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Maternal Valvular Heart Disease in Pregnancy

ARTICLE in OBSTETRICAL & GYNECOLOGICAL SURVEY SEPTEMBER 2011
Impact Factor: 1.86 DOI: 10.1097/OGX.0b013e318238605d Source: PubMed

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4 AUTHORS:
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Jeffrey A Kuller

University of California, San Diego

Duke University

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Volume 66, Number 9

OBSTETRICAL AND GYNECOLOGICAL SURVEY
Copyright 2011
by Lippincott Williams & Wilkins

CME REVIEWARTICLE

25

CHIEF EDITORS NOTE: This article is part of a series of continuing education activities in this Journal through which a total
of 36 AMA/PRA Category 1 CreditsTM can be earned in 2011. Instructions for how CME credits can be earned appear on the
last page of the Table of Contents.

Maternal Valvular Heart Disease

in Pregnancy
Hilary A. Roeder, MD,* Jeffrey A. Kuller, MD, Piers C. A. Barker, MD,
and Andra H. James, MD, MPH
*Fellow, Department of Reproductive Medicine, Division of Perinatology, University of California San Diego,
San Diego, CA; Professor, Associate Professor, Department of Obstetrics and Gynecology, Division of
Maternal-Fetal Medicine, Duke University Medical Center, Durham, NC; and Associate Professor,
Department of Pediatrics, Obstetrics and Gynecology, and Division of Cardiology, Duke University Medical
Center Durham, NC
Valvular heart disease is common in pregnancy. Maternal physiology changes significantly during
gestation with substantial increases in cardiac output and blood volume; this can cause unmasking or
worsening of cardiac disease. Acquired valvular lesions most frequently arise from rheumatic fever,
especially in patients who have emigrated from developing nations. Congenital lesions are also
encountered. The most common conditions seen, mitral stenosis and regurgitation and aortic stenosis and
regurgitation, each require a specific evaluation and management and are associated with their own set of
possible complications. Patients with prosthetic valves require anticoagulation, and maternal and fetal risks
and benefits must be carefully weighed. Patients with heart disease should be meticulously managed
preconceptionally up to the postpartum period by maternal-fetal medicine specialists, obstetricians, cardiologists, and anesthesiologists using a multi-disciplinary approach to their cardiac conditions.
Target Audience: Obstetricians & Gynecologists and Family Physicians
Learning Objectives: After the completing the CME activity, physicians should be better able to
examine the epidemiology of valvular heart disease in pregnancy, categorize key physiologic parameters
that change in the cardiovascular system during pregnancy, classify the pathophysiology of valvular
lesions, and evaluate the general principles of maternal and fetal management for cardiac disease.

Clinical Case Scenario

A 35-year-old nulligravida who recently emigrated
from Egypt presents for preconceptional counseling.
She suffered from rheumatic fever at the age of 10,
and subsequently developed mitral, aortic, and tricuspid valvular stenosis. In 2006, she underwent
mechanical valve replacements of her mitral and
aortic valves and additionally underwent tricuspid
The authors, faculty and staff in a position to control the content
of this CME activity and their spouses/life partners (if any) have
disclosed that they have no financial relationships with, or financial
interest in, any commercial organizations pertaining to this educational activity.
Correspondence requests to: Hilary A. Roeder, MD, UC San
Diego Health System, 200 W Arbor Drive-MC-8433, San Diego,
CA 92103. E-mail: haroeder@ucsd.edu.

valvuloplasty. She is currently under the care of a

cardiologist, and although she feels well, the findings
from her most recent echocardiogram demonstrate
dilated atria, and from electrocardiogram (ECG)
show atrial fibrillation. Her current medications are
warfarin, digoxin, amiodarone, and furosemide. She
has multiple questions about how her heart disease is
going to affect the pregnancy and how her pregnancy
is going to affect her heart disease. Should she get
pregnant? What are her risks of morbidity and mortality? Which medications including anticoagulation
she should be taking? When will maternal and fetal
surveillance be initiated?
This article introduces the reader to the problem of
heart disease in pregnant women. It will address the

www.obgynsurvey.com | 561

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Obstetrical and Gynecological Survey

epidemiology of valvular heart disease in pregnancy,

review key physiologic parameters that change in the
cardiovascular system during pregnancy, characterize the pathophysiology of valvular lesions, identify
highest-risk cardiovascular conditions, stratify lesions into general morbidity and mortality risk
categories, and describe the general principles of
maternal and fetal management for cardiac disease.
Epidemiology
Maternal heart disease complicates approximately
4% of all pregnancies.1 However, it accounts for 10%
to 25% of maternal mortality.2 In North America,
congenital heart disease is the most common cardiac
problem in pregnancy,3 and its high prevalence is due
in part to advances in corrective procedures available
to children born with lesions such as tetralogy of
Fallot, transposition of the great vessels, septal defects, and others. As a result, more women with
congenital heart disease are reaching reproductive
age and are bearing children of their own.4 The
incidence of acquired heart disease such as cardiomyopathy and coronary artery disease is also increasing as a larger proportion of women are delaying
pregnancy.5 Risk factors in women of advancing
maternal age such as type 2 diabetes mellitus, hypertension, and obesity can increase the risk of coronary
events.3 The distribution of heart disease has evolved
recently; before the 1980s, rheumatic heart disease
accounted for the majority of heart-related complications in pregnancy.6 Women with valvular heart
disease still provide a challenge in management be-

fore, during, and after pregnancy, and will be the

focus of this review.
Valvular Lesions
Valvular heart disease can be either congenital or
acquired. Valvular heart disease in pregnant women
is often due to rheumatic heart disease, but not all
valvular heart lesions affect patients with the same
severity. Some have low maternal and fetal risks, and
others pose more problems (Table 1).7 In general,
those asymptomatic and mild lesions associated with
The New York Heart Association (NYHA) class I/II
(Table 2) are considered low risk. On the contrary,
those with NYHA class III/IV, or otherwise symptomatic lesions, are considered high risk.
Rheumatic Heart Disease
Rheumatic heart disease accounts for greater than
90% of cardiac disease in pregnancy worldwide.8 In
the United States, rheumatic heart disease was previously the number one cause of maternal cardiac
complications in pregnancy.3 During the 1940s, the
incidence of rheumatic fever was greater than
200,000 cases per year in the United States, but this
is now a rare condition due to the advent and use of
penicillin. However, in Southeast Asia, Africa, India,
the Middle East, and parts of Australia and New
Zealand, rheumatic heart disease is still common.9
Approximately 3% of patients with untreated group
A streptococcal pharyngitis will subsequently develop rheumatic fever.9 The pathogenesis is largely

TABLE 1
Classification of valvular heart lesions according to maternal and fetal risks
Low Maternal and Fetal Risks
Asymptomatic aortic stenosis with a low
mean outflow gradient (50 mmHg);
normal LV systolic function
Aortic regurgitation, NYHA Class I or II with
normal LV function
Mitral regurgitation, NYHA Class I or II, with
normal LV function
Mitral valve prolapse with none to moderate
mitral regurgitation, normal LV function
Mild to moderate mitral stenosis, no
pulmonary hypertension
Mild to moderate pulmonary valve stenosis

High Maternal and Fetal Risks

High Maternal Risk

Severe aortic stenosis with or without

symptoms

Ejection fraction 40%

Aortic regurgitation, NYHA Class III or IV

Previous heart failure

Mitral stenosis, NYHA Class II, III, or IV

Previous stroke or transient ischemic

attack

Mitral regurgitation, NYHA Class III or IV

Aortic or mitral valve disease with
pulmonary hypertension
Aortic or mitral valve disease with LV
dysfunction
Maternal cyanosis
Reduced functional status, NYHA class III
or IV

NYHA indicates New York Heart Association; LV, left ventricular.

Maternal Valvular Heart Disease in Pregnancy Y CME Review Article

TABLE 2
New York Heart Association functional classification system
Class
Class I (mild)

Class II (mild)

Class III (moderate)

Class IV (severe)

Description
No limitations of physical activity. Ordinary
physical activity does not precipitate
cardiovascular symptoms such as
dyspnea, angina, fatigue, or palpitations
Slight limitation of physical activity.
Ordinary physical activity will precipitate
cardiovascular symptoms. Patients are
comfortable at rest
Less than ordinary physical activity
precipitates symptoms that markedly
limit activity. Patients are comfortable at
rest
Patients have discomfort with any physical
activity. Symptoms are present at rest

unknown, but it is hypothesized that the streptococcus toxin plays a role or that a bacterial antigen
cross-reacts with proteins in the endocardium.9 The
histologic diagnosis is made by the detection of Aschoff bodies, granulomatous inflammatory clusters
consisting of fibrinoid degenerating connective tissue
combined with lymphocytes and giant cells.10 These
areas resolve to form scar tissue on the valvular
endocardium, and it is thought that patients with
multiple episodes of rheumatic fever can develop a
chronic valvular deformity.9

REVIEW OF CARDIAC PHYSIOLOGY

IN PREGNANCY
There are 4 principal physiologic changes that occur during pregnancy and have a major impact on
pregnant women with heart disease.3
1. Increased intravascular volume. If cardiac output
(CO) is limited by heart failure, valvular lesions,
or coronary artery disease, the 50% increase in
intravascular volume associated with pregnancy
will be poorly tolerated and could lead to congestive heart failure or ischemia.
2. Decreased systemic vascular resistance (SVR).
The reduction in afterload mediated during
pregnancy by rising placental steroid levels can
lead to right to left shunting in patients with
congenital heart disease or difficult adaptation
to pregnancy in those with some valvular diseases. In contrast, this change also enables
some women with specific valvular lesions to
better tolerate pregnancy as will be discussed.

563

3. Increased hypercoagulability. Patients with artificial valves or arrhythmias necessitating anticoagulation are even more sensitive to their
propensity to clot due to pregnancy-mediated
resistance to activated protein C.11 Additionally, protein S decreases,12 and factors I, II, V,
VII, VIII, X, and XII increase.12,13 Optimal anticoagulation with warfarin, very important in
women with cardiac valvular prostheses, can
have adverse fetal consequences.
4. Marked fluctuations in CO. Throughout the antepartum period, and especially during the intrapartum period, there are significant changes in CO
with uterine activity. Specifically, with each uterine contraction, there is an autotransfusion from
the placenta into the maternal systemic circulation. This leads to an increase in venous return,
and is detrimental for patients with cardiac lesions
dependent on preload, such as mitral/aortic stenosis or pulmonary hypertension.
Antepartum Physiology
A series of compensative mechanisms are activated
from approximately 5 weeks gestation to maximize
oxygen delivery to maternal and fetal tissues.14 CO
increases progressively during pregnancy, reaching
30% to 50% above baseline by 28 weeks.15 Heart rate
(HR) increases approximately 10 to 15 beats per
minute during the course of gestation, and stroke
volume increases by 20% to 30%.16
SVR decreases progressively, and is reduced by
approximately 20% by 24 weeks.17 This is mainly
due to the effects of progesterone.18 Mean arterial
pressure (MAP) also decreases until approximately
28 weeks, then gradually returns to baseline by
term.19 Considering the components of MAP, diastolic blood pressure decreases until the end of the
second trimester, whereas systolic blood pressure
changes only minimally during gestation.
Intrapartum Physiology
During parturition, CO and MAP both increase. In
fact, there is approximately a 50% increase in CO
from about 7 L/min at term to 11 L/min at the end of
the first stage of labor.20 Positional changes also can
affect CO. The lateral recumbent position (displacing
the uterus to the left and off the inferior vena cava)
will increase CO, whereas the supine position will
decrease CO due to compression of the inferior vena
cava by the uterus.21 Pain and anxiety can increase

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Obstetrical and Gynecological Survey

CO, HR, and MAP. As discussed previously, contractions will result in an autotransfusion of approximately 500 mL of blood from the uterus which
increases preload and cardiac stroke volume.20

quela of rheumatic fever, with less than 1% of mitral

stenosis in pregnancy due to congenital anomalies or
endocarditis.20 This cardiac lesion is often asymptomatic until pregnancy, where physiologic changes
can unmask its presence.8

Postpartum Physiology
In the postpartum period, CO and stroke volume
again increase by approximately 50% and 60%70%,
respectively, 1 hour after delivery.22 This most likely
results from autotransfusion of the remaining uteroplacental blood into maternal systemic circulation
with uterine involution. There will also be a reflex
bradycardia as well as a postpartum diuresis of 2 to 5
L occurring 2 to 7 days after delivery.22 Cardiac
status returns gradually to baseline by 2 to 6 weeks
postpartum.23
Valvular Anatomy and Physiology
There are significant changes to the valves themselves during pregnancy that can be depicted by
echocardiography. The aortic root dimension and the
mitral and tricuspid annuli increase in size, yet the
flow velocity across the aortic valve is only minimally increased.3 Mild valvular regurgitation occurs
normally during gestation, and the mitral, tricuspid,
and pulmonic valves will demonstrate some insufficiency in up to 28%, 94%, and 94% of pregnancies,
respectively.24
Common complaints due to the physiologic
changes in pregnancy can mimic valvular disease,
and it is important for the attentive clinician to separate normal from pathologic. Many pregnant
women suffer from dyspnea; this is not cause for
concern if it begins before 20 weeks gestation so
long as it does not interfere with activities of daily
living or occur at rest.25 Normal changes in pregnancy include decreased exercise tolerance, peripheral edema, mildly elevated HR, and a systolic
ejection flow murmur less than III/VI.20 In contrast, the
provider should be alerted if a patient complains of
dyspnea that worsens with gestational age, or occurs at
rest. Angina, syncope, and most diastolic murmurs are
not normal and mandate further evaluation.26
VALVULAR HEART
DISEASE PATHOLOGY
Mitral Stenosis
Mitral stenosis is the most common valvular heart
disease in pregnancy.27 It is virtually always a se-

Pathophysiology
A stenotic mitral valve restricts flow from the left
atrium to the left ventricle, creating a pressure gradient. Since flow across the valve is fixed due to the
stenosis, the progressive increase in preload that occurs with advancing gestation results in an increase
in the left atrial to left ventricular pressure gradient.
This leads to an elevated left atrial pressure, which is
transmitted backward into the pulmonary vasculature, and can result in pulmonary edema or, in extreme cases, pulmonary hypertension.9 Chronically
elevated volumes of blood in the left atrium can
cause the chamber to dilate and disrupt the conduction system, leading to arrhythmias.
Evaluation
A baseline echocardiogram to determine the severity of the lesion and assess for the presence of an
enlarged left atrium should be obtained preconceptionally or in early pregnancy and repeated if symptoms worsen. A normal mitral valve is 4 to 6 cm2 in
area; moderate stenosis is considered 1.0 to 1.5 cm2;
and severe mitral stenosis is an area less than 1.0
cm2.8 Echocardiographic imaging can also demonstrate the classic features of the rheumatic mitral
valve, including fibrous thickening and calcification
of the valve leaflets, fusion of the commissures, and
thickening/shortening of the chordae tendineae.9 An
ECG should also be performed to rule out atrial
fibrillation, left atrial enlargement, and right ventricular hypertrophy that would suggest the presence of
pulmonary hypertension.
Complications
In patients with significant mitral stenosis, the first
episode of pulmonary edema occurs in approximately
60% of patients by around 30 weeks gestation, near the
peak of vascular volume increase; 20% of these occur
in the presence of atrial tachyarrhythmias.28 Atrial
fibrillation and supraventricular tachycardias can
cause the CO to decrease; this is especially concerning when the patient has a rapid ventricular response
to the atrial arrhythmia, resulting in decreased ventricular diastolic filling.20 Finally, a left atrial clot can

Maternal Valvular Heart Disease in Pregnancy Y CME Review Article

occur from stagnation of blood flow in the dilated left

atrium. If any clot dislodges and embolizes, a stroke
can result.
Management
Most cases of mitral stenosis can be managed conservatively with medications; however, catheter balloon
valvuloplasty is an option in pregnancy for temporarily
alleviating severe symptoms.20,29 In the antepartum period, it is of ultimate importance that tachycardia in the
patient with mitral stenosis be prevented so that diastolic flow across the stenotic mitral valve can be maximized. If pulmonary edema does occur, diuretics are
appropriate for use. If atrial fibrillation occurs, treating
with both anti-arrhythmic agents and anticoagulation is
necessary. Synchronized cardioversion may also be indicated in the setting of maternal hemodynamic instability or a rapid ventricular response not adequately
controlled with medications.30
During labor and delivery, keeping the HR less than
90 to 100 beats per minute is best accomplished by
actively managing pain and through the use of beta
blockers such as esmolol.20 Terbutaline is contraindicated because of its effect on HR elevation. Maintaining
preload during parturition is necessary to keep CO
consistent, although care must be taken to avoid fluid
overload. Hemodynamic monitoring may be recommended in severe cases of mitral stenosis. Cesarean
delivery should be reserved for obstetric indications.31
A passive second stage avoids the changes in intrathoracic pressure and the negative effects on venous return
and preload associated with Valsalva maneuvers.32
Mitral Regurgitation
Mitral regurgitation is a condition in which the
incompetent mitral valve allows a portion of the left
ventricular stroke volume to be ejected retrograde
into the left atrium. The most common etiology is
mitral valve prolapse, otherwise known as myxomatous mitral valve disease, a pathological weakening
of the connective tissue associated with an accumulation of glycosaminoglycans.33 In this situation,
loose connective tissue replaces the normal dense
collagen of the valve that subsequently becomes
floppy and can billow backwards into the left atrium
during systole.9 This condition can be isolated or
inherited as a primary autosomal dominant disorder
or associated with Marfan or Ehlers-Danlos syndromes.9 Other etiologies of mitral regurgitation
include rheumatic heart disease, ischemic heart disease, or primary rupture of the chordae tendineae.

565

Pathophysiology
Mitral regurgitation is generally a well-tolerated lesion
in pregnancy with good outcomes.34 As the SVR decreases in pregnancy, the balance between retrograde flow
to the atrium and forward flow to the aorta will increase,
permitting more flow to the systemic circulation.20
Evaluation
Echocardiography is useful to assess the severity of
the regurgitation, identify the etiology, assess left
ventricular systolic function, and determine the presence of left atrial enlargement. An ECG can identify
an arrhythmia such as atrial fibrillation.
Complications
These are relatively rare in pregnancy except for in
cases of severe mitral regurgitation or mitral regurgitation associated with dilated cardiomyopathy and
NYHA class III or IV symptoms, in which case the
maternal mortality reaches 7%.35
Management
Mitral valve regurgitation with normal left ventricular
systolic function can be managed with close observation or diuretic therapy if pulmonary edema develops.
Severe mitral valve regurgitation associated with decreased systolic function (left ventricular ejection
fraction less than 60%) and left ventricular dilation is
suggestive of a chronic severe process36 or dilated cardiomyopathy. Medical management is necessary for
these patients, but surgical intervention may need to be
considered in the setting of significant symptoms which
imposes a significant risk to the fetus.37 A severely
dilated left atrium, resultant arrhythmia, or severely
decreased systolic function (ejection fraction less than
35%) should be treated with anticoagulation.38 Inoptropic agents may be necessary.8,35 Cesarean delivery
should be reserved for obstetric indications.31
Aortic Stenosis
Aortic stenosis, or an abnormal narrowing in the
valve, is most commonly due to a congenital bicuspid aortic valve or rheumatic heart disease in women
of reproductive age. An isolated defect is generally
congenital,39 but if multiple valves are involved, especially right heart valves (such as described in the
initial patient scenario), the etiology is often acquired
rheumatic heart disease.40
Pathophysiology
The stenotic aortic valve obstructs flow from the
left ventricle to the aorta, and a pressure gradient is

566

Obstetrical and Gynecological Survey

created. Hypertrophy of the left ventricle results as

the heart must work harder to push blood forward
through the narrowed orifice. Cardiac output is
essentially fixed,41 as only a certain volume of
blood can be pumped forward regardless of the
bodys increased demand in high-output situations,
such as pregnancy. Therefore, aortic stenosis is
sensitive to fluctuations in preload34; an increase in
venous return will cause transmission of pressure
back to the left atrium and pulmonary arteries
resulting in similar symptoms as those occurring
with mitral stenosis. However, decreased preload
or volume depletion can result in life-threatening
underperfusion8 if the necessary pressure does not
exist to force blood past the narrowed aortic valve
into the systemic vasculature to the coronary arteries and the brain.
Evaluation
An echocardiogram can measure the aortic valve
area, the aortic valve pressure gradient, the severity of left ventricular hypertrophy, and the ejection
fraction. A normal aortic valve is 3 to 4 cm2 in
area, with a pressure gradient of less than 5 mmHg.
Mild disease occurs with a valve area of greater
than 1.5 cm2, and a pressure gradient of less than
50 mmHg; this is relatively well tolerated in pregnancy. Severe disease is present when the valve
area is less than 1 cm2 and the pressure gradient is
greater than 75 mmHg.8 An ECG can identify left
ventricular hypertrophy, left ventricular ischemia,
left atrial enlargement, and arrhythmias such as
atrial fibrillation.
Complications
Angina, syncope, and congestive heart failure9
are severe complications of aortic stenosis and
suggestive complaints should lead to prompt evaluation. Pregnant women should exercise caution in
undertaking physical activities that will increase
metabolic demand, which can in turn cause decreased blood flow to the extremities and to the
brain (resulting in syncope). The combination of
increased left ventricular wall stress (due to higher
left ventricular pressure) and left ventricular hypertrophy may result in decreased coronary perfusion, ischemia, and symptoms of angina. Hypotension should be avoided as this will cause decreased
perfusion to the coronary arteries.42 Likewise,
postpartum hemorrhage or extreme Valsalva maneuvers could lead to hypotension due to decreased venous return and preload. Bradycardia

may also decrease CO, as stroke volume cannot

increase to compensate.8
Management
This lesion ideally should be corrected before
pregnancy. Repairs are not without risk during
pregnancy and catheter-based valvuloplasty is not
very effective; restenosis of the aortic valve can
occur within 1 year.43 Arrhythmias should be medically treated if present. Intrapartum, epidural
anesthesia should be administered slowly and cautiously to avoid hypotension; the anesthesia team
may opt for a predominantly opioid infusion instead of local anesthetics to minimize the risk of
hypotension.34 Cesarean delivery should be reserved for obstetric indications.31 A passive second
stage could be considered for similar indications as
in patients with mitral stenosis.
Aortic Regurgitation
Aortic regurgitation involves a faulty valve that
allows a portion of the stroke volume to travel retrograde into the left ventricle during diastole.
Pathophysiology
As with mitral regurgitation, this lesion is generally well-tolerated in pregnancy.3 The decrease
in SVR decreases both the systolic and diastolic
gradient between the aorta and left ventricle, promoting better cardiac output and less regurgitation.
In severe cases, long-standing aortic regurgitation
can lead to left ventricular dilatation and eventual
dysfunction.
Evaluation
An echocardiogram will assess the severity of the
aortic regurgitation, determine the left ventricular
size and systolic function, and rule out left atrial
enlargement. An ECG can identify left ventricular
dilatation, left atrial dilatation, and arrhythmias such
as atrial fibrillation.
Complications
These are relatively rare in pregnancy except for in
severe cases.3
Management
Arrhythmias associated with aortic valve disease
should be treated with anti-arrhythmic medications
and anticoagulation.8,35 Cesarean delivery should be
reserved for obstetric indications.31

Maternal Valvular Heart Disease in Pregnancy Y CME Review Article

Pulmonic Stenosis or Regurgitation, and

Tricuspid Stenosis or Regurgitation
These lesions are usually well tolerated in pregnancy with minimal risk of heart failure.44 This is due
to the low pulmonary vascular resistance at baseline,
and the rare presence of severe tricuspid and pulmonary valve disease persisting into adulthood; severe
lesions are typically corrected in childhood. Exceptions to the rule and conditions that are less welltolerated include uncorrected severe congenital
pulmonary valve stenosis with right ventricular systolic pressures greater than 75% of systemic pressures, severe pulmonary regurgitation after tetralogy
of Fallot repair,45 and Ebstein anomaly of the tricuspid valve with severe regurgitation.46 Patients with
Ebstein anomaly of the tricuspid valve frequently
have a persistent right to left atrial shunt and a higher
risk of supraventricular tachycardia, which may complicate pregnancy.46 Pulmonary and tricuspid lesions
can also be the result of endocarditis from intravenous drug use.3
Evaluation
An echocardiogram will assess severity of the right
heart outflow obstruction in pulmonary stenosis as
well as tricuspid valve function. A pressure gradient
of 60 mmHg or estimated right ventricular systolic
pressures greater than 75% systemic is indicative of
severe disease.8 An ECG will evaluate right ventricular hypertrophy.
Management
Cesarean delivery should be reserved for obstetric
indications.31
Prosthetic Valves
Cardiac valve replacement is indicated when a
patient with valvular disease has symptoms of severe
heart failure and a diminished quality of life.36 A
replaced valve can have tremendous benefits to patients as it can markedly improve hemodynamic parameters and symptoms. Cardiac valve replacements
can be of several different varieties: mechanical
(prosthetic), bioprosthetic (tissue valves in a structured support apparatus), biologic (porcine, bovine,
or cadaveric), and even an autograft of the patients
native pulmonary valve into the aortic position (Ross
procedure). Replacement valves each have limitations. With a mechanical valve, a patient will require
lifelong anticoagulation, ideally with warfarin.44 Biologic valves do not usually require anticoagulation,
but have an increased incidence of deterioration and

567

failure.47 Both mechanical and biologic valves carry

a risk of endocarditis.48 Patients with prosthetic
valves have a reported average 10-year survival of
70%, and this number is not affected by pregnancy,49
although a patient has a 3% to 5% mortality associated
with pregnancy if she conceives with a mechanical
valve in place.50 Thrombosis is a major complication of
artificial valves, and is of most concern with 2 major
risk factors. The first is the presence of any mechanical
mitral valve.51 These valves have a high propensity to
produce thromboses, as they are located in a lowflow, low-pressure system. They are also subjected to
significant stress as the valves close under the higher
pressures of systole. The second risk factor is a
mechanical aortic valve in the presence of atrial
fibrillation, a previous thromboembolism, an ejection
fraction of 30%, or with multiple mechanical
valves.51
Patients with mechanical valves require anticoagulation during pregnancy, and an extensive discussion is beyond the scope of this review.
Outside of pregnancy, warfarin is the anticoagulation of choice in a patient with a mechanical valve.
However, warfarin, unlike unfractionated heparin or
low-molecular-weight heparins (LMWHs), crosses
the placenta,52 and there is a risk of placental and
fetal hemorrhage throughout pregnancy. Fetal Warfarin Syndrome can occur with exposure between 7
and 12 weeks gestation and is associated with a risk
as high as 30% of embryopathy,3 with hypoplasia of
the nasal bridge, congenital heart defects, agenesis of
the corpus callosum, ventriculomegaly, and stippled
epiphyses.53
During parturition, caution should be exercised in
patients with prosthetic valves and especially those
receiving anticoagulation. Enoxaparin or other
LMWHs should be discontinued 24 hours before
epidural placement to avoid the increased risk of
epidural hematoma. Women receiving unfractionated
heparin should have the equivalent of a normal activated partial thromboplastin time before epidural
placement.54 Because of the longer half-life of
LMWH, patients are typically transitioned to unfractionated heparin at 36 weeks gestation, and this
therapy is usually discontinued at least 4 to 6 hours
before delivery. Either unfractionated heparin or
LMWH can be resumed as early as 6 hours postpartum depending on the circumstances of delivery.
Warfarin is initiated as long as there is no longer a
significant risk of bleeding. The LMWH or unfractionated heparin is continued until the international
normalized ratio has been therapeutic at 2.5 to 3.5 for
24 to 72 hours.8,55

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Obstetrical and Gynecological Survey

MATERNAL MORBIDITY
AND MORTALITY

MATERNAL AND FETAL MANAGEMENT

IN PREGNANCY

A multicenter, prospective study by Siu et al observed 562 pregnant women with heart disease.56
These patients were followed during their second and
third trimesters and evaluated at 6 weeks and 6
months postpartum, and their pregnancy outcomes
were recorded. Complications of pulmonary edema,
arrhythmia, stroke, or cardiac deaths were noted in
13% of the patients. After analysis, 4 predictors for
maternal complications were identified:
1. A prior cardiac event, such as heart failure,
transient ischemic attack, stroke before pregnancy, or arrhythmia.
2. A prepregnancy NYHA class greater than II or
the presence of cyanosis.
3. Left heart obstruction with a mitral valve area
less than 2 cm2 or aortic valve area less than 1.5
cm2 or an aortic outflow gradient greater than
30 mmHg by echocardiography.
4. A left ventricular ejection fraction of less than 40%.

Preconception

The authors concluded that maternal cardiac morbidity could be predicted with the use of a risk index
where 0, 1, or 1 of these factors would lead to a
5%, 27%, or 75% risk of a cardiac event in pregnancy, respectively. There were 3 maternal deaths in
this study, and all had greater than one of the above
predictors.56
Maternal mortality varies significantly depending
on the lesion and the degree of myocardial dysfunction associated with it. Table 3 outlines the mortality
categories of 1%, 5% to 15%, and 25% to 50%.3
Those in the first category often tolerate pregnancy
well, and those in the last have an extremely high
risk, and pregnancy termination should be discussed
or even in some cases recommended.

During this period, it is important to obtain a baseline evaluation of cardiac function, and to confirm
the diagnosis. Counseling can be provided to the
patient regarding her pregnancy risk and the risk to
her fetus. This is an ideal time to involve both cardiology and maternal-fetal medicine to review the
patients current medications and evaluate the risk of
teratogenicity should she become pregnant. If there is
agreement between the medical team and the patient
that pregnancy carries an unacceptably high risk,
reliable contraception should be immediately initiated.57 Options of surrogacy or adoption may be
alternatives for some women, and should be discussed as safer alternatives.
Antepartum
At this time, the patient should have a consultation
with cardiology, maternal-fetal medicine, and anesthesia. Some patients may need to deliver at a tertiary
care center. Throughout pregnancy, the patient
should be evaluated for symptoms of cardiac decompensation.8 She should undergo a fetal echocardiogram if her disease is congenital.58
Intrapartum
In labor and during delivery, it is important to
strictly monitor fluid intake and urine output. Supplemental oxygen should be available. Cardiac telemetry should be used if the patient is prone to an
arrhythmia. An active management of the third stage
should be planned with oxytocin or misoprostol

TABLE 3
Maternal risk of mortality associated with pregnancy
Group I: Minimal Risk of
Complications (Mortality 1%)

Group II: Moderate Risk of Complications

(Mortality 5%15%)

Group III: Major Risk of Complications

or Death (Mortality 25%)

Atrial septal defect*

Ventricular septal defect*
Patent ductus arteriosus*
Pulmonic/tricuspid disease
Corrected tetralogy of Fallot
Bioprosthetic valve
Mitral stenosis, NYHA classes I and II
Marfan syndrome with normal aorta

Mitral stenosis with atrial fibrillation

Mechanical valve
Mitral stenosis, NYHA classes III and IV
Aortic stenosis
Coarctation of the aorta, uncomplicated
Uncorrected tetralogy of Fallot
Previous myocardial infarction
Mitral regurgitation with dilated cardiomyopathy

Pulmonary hypertension
Coarctation of the aorta, complicated
Marfan syndrome with aortic involvement

*If unassociated with pulmonary hypertension.

NYHA indicates New York Heart Association.

Maternal Valvular Heart Disease in Pregnancy Y CME Review Article

available for postpartum hemorrhage.59 Methergine

should be avoided in patients with valvular disease
due to the potential risk of coronary artery vasoconstriction and elevation of systemic blood pressure.60

Postpartum
After delivery, patients with significant disease
should be observed in an intensive care unit for
approximately 12 to 24 hours for intensive hemodynamic monitoring.28 Delivery and especially autotransfusion from the placenta and fluid shifts from
the extracellular space are associated with changes in
preload and afterload that can lead to heart failure. It
is important to discuss contraception options with the
patient before she is discharged from the hospital.

Anesthesia Considerations
Adequate anesthesia or analgesia is paramount, as
pain and anxiety can lead to tachycardia, hypertension, and complications as discussed previously. Either a combined spinal epidural or an epidural are
typically safe options. Local anesthetics are often
avoided in the epidural catheter, and opiates such as
fentanyl are used in patients sensitive to preload and
afterload, as they do not lower peripheral vascular
resistance.34 Anesthesiologists may potentially elect
for central venous monitoring and/or an arterial line34
in severe cases or before a cesarean delivery. Intravenous fluids should be carefully titrated to avoid
heart failure.

Subacute Bacterial Endocarditis

(SBE) Prophylaxis
In 2008, the American Heart Association guidelines stated that obstetrics patients, in general, do not
need SBE prophylaxis because vaginal and cesarean
deliveries result in a low rate of bacteremia.61 In the
highest risk patients (those with prosthetic heart
valves or prosthetic materials, a prior history of endocarditis, an unrepaired congenital cyanotic heart
lesion, or a transplant patient with valvulopathy),
antibiotic prophylaxis can be considered at rupture of
membranes, understanding that there is no evidence
to support the benefit of this approach. If a patient
does develop chorioamnionitis or is admitted during
the antepartum period with pyelonephritis, the physicians choice of antibiotic coverage should include
SBE prophylaxis.61

569

Clinical Case Scenario Follow-Up

In conclusion, patients with valvular heart disease
in pregnancy have varying degrees of impairment,
and recommendations for their care will vary widely.
The patient described at the beginning of this article
had rheumatic heart disease necessitating mechanical
mitral and aortic valves and required lifelong anticoagulation with warfarin. She was counseled not to
attempt pregnancy and after she conceived, termination was discussed. She was quoted a maternal risk of
death of 5%10%, but she chose to proceed with the
pregnancy and continue her medications. After a
discussion of the risks and benefits of LMWH versus
warfarin for anticoagulation, her warfarin was
switched to enoxaparin. With outstanding coordinated care efforts, she had no major complications
during pregnancy and carried her fetus until nearterm at which time she underwent induction of labor.
Both the patient and the baby did well. There are
myriad combinations and approaches to management
of these patients; this review outlined the major
causes, effects, and pathophysiology of maternal valvular heart disease in pregnancy, and it additionally
addressed management principles to provide the
practicing obstetrician with a general reference.
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