving to bear children. Despite these advances, congenital heart disease remains an important
cause of maternal mortality and morbidity. (See 'Epidemiology' above.)
Normal alterations in circulatory and respiratory physiology during pregnancy can have
deleterious effects on the mother with congenital heart disease and on her developing fetus. (See
'Hemodynamic changes' above.)
Pulmonary edema and marked peripheral edema should be distinguished from the normal
physiologic peripheral edema of pregnancy. The edema typically seen during pregnancy does not
connote excess risk and is due to an increase in total exchangeable sodium and water and inferior
vena caval compression. Diuretics are not indicated and sodium restriction is not helpful for
physiologic edema. (See 'Prenatal care' above.)
Hypercoagulability is a particular concern in women at risk for thrombosis related to prosthetic
heart valves, atrial fibrillation, or previous thromboembolic events or intracardiac shunts. Such
patients are candidates for anticoagulation. Considerations in choosing an anticoagulation
regimen should include adverse fetal effects (eg, warfarin embryopathy) in the first trimester,
bleeding risk, and the risk of thrombosis on the prosthetic valve. (See 'Risk of
thromboembolism' above and 'Prenatal care' above and "Use of anticoagulants during pregnancy
and postpartum" and "Management of pregnant women with prosthetic heart valves".)

We favor using the modified World Health Organization (WHO) risk classification for maternal
risk assessment. (See 'Overview' above.)
Modified WHO classification of risk according to maternal cardiovascular condition provides
guidance regarding the frequency of prenatal cardiology and obstetric follow-up. (See 'Modified
WHO classification' above and 'Prenatal care' above.)
The following maternal conditions pose very high maternal and/or fetal risk during pregnancy:
significant pulmonary arterial hypertension of any cause, severe mitral stenosis, severe aortic
stenosis, bicuspid aortic valve with ascending aorta diameter >50 mm, Marfan syndrome with
aorta dilated >45 mm, severe systemic ventricular systolic dysfunction (left ventricular ejection
fraction <30 percent, New York Heart Association III to IV), and native severe coarctation. (See
'Modified WHO classification' above.)
Impaired maternal functional class, maternal cyanosis, and maternal medications expose the
fetus to risks that threaten normal intrauterine growth and development. (See 'Fetal risk' above.)
The option of pregnancy termination should be discussed with women in whom gestation
represents a major maternal or fetal risk. (See 'Pregnancy termination' above.)
Counseling regarding contraception should include consideration of whether a reversible
contraceptive method or sterilization is preferable, the efficacy of various methods, patientspecific factors that affect compliance, and medical issues that affect the risk-to-benefit profile of
various methods. (See 'Contraception' above.)
For women with congenital heart disease, cesarean delivery should be reserved for obstetrical
indications, such as cephalopelvic disproportion, placenta previa, or preterm labor in a gravida
on oral anticoagulants. (See 'Role of cesarean delivery' above.)
Offspring of women with congenital heart disease are at increased risk of congenital heart
defects. (See 'Inheritance' above.)
ACKNOWLEDGMENT We are saddened by the death of Joseph K. Perloff, MD, who passed
away in August 2014. The UpToDate editorial staff wishes to acknowledge Dr. Perloffs past
work as an author and also wishes to thank Dr. Thomas P. Graham for his contributions as a
Section Editor to previous versions of this topic review.
Use of UpToDate is subject to the Subscription and License Agreement.

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