S EMINARSIN P ERINATOLOGY ](2014)]]]–

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Available online at

Cardiomyopathy in pregnancy
n
Jennifer Lewey, MD, and Jennifer Haythe, MD
Division of CARDIOlogy, DEPARTMEnt of Medicine, ColumbIA University MEDICAL Center, New York, NY

A R T I C L E I N F O
A B S T R a C T
Keywords:
Cardiomyopathy during pregnancy is uncommon but potentially catastrophic to maternal
Cardiomyopathy
health, accounting for up to 11% of maternal deaths. Peripartum cardiomyopathy is
Pregnancy
diagnosed in women without a history of heart disease 1 month before delivery or within
Peripartum
5 months postpartum. About half of all women will have full myocardial recovery within 6
Heart failure
months of diagnosis, but complications such as severe heart failure or death are not rare.
African-American women have higher rates of diagnosis and adverse events. Women with
preexisting cardiomyopathy, such as dilated or hypertrophic cardiomyopathy, followed
closely during pregnancy often tolerate pregnancy and delivery. Risk factors for adverse
outcomes include functional status at baseline, severity of systolic dysfunction or out flow
tract gradient, or history of prior cardiac event, such as arrhythmia or stroke. The level of
brain natriuretic peptide (BNP) can be used to risk stratify women for adverse events.
Pregnant women with cardiomyopathy should be followed closely by a multidisciplinary
team comprised of nurses, obstetricians, neonatologists, cardiologists, anesthesiologists,
and cardiac surgeons.
& 2014 Elsevier Inc. All rights reserved.

Cardiomyopathy in pregnancy Hemodynamic changes during pregnancy

Cardiovascular disease is present in 0.5–4% of pregnant Antepartum changes

women in developed countries. 1 In the United States and
the United Kingdom, cardiovascular disease is the most
common cause of pregnancy-related deaths. 2,3 Among
women of childbearing age, cardiomyopathy is a relatively
uncommon diagnosis. However, this condition accounts for
over 11% of maternal deaths in the U.S. (Fig. 1).2
The purpose of this review is to describe the clinical
characteristics, risk factors for adverse outcomes, and man-
agement of cardiomyopathy during pregnancy. We will focus
on the most common cardiomyopathy encountered during
pregnancy, peripartum cardiomyopathy (PPCM), and
discuss the management of women with preexisting dilated
cardio- myopathy and hypertrophic cardiomyopathy.
During pregnancy, several hemodynamic changes occur that
can significantly impact even asymptomatic women with
cardiomyopathy.4 These changes are summarized below
and are illustrated in Figure 2.
— PRELOAD INCREASEs: Maternal blood volume begins to
increase at 6 weeks' gestation. By the second trimester,
maternal blood volume increases to 50% above baseline. This
effect leads to increased preload and left ventricular end-
diastolic volume.
— AFTERLOAD DECREASES: As the uterine circulation increases
during the first trimester, the systemic vascular resistance

n
Correspondence to: Center for Advance Cardiac Care, 622 W 168th St, PH 1265, New York, NY 10032.
E-mail address: jhh28@columbia.edu (J. Haythe).

http://dx.doi.org/10.1053/j.semperi.2014.04.021
0146-0005/& 2014 Elsevier Inc. All rights reserved.
2 SE
Fig. 1 – Causes of pregnancy-associated deaths in the
United States by time period. (Adapted with permission
from Berg et al.2)
falls. There is further peripheral vasodilatation that
occurs, likely from decreased vascular responsiveness to
angiotensin and norepinephrine. These cumulative
changes result in a decrease in maternal blood pressure.
— HEART RATE INCREASEs: The heart rate increases by 15–20%
by the third trimester.
The cumulative effect of these changes results in an
increase in cardiac output of 30–50% by the end of the first
trimester. The increase in cardiac output is driven by the
increase in stroke volume during the beginning of pregnancy
and the increase in heart rate in the last trimester. Cardiac
output varies by maternal position. When supine, the gravid
uterus can compress the inferior vena cava and impede
venous return to heart. In this position, the cardiac output
can decrease by 25%. The cardiac output will be greatest
when there is unobstructed venous return to the heart, such
as when the woman is in the left lateral decubitus position.
Labor and delivery
During labor and delivery, marked fluctuations in cardiac
output occur. Each uterine contraction can contribute up to
500 milliliters (ml) of maternal blood volume due to auto-
transfusion of uterine blood. Cardiac output can increase as
60
Cardiac output
Stroke volume Heart rate
Change From Baseline (%)
40
20
0
0 5 8 12 16 20 24
Gestational Age (weeks)
Fig. 2 – Changes in hemodynamic properties throughout
pregnancy. (Data derived from Robson et al.4)
MI N ARSINP E R I NA T O L O GY] (201 4 )] ] ]–] ] ]
much as 30% during the first stage of labor and up to 50%
during the second stage due to maternal pushing.5 Cardiac
output is also augmented by the sympathetic surge induced
by pain and anxiety. Rapid increases in plasma volume can
lead to pulmonary edema in women with cardiomyopathy.
Epidural anesthesia is associated with peripheral vasodilata-
tion and transient hypotension.
The increase in stroke volume is somewhat counteracted
by the blood loss during delivery, with an estimated blood
loss of 500 ml for an uncomplicated vaginal delivery and
1000 ml for a cesarean section. Immediately after delivery,
the cardiac output can increase by 80% above pre-labor
values due to autotransfusion of uterine blood and relief of
IVC congestion. Over the subsequent days to weeks, systemic
venous resistance begins to rise and cardiac output
normalizes.
Risk assessment
Patients with any history of cardiomyopathy should ideally
receive pre-conception risk assessment and counseling. The
purpose of this evaluation is to determine the maternal and
fetal risk of a potential pregnancy. 6 Clinical evaluation
includes a careful history, physical exam, determination of
functional capacity, and assessment of New York Heart
Association (NYHA) functional class (Table). A 12-lead elec-
trocardiogram (ECG) and transthoracic echocardiogram
should be obtained. The echocardiogram will assess for any
impairment in systolic function or left ventricular dilatation,
as measured by the ejection fraction (EF) and LV end-
diastolic diameter (LVEDD), respectively. The
echocardiogram will also quantify valvular stenosis or
regurgitation, pulmonary hyper- tension, and aortic
dilatation if present. Further diagnostic studies may be
required, such as cardiac MRI for women with congenital
heart disease or a right heart catheterization (RHC) for
women with severe pulmonary hypertension.
A prospective study of 562 pregnant women with known
cardiac disease demonstrated that the following risk factors
were associated with an adverse cardiac event during preg-
nancy: prior cardiac event (heart failure, transient ischemia
attach, or stroke) or arrhythmia, baseline NYHA class III or
IV or cyanosis, significant left-sided valvular or outflow
obstruc- tion, and reduced systemic ventricular systolic
function (EF o 40%).7 Women with no risk factors had a 5%
risk of having a cardiac event, whereas women with more
than one risk factor had a 75% risk of a cardiac event. The
population studied included mostly women with congenital
heart dis- ease (74%) and excluded women with PPCM. For
women with known cardiomyopathy prior to pregnancy, this
risk score may be helpful in identifying which women are at
greatest risk for having a cardiac event and planning a
management strategy during the pregnancy and delivery. 6
The risk of women with a history of PPCM who undergo
subsequent pregnancy is discussed in more detail below.
28 32 36 38 Brain natriuretic peptide (BNP) and NT-proBNP have been
used for risk assessment in pregnant women with heart
disease.8,9 In one study following pregnant women with heart
disease, a BNP o 100 pg/ml had a negative predictive value
of 100% for identifying cardiac events during pregnancy.
BNP is
 SE MINARSINP E R I NA T O L O GY] (201 4 )] ] ] –] ] ] 3

rate Table New York

of twin– births Heart Association
is generally classification.
higher in reports of PPCM than in the general population. Although multiparity has tradition- ally
34
been(Adapted
defined aswith permission
a risk from Yancy
factor for PPCM, 19 et al.
recent )
studies have shown that the majority of cases of PPCM in the United States occur
during the first or second pregnancy.18
NYHA Symptoms
The incidence of PPCM appears to be rising in the United States. This trend may be explained by increasing rates of associated
class
risks, such as increasing maternal age and number of multifetal pregnancies, and/or an increasing recognition and diagnosis of
12,17
I
disease. No limitation of physical activity. Ordinary physical
activity does not cause symptoms of HF.
II Slight limitation of physical activity. Comfortable at
Clinical presentation
rest, but ordinary physical activity results in
symptoms of HF.
III Marked limitation of physical activity. Comfortable Timely diagnosis of PPCM can be difficult: heart failure
at rest, but less than ordinary activity causes symptoms during pregnancy may mimic signs and symp-
symptoms of HF. toms that occur during normal pregnancy. At the time of
IV Unable to carry on any physical activity without diagnosis, patients most commonly complain of dyspnea and
symptoms
a quick and useful tool to of HFstratify
risk or symptoms of HF early
pregnancy at rest.on and orthopnea. Other common symptoms include cough, parox-
during pregnancy. ysmal nocturnal dyspnea, palpitations, chest pain, and
HF: heart failure.
abdominal pain. On physical exam, signs of heart failure
may be present, including tachycardia, elevated jugular
Peripartum cardiomyopathy venous pressure (JVP), displaced apical impulse, third heart
sound, systolic murmur of tricuspid or mitral regurgitation,
Definition pulmonary rales, and peripheral edema. Women may have
difficulty lying flat for the exam.
Peripartum cardiomyopathy is an uncommon condition that Initial diagnostic studies will often include an electrocar-
occurs during the peripartum period in women without diogram (ECG), blood work, and echocardiogram. A chest x-
known preexisting heart disease. The modern definition of ray is not necessary for diagnosis but if it is obtained, fetal
peripartum cardiomyopathy as agreed upon by participants shielding should be used. The chest x-ray may show an
in National Heart, Lung, and Blood Institute workshop in enlarged cardiac silhouette, evidence of pulmonary conges-
1997 identifies four diagnostic criteria: (1) the development of tion, interstitial edema, or less commonly, pleural effusions.
heart failure in the last month of pregnancy or within 5 ECG findings include sinus tachycardia with non-specific ST-
months of delivery; (2) absence of an identifiable cause for the and T-wave changes. Basic blood work will usually be
heart failure; (3) no history of recognizable heart disease prior unremarkable but levels of brain natriuretic peptide (BNP) or
to the last month of pregnancy; and (4) left ventricular N-terminal pro-BNP (NT-proBNP) will frequently be
systolic dysfunction as determined by echocardiography. 10 elevated.9 The echocardiogram will show global reduction in
Echocardiography has become important to the diagnosis of LV systolic function with various degrees of LV dilatation.
the condition, and specific recommendations for diagnosis Other com- mon echocardiographic findings include right
include a left ventricular ejection fraction (EF) less than 45% ventricular dilatation, biatrial dilatation, and mitral and
and/or fractional shortening less than 30%.11 tricuspid regur- gitation.20 The suggested echocardiographic
criteria for PPCM diagnosis is an LVEF o 45%, but many
Epidemiology woman with PPCM will
present with an EF significantly lower than this.
The incidence in the United States is estimated to be one per The use of magnetic resolution imaging (MRI) for diagnosis
3000 live births12 with much higher rates reported in Haiti 13 of PPCM is not routine practice. In some cases, MRI may be
and South Africa.14 A higher incidence of disease is found used to confirm the diagnosis of ventricular thrombus.
among African-American woman, who are 3- to 16-fold more According to the American College of Radiology MRI safe
likely to be diagnosed with PPCM. 15,16 Women of African practices guidelines, the use of MRI is probably safe during
descent may be more likely to develop PPCM, but it remains pregnancy; however, gadolinium should be avoided unless it
to be seen how different genetic, environmental, and social is considered absolutely essential.21
factors contribute to this risk.
Risk factors for developing PPCM are not clearly Clinical outcomes
understood. Studies have shown that age, history of
hypertension, parity, and twin pregnancies are associated About half of all women diagnosed with PPCM will have full
with PPCM. PPCM has been diagnosed across the age recovery of LV function (EF 4 50%) by 6 months postpar-
spectrum. Most women in the United States are diagnosed tum.17,22,23 Amos et al.22 conducted a retrospective cohort
between 27 and 33 years of age. 17 In a study by Elkayam et study of 55 women diagnosed with PPCM at Duke University
al.,18 more than 60% of women were older than 30 years. Medical Center. In the follow up period, 62% of women
Women with PPCM are likely to carry a diagnosis of diagnosed with PPCMP had an improvement in their EF,
hypertension, including chronic hypertension, pregnancy- 13% worsened and 25% had no change. 45% had recovery to
induced hypertension, or preeclampsia. 12,18 The nad EF 450%. For the subset of women who ultimately had a
4 SE MI N ARSINP E R I NA T O L O GY]
normalized EF, recovery occurred mostly within the first 2
months after diagnosis.22
We still are not able to predict which women will go on
to have full recovery of ventricular function. Several
studies suggest that the presence of more severe disease at
time of diagnosis, such as echo findings of an EF o 30% or
LVEDD 4
5.6 cm, may predict worse outcomes. However, this relation-
ship is not consistent enough to make predictions for indi-
–
vidual patients.22 24 Compared to individuals with other
causes of cardiomyopathy, women with PPCM are more
likely to have myocardial recovery.25,26
Women with PPCM are at an increased risk of serious
complications including severe heart failure, cardiogenic
shock, cardiopulmonary arrest, arrhythmias, and death.17
Thromboembolic events are not uncommon; LV thrombus
is detected in up to 17% women at the time of
diagnosis.22 Serious complications usually occur within the
first 6 months of diagnosis. Risk factors for developing
complications include an LVEF o 25%, delay in diagnosis
from onset of heart failure symptoms, and African-
American background.27 The estimated mortality rate of
PPCM in the United States vary significantly by
report, ranging from 0% to
–
16.5%.12,15,18,22,26 30 These disparate estimates reflect the
heterogeneity of studied populations, varying duration of
follow-up, different diagnostic criteria, and reporting bias.
A review of deliveries between 1996 and 2005 within the
south- ern California Kaiser healthcare system revealed 60
cases of PPCM with a mean follow-up of 4.7 years.15 The
estimated survival was 96.7% with no patients undergoing
transplant. Goland et al.29 conducted a retrospective chart
review at two institutions for cases of PPCM over a period
of 14 years and found that 7% of women died at an
average follow-up of 19 months. Up to 10% of women with
PPCM develop severe heart failure and ultimately undergo
heart transplantation,
although reporting is influenced by referral bias.22,27,29
African-American women with PPCM have higher rates of
serious complications and death as compared to white
–
women.12,15,22,27 30 This trend mirrors that seen among all
pregnancy-related deaths, where African-American women
have a 3- to 4-fold higher risk of pregnancy-related death
than white women.2 It is not clear how much of this relation-
ship is mediated by differential access to timely diagnosis
and high-quality medical care and environmental factors.
Subsequent pregnancy
Women with a history of PPCM are at increased risk of
adverse events during a subsequent pregnancy compared to
pregnant women without PPCM.24,31,32 The women at greatest
risk are those who have not had full myocardial recovery (EF
o 50%). Elkayam et al.31 identified 44 women with PPCM
who went on to have at least one additional pregnancy. Of
the 28 women who had full myocardial recovery before the
subse- quent pregnancy, 21% experienced a significant
reduction in EF (Z 20% from baseline), 21% developed
symptoms of heart failure during pregnancy, and 14% had a
decreased EF at the time of last follow-up. There were no
deaths in this group of women. Among the 16 women who
had persistent LV dysfunction before the subsequent
(201 4 )] ] ]–] ] ]
experienced heart failure symptoms, and 31% had a
decreased EF at last follow-up. Three of the women (19%) in
this group died.
Compared to women without a history of PPCM and who
have normal ventricular function, women with PPCM with
full myocardial recovery have decreased contractile reserve
as measured by dobutamine stress echo. 33 Although compel-
ling, the use of this test has not been validated in terms of
predicting which women will be able to tolerate the hemody-
namic stress of subsequent pregnancy.
Dilated cardiomyopathy
Dilated cardiomyopathy (DCM) is defined as the presence of
decreased LV contractility and LV dilatation in the absence of
other causes of LV dysfunction, such as congenital, hyper-
tensive, or valvular heart disease. 34 The prevalence of DCM in
the U.S. is 36 cases per 100,000 people and the disease is more
common in African-Americans as compared to white
patients.34 The prevalence of DCM among women of child-
bearing age is uncommon. DCM includes a range of diverse
myocardial processes, including myocarditis, ischemic heart
disease, familial cardiomyopathy, and toxins such as alcohol
or cocaine. Women of childbearing age may develop cardio-
myopathy related to cancer therapies, such as Doxorubicin,
which is used for the treatment of leukemia, lymphoma, and
breast cancer, among other malignancies. Up to half of all
cases of DCM are considered idiopathic.34
Women with preexisting DCM who become pregnant are
most at risk for having an adverse maternal or fetal outcome,
if they have moderate to severe LV dysfunction or poor
functional class prior to pregnancy. 7,35 Among 36 women
with DCM who became pregnant, those who had mild LV
dysfunction (EF 4 45%), good functional status prior to base-
line, and no history of cardiac events (i.e. atrial tachyarrhyth-
mia, transient ischemic attack, and heart failure syndrome)
underwent successful pregnancy without any adverse cardi-
ovascular outcome.
Pregnancy likely impacts the long-term clinical course of
women with DCM, though it is not clear to what extent. 6
Among women with DCM with moderate to severe LV
dysfunction, those who become pregnant have worse clinical
outcomes at 16 months.35 The hemodynamic challenge of
pregnancy, labor, and delivery may contribute to increased
LV dilatation. Furthermore, women who become pregnant
appro- priately interrupt their use of evidence-based heart
failure medications that are teratogenic, such as ACE
inhibitors.
pregnancy (mean EF ¼ 36%), 25% had significant further
reduction in EF, 44%
Hypertrophic cardiomyopathy

Background and genetics

Hypertrophic cardiomyopathy (HCM) is a clinical

condition of LV hypertrophy without LV chamber
dilatation that results in diastolic dysfunction with normal
contractile function. 36 HCM occurs in one of every 500
individuals but often goes undiagnosed. The disease is
diagnosed across the age spec- trum from children to the
elderly and may be diagnosed for
 SE MINARSINP E R I NA T O L O GY]
the first time in women of childbearing age or pregnant
women.
HCM is an autosomal dominant disease with more than
1400 mutations identified among 11 genes encoding proteins
of the cardiac sarcomere. 37 The most common mutations
occur within the genes for beta-myosin heavy chain and
myosin-binding protein C. The expression of any one muta-
tion is heterogeneous: a specific genotype does not predict
one phenotype. Genetically prone individuals may not even
manifest disease until much later in life. Conversely, patients
with a phenotypic presentation of HCM without positive
genetic mutation may have an undiagnosed mutation. Half
of the cases of HCM are caused by familial mutations and the
other half are caused by sporadic mutations.
Clinical course
HCM is diagnosed in the presence of LV wall thickness of
more than 15 mm by echocardiography in the absence of
another condition that could explain LV hypertrophy (i.e.,
hyperten- sion). Hypertrophy can be present at the level of the
ventricular septum, the mid-ventricle, or the apex. Some
patients with HCM will develop left ventricular outflow tract
(LVOT) obstruc- tion as detected by a gradient on
echocardiography. This
represents whether there is partial obstruction of blood flow
from the left ventricle into the aorta caused by systolic anterior
motion of the anterior mitral valve leaflet. Tachycardia and
hypovolemia can worsen the degree of LVOT obstruction.
Approximately one-third of HCM patients will have a signifi-
cant gradient (Z30 mmHg) at rest, one-third will have a
gradient with provocation, and one-third will have no gradient.
The clinical manifestation of the condition is heterogeneous but
most patients fall into one of the following four categories36:
— BENIGN/STAble DISEASE: The patient may be asymptomatic
or develop symptoms later in life.
— HEARt fAIlure: Impaired ventricular relaxation can cause
symp- toms of exertional dyspnea and chest pain requiring
medical therapy. Beta-blockers are the initial therapy and
slow heart rate, increase diastolic filling time, and reduce
myocardial oxygen demand. Some patients may progress to
NYHA class III or IV symptoms requiring an invasive
intervention, such as an alcohol septal ablation or surgical
myectomy. Rarely end-stage patients develop systolic
dysfunction.
— ATRIAL fiBRILLATION: Atrial fibrillation may be associated with
various degrees of heart failure and increases the risk of
stroke.
— Sudden CARDIAC DEATH (SCD): Unpredictable ventricular
arrhythmias can cause SCD especially in young patients
and competitive athletes. Patients with risk factors for
SCD may undergo placement of a primary prevention
cardiac defibrillator.
Risks in pregnancy
Family planning and pre-pregnancy counseling are especially
important for women with HCM because a pregnant woman
has a 50% chance of having an affected child. Genetic screen-
ing should be offered to expectant mothers, if not previously
done. Children born to affected mothers should undergo
(201 4 )] ] ] –] ] ] 5
genetic screening and/or routine clinical follow-up. Women
should also be prospectively counseled about the risk of
pregnancy.
Most women with HCM will safely experience pregnancy
and labor with minimal risk. Women with no or mild
symptoms are unlikely to experience symptoms during preg-
nancy.38,39 The risk of experiencing an adverse cardiac event
appears to be proportional to the degree of symptoms and/or
the degree of LVOT obstruction prior to pregnancy. Patients
with advanced heart failure are at the highest risk of hemo-
dynamic deterioration during pregnancy and should be coun-
seled that the risk of pregnancy may be prohibitively high. 6,36
During pregnancy, the increased plasma volume and stroke
volume are well tolerated and can lower a dynamic outflow
tract gradient. Symptoms may worsen in the setting of a
higher heart rate, as seen most commonly in the third
trimester of pregnancy. For this reason, women who are on
beta-blockers for symptoms prior to pregnancy should con-
tinue the medication during pregnancy and women who
become symptomatic should be started on one. Beta-blockers
are generally safe during pregnancy, although routine mon-
itoring for fetal bradycardia and fetal growth should be con-
ducted. Dehydration should be avoided during pregnancy.
Medical management
Stable heart failure
The goal of medical therapy for pregnant women with
preexisting or newly diagnosed cardiomyopathy is to initiate
or continue medication that improves symptoms and/or
long-term outcomes. Women with preexisting heart disease
should undergo pre-pregnancy counseling, including a
review of currently prescribed medications, so that
medications that are unsafe in pregnancy can be
discontinued. If not per- formed during pregnancy, this
medication review should occur as soon as pregnancy is
recognized. Treatment of cardiomyopathy should follow
current heart failure guide- lines with special consideration to
safety of medications during pregnancy.34 Comprehensive
and current information regarding the safety profile of
medications during lactation is maintained at LactMed
(http://www.lactmed.nlm.nih.gov), a website maintained by
the National Library of Medicine and supported by the
American Academy of Pediatrics.
Angiotension inhibition
ACE inhibitors and angiotensin receptor blockers (ARB) are
FDA risk category D and are contraindicated in pregnancy.
These medications are associated with a high rate of terato-
genic effects and may cause oligohydramnios, renal agenesis,
and fetal death. ACE inhibitors and ARBs should be discon-
tinued in women who are trying to get pregnant and as soon
as possible in women once pregnancy is detected.
Whether to switch women to another vasodilator, such as
hydralazine or amlodipine (both risk category C), is left to the
discretion of the provider. Hydralazine and amlodipine do
not have the same long-term benefit in patients with
cardiomy- opathy, so we prefer to start these medications in
women
6 SE MI N ARSINP E R I NA T O L O GY]
who have another indication, such as hypertension or heart
failure decompensation. Captopril and enalapril are consid-
ered compatible with breast-feeding.40
Beta-blockers
Beta-blockers are generally safe during pregnancy although
some reports suggest that they can cause intrauterine growth
restriction. Carvedilol, metoprolol, and bispoprolol are
approved for chronic heart failure treatment and are risk
category C. Women with known cardiomyopathy should
continue taking a beta-blocker during pregnancy. Beta-
blocker initiation should be considered in women with a
new diagnosis of cardiomyopathy. Beta-1 selective agents are
generally preferred, such as metoprolol, since non-selective
agents may facilitate uterine activity. Women with cardio-
myopathy and significant hypertension may benefit from
labetalol, a non-selective beta-blocker commonly used to
treat hypertension during pregnancy. Newborns born to
mothers on beta-blockers should be observed for 48–72 h to
monitor for bradycardia, hypoglycemia, and respiratory
depression.
Diuretics
Loop diuretics, such as furosemide (risk category C), are used
to treat pulmonary congestion and symptomatic volume
overload during pregnancy. The benefit of symptom relief
must be weighed against the risk of volume depletion and
decreased uterine perfusion. Women with cardiomyopathy
should only be prescribed furosemide if needed, and fetal
amniotic fluid volume should be measured regularly.
Spironolactone
Spironolactone is an androgen antagonist that improves
survival in selected patients with cardiomyopathy. The med-
ication is considered risk category C, but concern remains
about the anti-androgen effect in humans and feminization
reported in male rat fetuses. We generally discontinue spi-
ronolactone during pregnancy.
Digoxin
Digoxin (risk category C) can be used in pregnancy for
women who have persistent heart failure symptoms, despite
treat- ment with beta-blocker and vasodilators. Digoxin can
also be used for treatment of maternal tachycardia, such as
atrial fibrillation with rapid ventricular response. Digoxin
crosses the placenta and has been used in the treatment of
fetal tachyarrhythmias.41
Anticoagulation
The risk for thromboembolic phenomenon is increased dur-
ing pregnancy and further increased in the setting of cardi-
omyopathy with severely reduced ventricular function. The
incidence of thromboembolic complications in pregnant
women with cardiomyopathy is not known, but several
reports of thromboembolic phenomena in women with
PPCM
(201 4 )] ] ]–] ] ]
suggest that risk is high. Anticoagulation should be consid-
ered for all pregnant and postpartum women with cardiomy-
opathy and an LVEF o 35%. Our practice is to anticoagulate
women with a definite indication for anticoagulation, such as
atrial fibrillation or known left ventricular thrombus. Unfrac-
tionated heparin and low-molecular-weight heparin (risk
category C) do not cross the placenta and are safe to use
during pregnancy. Warfarin is a risk category D and should
be avoided during pregnancy, although it is considered safe
during breast-feeding.
Decompensated heart failure
Women who develop symptoms of decompensated heart
failure should be hospitalized in an intensive care setting
and co-managed by high-risk obstetrics team and cardiology
with careful fetal monitoring. Intravenous dilators can be
considered for women with decompensated heart failure in
the setting of hypertension. Nitroglycerin (risk category B),
which has also been used in the setting of preeclampsia and
severe pregnancy-induced hypertension, is preferred over
nitroprusside and nesiritide.42 In the presence of hypotension
or ongoing pulmonary edema with low cardiac output,
inotropic agents may be necessary. Heart failure guidelines
recommend the use of dobutamine (risk category B), milri-
none (risk category C), or dopamine (risk category C) in acute
decompensated heart failure, but little evidence exists to
guide the use of these therapies in pregnant women.
Patients who develop symptoms of decompensated heart
failure with hemodynamic instability, despite optimal med-
ical therapy may need to undergo urgent delivery regardless
of gestational age. Invasive therapies, such as intraaortic
balloon pump or extracorporeal membrane oxygenation, are
not recommended during pregnancy but may be required to
bridge a woman with severe hemodynamic undergoing preg-
nancy termination or delivery.
Management during labor and delivery
Preparation for labor
The medical team taking care of women with cardiomyop-
athy around labor and delivery is interdisciplinary and con-
sists of an obstetrician, obstetric anesthesiologist, and
cardiologist. The team should ideally discuss a delivery plan
prior to the commencement of labor that identifies the plan
for type of labor, anesthesia, and need for any invasive
hemodynamic monitoring. Some women with advanced
heart failure symptoms may need to be admitted late in the
third trimester, prior to the onset of labor for symptom
management and hemodynamic optimization.
Women should be monitored carefully during labor, deliv-
ery, and the postpartum period. Continuous electrocardio-
graphic monitoring and non-invasive blood pressure
monitoring should be provided. In some cases, invasive
monitoring will be helpful but is not routine. An arterial line
is low risk and provides constant blood pressure monitoring.
A central venous line is sometimes placed in anticipation of
needing ionotropes or vasopressors. A Swan–Ganz catheter
to
 SE MINARSINP E R I NA T O L O GY]
measure cardiac filling pressures is not routine and should be
reserved for use in women with decompensated symptoms
or who are at very high risk for decompensation due to the
large and sudden volume shifts during labor and delivery.
Mode of delivery
All women with cardiomyopathy deserve a chance at vaginal
delivery unless they have an obstetric reason for a cesarean
section. Cesarean section is associated with greater risks,
including sudden hemodynamic changes seen with general
anesthesia, greater blood loss as compared to vaginal deliv-
ery, increased risk of wound and uterine infections, and
postoperative thrombophlebitis. The risk of postoperative
incisional bleeding is higher among women on anticoagula-
tion. The average amount of blood loss in a women under-
going vaginal delivery is 500 ml, as compared to 1000 ml in
women undergoing cesarean section.
Women who are at low risk for a cardiac event may be
allowed to have spontaneous vaginal delivery. Women at
higher risk, including those with active heart failure symptoms
or prior cardiac events, may benefit from planned induction
from labor. Labor can be induced at a time when all necessary
medical teams are present in the hospital. Timing of delivery is
decided based upon the clinical condition or the mother and
the fetus. Heart failure symptoms that are refractory to
medical therapy may necessitate premature delivery of the
fetus; however, this decision must be individualized to the
patient.
Induction of labor in women with a favorable cervix may
only require oxytocin administration and rupture of mem-
branes. In order for women with an unfavorable cervix to
avoid a long induction, prostaglandin E analogs are often
used. Prostaglandin analogs are absorbed into the systemic
circulation and can decrease blood pressure, lower systemic
venous resistance, and increase the heart rate. These medi-
cations should be used cautiously in women with cardiomy-
opathy. Cervical ripening can also be assisted by use of Foley
catheter balloon.
Once in labor, the woman should be placed in the lateral
decubitus position in order to relive compression on the IVC
and allow unobstructed venous return to the heart. During
the second stage of labor, the obstetrician should allow the
fetal head to descend to the perineum without maternal
assistance. The Valsalva maneuver during labor may have
undesirable hemodynamic consequences and should be
avoided. The second stage of labor may be assisted by the
use of forceps or vacuum extraction.
Anesthesia
Anesthesia during labor and delivery can cause rapid hemo-
dynamic changes, such as hypotension and lowering of the
systemic venous resistance, which can be challenging for the
woman with cardiomyopathy. Careful use of regional anes-
thesia, such as an epidural or spinal block, can effectively
control pain, blunt the sympathetic stimulation induced by
pain and anxiety, and reduce the maternal urge to push.
General anesthesia should be avoided, if at all possible. The
epidural should be dosed slowly and used in conjunction
with local anesthesia in order to maintain stable
(201 4 )] ] ] –] ] ] 7
hemodynamics. The decrease in preload that often accom-
panies the use of anesthesia can be treated with careful
administration of intravenous fluids and the use of
vasopressors.
In a cohort of 32 women with dilated cardiomyopathy who
underwent 36 deliveries, epidural anesthesia was used in
86% deliveries. Vaginal delivery was achieved in 81% of
women and only 11% women were induced. 35 Half of the
women in this cohort had only mild depression of LV systolic
function and two-thirds had NYHA class I symptoms at
baseline.
Postpartum care
After delivery, uterine bleeding is controlled by uterine con-
tractions from endogenous oxytocin secretion. Bleeding is
further reduced by uterine massage and low-dose intrave-
nous oxytocin administration, in order to reduce its hypo-
tensive effects. The redistribution of volume after delivery
occurs over 12–24 h. Women with severe reduction in LV
systolic function or HCM with severe gradient are at
increased risk for developing heart failure symptoms and
pulmonary edema during this period.
Special considerations for HCM
Women with HCM who tolerate pregnancy may have more
difficulty with labor and delivery due to rapid volume shifts
and change in blood pressure. Labor results in a high
catecholamine state with significant increases in heart rate,
stroke volume, and cardiac output. If beta-blockers have
already been started prior to or during pregnancy, they
should be continued during labor and delivery. Tachycardia
decreases ventricular filling, decreases preload, and may
worsen a dynamic outflow obstruction.6 In this setting, the
increased stroke volume may lead to pulmonary edema and
diuretics may be needed.
The Valsalva maneuver during the second stage of labor
should be avoided with an assisted second stage of delivery.
The use of prostaglandins, which are vasodilatory, for the
induction of labor should be limited. Vaginal delivery is
generally preferred and regional anesthesia, if needed,
should be used cautiously given its potential for
hypotension.43 Cesarean section should be reserved for
obstetric reasons. Anesthesia used during cesarean section
combined with increased blood loss compared to vaginal
delivery can cause a precarious situation for women with
HCM. Intravenous fluids and alpha-agonists, such as
phenylephrine, can be used to counter any adverse effects
of sudden volume loss.6
Postpartum management
The American Academy of Pediatrics recommends human
milk for all infants in whom breast-feeding is not specifically
contraindicated. Women with preexisting cardiomyopathy
should be encouraged to breast-feed. Whether or not women
with PPCM should breast-feed is more controversial. A small
study that recruited women with PPCM via the internet and
followed them prospectively reported that women who
breast-fed had no adverse effects and ultimately had greater
improvement in systolic function.44 In contrast, increasing
8 SE MI N ARSINP E R I NA T O L O GY]
evidence suggests that the use of the dopamine agonist
bromocriptine results in improved systolic function in
women with PPCM.45,46 Bromocriptine is a dopamine receptor 4.
agonist that prevents lactation by suppressing prolactin
production. There are no definitive guidelines at this point
regarding breast-feeding in PPCM/cardiomyopathy.
5.
In the postpartum period, beta-blockers should be contin-
ued and a low-dose ACE inhibitor should be introduced with
uptitration of dose as tolerated, even in breast-feeding 6.
women. Women with PPCM with persistent systolic dysfunc-
tion (EF o 50%) should continue heart failure therapy indef-
initely. Women who do not have improvement in left 7.
ventricular systolic function by 6–12 months are unlikely to
have significant improvement by a later time. Consistent
8.
with heart failure guidelines for patients with other types of
cardiomyopathy, we recommend that women with PPCM
with an EF r 35% after 6–12 months on optimal medical 9.
therapy should be referred for an implantable cardiac defib-
rillator (ICD) and cardiac resynchronization therapy (CRT) if
criteria are met. Women who are at high risk for ventricular
10.
arrhythmias can use a wearable ICD while waiting to assess
if LV function will recover.
The optimal duration of medical therapy for women with
PPCM who go on to have full recovery of systolic function is
not known. For women who have experienced normalized EF 11.
for at least 6 months, it is probably safe to wean medical
therapy with regular assessment by echocardiography.
12.
Annual echocardiography has been recommended in order
to identify any spontaneous deterioration of LV function.17
13.
Conclusions
14.
With an experienced multidisciplinary team, women with
PPCM/cardiomyopathy can be successfully managed through-
out pregnancy and delivery. Close follow-up throughout the
15.
peripartum period is essential. At Columbia, a multidiscipli-
nary team comprised of obstetricians, neonatologists, cardi-
ologists, anesthesiologists, cardiac surgeons, and nurses 16.
provides our patients with a clear peripartum medication
plan, anesthesia plan, and birthing plan. Preparing for com-
plications as a team is essential and allows for an effective
17.
execution of care should a patient require transfer to an ICU,
emergent mechanical support, or urgent cesarean section.
18.
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