C ardiovascular Critical Care

P ERIPARTUM
CARDIOMYOPATHY:
REVIEW AND PRACTICE
GUIDELINES
By Leah Johnson-Coyle, RN, MN, Louise Jensen, RN, PhD, and Alan Sobey, MD

Peripartum cardiomyopathy, a type of dilated cardiomyopathy

of unknown origin, occurs in previously healthy women in the
final month of pregnancy and up to 5 months after delivery.
Although the incidence is lowless than 0.1% of pregnancies
morbidity and mortality rates are high at 5% to 32%. The
outcome of peripartum cardiomyopathy is also highly variable.
For some women, the clinical and echocardiographic status
improves and sometimes returns to normal, whereas for oth-
ers, the disease progresses to severe cardiac failure and even
sudden cardiac death. In acute care, treatment may involve
the use of intravenous vasodilators, inotropic medications, an
intra-aortic balloon pump, ventricular-assist devices, and/or
extracorporeal membrane oxygenation. Survivors of peripartum
cardiomyopathy often recover from left ventricular dysfunction;
however, they may be at risk for recurrence of heart failure
and death in subsequent pregnancies. Women with chronic
left ventricular dysfunction should be managed according to
guidelines of the American College of Cardiology Foundation

2012 American Association of Critical-Care Nurses

and the American Heart Association. (American Journal of
doi: http://dx.doi.org/10.4037/ajcc2012163
Critical Care. 2012;21(2):89-98)

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 P
eripartum cardiomyopathy (PPCM) is a type of dilated cardiomyopathy of unknown
origin. It occurs in previously healthy women in the final month of pregnancy and
up to 5 months after delivery.1 Although the incidence is lowless than 0.1% of
pregnanciesmorbidity and mortality rates are high, ranging from 5% to 32%.2,3
For some women, the clinical and echocardiographic status improve and may
return to normal, whereas for others, PPCM progresses to cardiac failure and even sudden
cardiac death.4 In severe cases, women experience a rapid deterioration in health, show no
improvement with medical therapy, and may require cardiac transplantation or die of heart
failure, thromboembolic events, and/or cardiac arrhythmias.5 Thus, initial severity of left ven-
tricular dysfunction or dilatation is not necessarily predictive of long-term functional outcome.5
In this article, we review PPCM and present guidelines for practice.

Epidemiology that African American women were 2.9 times more

The reported incidence of PPCM varies because
the diagnosis is not always consistent and a com-
parison with age-matched nonpregnant women does
not exist.4,6,7 Reported incidences range from 1 in
299 live births in Haiti8 to 1 in 2229 live births in
Southern California9 to 1 in 4000 live births in the
United States.4 The wide variation most likely is the
result of geographic differences and
Initial severity reporting patterns.10 Also, limited access
to echocardiography in some areas
of left ventricular may lead to overestimation of PPCM.11
dysfunction is Several risk factors predispose a
woman to PPCM, including increased
not necessarily maternal age, multiparity, multiple
pregnancies, and pregnancies compli-
predictive of cated by preeclampsia and gestational
long-term outcome. hypertension.4,6,12 Although PPCM
occurs more frequently in women at
the upper and lower extremes of child-bearing ages
and in older women of higher parity,4,13 the disease
has also been reported in 24% to 37% of young
primigravid women.3,6,14 In contrast, the results of a
large population-based study from Haiti suggested
that multiparity and increasing maternal age are not
risk factors.8 Demakis et al12 and Brar et al15 found
About the Authors
Leah Johnson-Coyle is a nurse practitioner in cardiac
sciences and Alan Sobey is an intensive care physician
in the cardiovascular surgery intensive care unit at the
Mazankowski Alberta Heart Institute, University of Alberta
Hospital, Edmonton, Alberta, Canada. Louise Jensen is
a professor in the Faculty of Nursing at the University of
Alberta in Edmonton.
Corresponding author: Louise Jensen, RN, PhD, Professor,
Faculty of Nursing, 3rd Floor, Edmonton Clinic Health
Academy, University of Alberta, Edmonton, AB, Canada
T6G 1C9 (e-mail: louise.jensen@ualberta.ca).
likely to have PPCM than were white women and 7
times more likely than were Hispanic women. The
greater incidence of hypertension in African Ameri-
cans may influence this finding.4,7
Etiology
PPCM is distinguished from other forms of
cardiomyopathies by its occurrence during preg-
nancy.16 Precise mechanisms that lead to PPCM
remain poorly defined. Many etiological processes
have been suggested: viral myocarditis, abnormal
immune response to pregnancy, maladaptive response
to hemodynamic stresses of pregnancy, stress-activated
cytokines, excessive prolactin excretion, and pro-
longed tocolysis.4,10,17,18 Also, a familial predisposition
to PPCM has been reported.19-21 Although underly-
ing genetic variants common to dilated cardiomy-
opathies are being proposed,22 a genetic basis specific
to PPCM has not been systematically studied.23 The
European Society of Cardiology currently classifies
PPCM as a nonfamilial, nongenetic form of dilated
cardiomyopathy.24
Viral Myocarditis
Viral myocarditis has been proposed as the main
mechanism for PPCM and was first reported by
Goulet et al.25 This proposal was later supported by
Melvin et al,26 who found myocarditis during endomy-
ocardial biopsy in 3 women with PPCM. The biopsy
specimens had dense lymphocytic infiltration with
a variable amount of myocytic edema, necrosis, and
fibrosis. Others27 have also reported an association
between PPCM and viral myocarditis. In a study by
Felker et al,28 62% of women with PPCM had
myocarditis or borderline myocarditis on biopsy;
however, clinical outcomes did not differ between
women with and without myocarditis.

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Abnormal Immune Response
An abnormal immune response to fetal micro-
chimerism (harboring of fetal cells in maternal cir-
culation) has been studied as a cause for PPCM.29
Other researchers4,6,16,18 support this theory that dur-
ing pregnancy fetal cells released into the maternal
bloodstream are not rejected by the mother because
of the natural immunosuppresion that occurs dur-
ing pregnancy. However, after delivery, women lose
the increased immunity, and if fetal cells reside on
cardiac tissue when the fetus is delivered, a patho-
logical autoimmune response can occur, leading to
PPCM in the mother after birth.
Abnormal Hemodynamic Response
During pregnancy, blood volume and cardiac
output increase.4 In addition, afterload decreases
because of relaxation of vascular smooth muscle.13
These changes cause a brief, and reversible, hyper-
trophy of the left ventricle to meet the needs of the
mother and fetus.2 This transient left ventricular
dysfunction during the third trimester and early
postpartum period resolves shortly after birth in a
normal pregnancy.2,18 Pearson et al4 suggested that
PPCM might be due, in part, to an exaggerated
decrease in left ventricular function when these
hemodynamic changes of pregnancy occur.
Apoptosis and Inflammation
An increased concentration of plasma inflamma-
tory cytokines, specifically tumor necrosis factor a;
C-reactive protein; and Fas/Apo-1, a plasma marker
for apoptosis (programmed cell death), have been
identified in women with PPCM.3 Levels of Fas/Apo-1,
a ligand found on cell-surface proteins that plays a
key role in apoptosis, were higher in women with
PPCM than in healthy volunteers.3 Furthermore, these
Fas/Apo-1 levels were higher among women with
PPCM who died than among those with PPCM who
survived. However, a correlation between increased
plasma cytokine levels and left ventricular function
or outcomes has not been demonstrated. Van Hoeven
et al30 further concluded that ejection fraction at the
time of clinical findings suggestive of PPCM was the
strongest predictor of outcome.
Prolactin
Hilfiker-Kleiner et al16,31 have proposed a new
pathogenic mechanism for PPCM: excessive pro-
lactin production. Levels of prolactin are associated
with increased blood volume, decreased blood
pressure, decreased angiotensin responsiveness, and
a reduction in the levels of water, sodium, and potas-
sium.31 Prolactin also increases the level of circulating
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erythropoietin, and hence hematocrit levels.18 Hil-
fiker-Kleiner et al31 discovered that PPCM develops
in mice bred to have a cardiomyocyte-specific dele-
tion of STAT3, a protein that plays a key role in
many cellular processes such as cell growth and
apoptosis. The deletion of STAT3 led to enhanced
expression of cardiac cathepsin D, promoting the
formation of a 16-kD form of prolactin. In women
with PPCM, STAT3 protein levels were low in the
heart, and serum levels of activated cathepsin D
and 16-kD prolactin were elevated.16,31
Selenium and Malnutrition
Nutritional disorders, such as deficiencies in
selenium and other micronutrients, were thought
to play a role in the pathogenesis of PPCM.12,26 Defi-
ciencies of selenium increase cardiovascular suscep-
tibility to viral infections, hypertension, and
hypocalcemia. However, Fett et al32 concluded that
neither low serum levels of selenium nor deficien-
cies of other micronutrients (vitamins A, B12, C, E,
and b-carotene), played a significant role in the
development of PPCM in Haitian women. In con-
trast, women with PPCM from the
Sahelian region of Africa had low
levels of selenium.33
Viral myocarditis
has been
Prolonged Tocolysis
Prolonged tocolysis refers to
proposed as the
the use of tocolytic agents (b-sym- main mechanism
pathomimetic drugs) for more
than 4 weeks.18 The association for peripartum
between tocolytic therapies and cardiomyopathy.
heart failure appears to be unique
to pregnancy. Tocolytic agents are used for the man-
agement of various other conditions without the
occurrence of signs and symptoms of heart failure
like those experienced by pregnant women. Such
signs and symptoms may develop in pregnant
women as a result of normal physiological changes
that occur, including an increase in circulating
blood volume.18 Lampert et al34 found an associa-
tion between use of tocolytic therapies and develop-
ment of pulmonary edema in pregnant women and
proposed a link between chronic use of b-sympath-
omimetic medications and PPCM.
Clinical Manifestations and Diagnosis
Features of a normal pregnancy include increased
blood volume, increased metabolic demands, mild
anemia, changes in vascular resistance associated
with mild ventricular dilatation, and increased car-
diac output.11 Thus, the onset of PPCM can easily be
maskedand missedbecause the manifestations
91
 can mimic those of mild heart failure. Women with
PPCM most commonly have dyspnea, dizziness,
chest pain, cough, neck vein distension, fatigue,
and peripheral edema.4,10,13 Women can also have
arrhythmias, embolic events due to the dilated,
dysfunctional left ventricle, and acute myocardial
infarction due to decreased perfusion to the coronary
arteries.4,10,35 They can also have other indications
typical of heart failure: hypoxia, jugular venous dis-
tention, S3 and S4 gallop, rales, and hepatomegaly.35
Blood pressure is often normal or decreased, and
tachycardia is common.20
PPCM was first defined in 1971 as the develop-
ment of myocardial disease that occurs for the first
time toward the end or in the early stage of the
pregnancy.12 A modification of this classic definition
added a strict echocardiographic crite-
rion.1 The National Heart, Lung, and
Magnetic Blood Institute and the Office of Rare
resonance images Diseases workshop adopted the modi-
fied definition in 2000.4 In 2010, the
can be used to European Society of Cardiology Work-
ing Group on Peripartum Cardiomy-
measure global opathy36 proposed a modification to
and segmental the existing definition of PPCM. PPCM
is defined as an idiopathic cardiomy-
contraction and opathy manifested as heart failure due
identify inflamma- to left ventricular systolic dysfunction
toward the end of pregnancy or in the
tory processes. months after delivery when no other
cause of heart failure is found. Thus,
PPCM is a diagnosis of exclusion, suggesting that a
broader definition would eliminate PPCM as a
missed diagnosis.36
The definitive diagnosis of PPCM depends on
echocardiographic identification of new-onset heart
failure during a limited period around parturition.
A diagnosis of PPCM requires the exclusion of other
causes of heart failure: myocardial infarction, sepsis,
severe preeclampsia, pulmonary embolism, valvular
diseases, and other forms of cardiomyopathy.17,37
Chest radiographs should be obtained in sus-
pected cases of PPCM.35 Chest radiographs may be
helpful in acute pulmonary edema, but much less
so if no clinical evidence of pulmonary congestion
is revealed. Radiological indications of heart failure
such as cardiomegaly, pulmonary congestion, and
pleural effusions may be evident.35 However, diag-
nosing cardiomegaly on the basis of a chest radi-
ograph in a pregnant patient is difficult because
the heart is pushed upward and laterally, giving the
false impression of cardiomegaly.
Electrocardiograms should also be obtained. In
PPCM, the tracings may be normal or may show
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left ventricular hypertrophy, ST-T wave abnormalities,
dysrhythmias, Q-waves in the anteroseptal precor-
dial leads, and prolonged PR and QRS intervals.4,38
Several laboratory tests should be performed: com-
plete blood cell counts and serum levels of troponin,
urea, creatinine, and electrolytes.11,20 Liver function
tests should be done, and levels of thyroid-stimulat-
ing hormone should be measured. In the initial
evaluation, the serum level of troponin may be
helpful in ruling out myocardial infarction; how-
ever an increase in troponin in the acute phase of
PPCM, without myocardial infarction, can occur.35
Levels of B-type natriuretic peptide and N-terminal
pro-B-type natriuretic peptide can help in confirm-
ing the diagnosis.38,39
Unlike pulmonary artery catheterization,
echocardiography is noninvasive and allows serial
evaluations in pregnant women.1 Serial echocardio-
graphy with Doppler imaging is used to evaluate
and monitor regional and global left and right ven-
tricular function, valvular structure and function,
possible pericardial pathological changes, and
mechanical complications.11 Findings in women
with PPCM are consistent with the findings in heart
failure: decreased ejection fraction, global dilata-
tion, and thinned-out cardiac walls.4,11,38
Cardiac magnetic resonance imaging has been
suggested as a complementary tool in the diagnosis
and evaluation of women with PPCM.13,21,30 Such
imaging can be used to measure global and seg-
mental myocardial contraction, can help in charac-
terizing the pathogenesis of the disease, and can
reveal inflammatory processes.13 Baruteau et al21
maintain that because cardiac magnetic resonance
imaging can be used to distinguish inflammatory
from noninflammatory pathogenesis, it can be
helpful at the initial evaluation of a woman with
PPCM to determine the pathophysiology and to
guide further therapeutic options. Ntusi and Chin40
disagree, however, and do not see a benefit for
obtaining cardiac magnetic resonance images in all
women who have PPCM. Guidelines for diagnosis
of PPCM according to the American College of Car-
diology Foundation (ACCF) and the American Heart
Association (AHA)39 are provided in Figures 1 and 2.
Management
Compensated Heart Failure
Management of PPCM is similar to standard
treatment for other forms of heart failure.4,39,41 How-
ever, no randomized clinical trials have been done
to evaluate these therapies specifically in PPCM.
Furthermore, careful attention should be paid to fetal
safety and to excretion of drug or drug metabolites
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during breastfeeding after delivery.4 The goals in
treating heart failure are to improve hemodynamic
status, minimize signs and symptoms, and optimize
the long-term outcomes. Treatment focuses on
reducing preload and afterload and increasing car-
diac inotropy.2,4,35 Pearson et al4 reinforce that col-
laboration among medical specialists, including
obstetricians, cardiologists, perinatologists, and
neonatologists, is essential in care of women with
PPCM. Of note, polypharmacy may be required for
optimal management, to slow progression of heart
failure and to improve outcomes in women with left
ventricular systolic dysfunction.42 Medications should
be continued until evidence indicates improved
and/or resolved left ventricular dysfunction.4,35
Women with PPCM should be treated in the hospital
when they have evidence of hypotension, worsening
heart failure, altered mental status, and increased
work of breathing.42
Preload reduction is accomplished by adminis-
tration of vasodilators, such as nitrates, most of
which are safe during pregnancy and breastfeeding.35
Loop diuretics are important for management of
signs and symptoms and for preload reduction,
although caution is warranted in antepartum women
because rapid changes in intravascular volume can
lead to a decrease in blood supply to the uterus and
therefore the fetus.35,43 Restriction of dietary sodium
is also helpful in preload reduction.2 Bed rest was
once standard care but is no longer recommended
because of the increased risk of thromboembolism.20
The current recommendation is light exercise such
as walking.4,10
Ideal medications intrapartum include hydrala-
zine, nitrates, digoxin, and diuretics. Angiotensin-
converting enzyme inhibitors are contraindicated
during pregnancy because of their teratogenicity,
but these medications are the mainstay of treatment
of PPCM after delivery for afterload reduction.4,13,38
Safe alternatives during pregnancy include hydrala-
zine and nitrates.4,10 Aldosterone antagonists have
been effective when angiotensin-converting enzyme
inhibitors were not tolerated, but the antagonists
should not be used during pregnancy.41
b-Adrenergic antagonists, such as extended-release
metoprolol and carvedilol, have been approved for
use in PCCM and can improve survival.4,20,43 How-
ever, b-blockers should not be given in the early
stages of PPCM because they can decrease perfusion
in the acute decompensated phase of the disease.35
Pearson et al4 have proposed that carvedilol be used
in postpartum women who continue to have signs
and symptoms of heart failure and have echocardio-
graphic evidence of left ventricular compromise
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Woman with signs and symptoms of heart failure who is
In last month of pregnancy or
Within 5 months postpartum
Signs and symptoms of heart failure
Dyspnea Cough
Fatigue (at rest or exertion) Heart palpitations/tachycardia
Neck vein distention Sudden weight gain, fluid retention
Exercise intolerance Arrhythmias
Peripheral edema Paroxysmal nocturnal dyspnea
Weight gain (water retention) Hepatomegaly
Chest pain Weakness
Diagnostic testing
Complete family history, to identify possible familial association
Serum tests
Complete blood cell count with differential
Creatinine and urea levels
Electrolyte levels, including magnesium and calcium
Levels of cardiac enzymes, including troponin
Level of B-type natriuretic peptide and/or N-terminal
pro-B-type natriuretic protein
Liver function and level of thyroid-stimulating hormone
Chest radiograph
Electrocardiogram
Transthoracic echocardiogram
Cardiac magnetic resonance imaging and/or endomyocardial
biopsy (when indicated)
Figure 1 Evaluation of peripartum cardiomyopathy.
after more than 2 weeks of therapy. Digoxin, an
inotropic agent, is also safe during pregnancy and
should be considered for women with left ventricu-
lar systolic dysfunction and an ejection fraction of
less than 40% who have signs and symptoms of
heart failure while receiving standard therapy.42
Guidelines for management of compensated heart
failure in PPCM are presented in Table 1.
Decompensated Heart Failure
In pregnant women with acute decompensat-
ing heart failure, management begins with the
ABCs (airway, breathing, circulation).35 Assessment
of the airway is critical because pregnancy or recent
pregnancy with associated third spacing of excess
intravascular volume can result in a suboptimal air-
way.35 Women with impending respiratory failure
from pulmonary edema require rapid initiation of
supported ventilation.44 Endotracheal intubation is
93
 Diagnostic criteria for peripartum cardiomyopathy
All 4 of the following:
Classic
1. Development of cardiac failure in the last month of pregnancy or
within 5 months postpartum
2. No identifiable cause for the cardiac failure
3. No recognizable heart disease before the last month of
pregnancy
Additional
1. Strict echocardiographic indication of left ventricular dysfunction:
a. Ejection fraction <45%
and/or
b. Fractional shortening <30%
c. End-diastolic dimension >2.7 cm/m2
No: Consider other cause Yes: Meets criteria for
diagnosis for peripartum
cardiomyopathy
Consultation with cardiologist, obstetrician, perinatologist
If diagnosis is made before the woman gives birth: involve
anesthesiology and neonatology also, and consider transfer to
a high-risk perinatal center
Figure 2 Diagnosis of peripartum cardiomyopathy.
Based on Pearson et al4 and Sliwa et al.36
often required; however, attempts involving nonin-
vasive ventilation may obviate intubation.44 Nonin-
vasive ventilation must be used with caution because
of the high risk for aspiration. Breathing is supported
with supplemental oxygen to relieve signs and symp-
toms related to hypoxemia and is assessed via con-
tinuous pulse oxymetry. Women should have cardiac
monitoring, including ST-segment monitoring when
available.13,35 Blood pressure should be monitored
with noninvasive blood pressure cuffs until arterial
catheters are placed. Venous and arterial access
should be obtained early so that medications can
be administered promptly and monitoring can be
streamlined.35 Some clinicians39 advocate for the use
of pulmonary artery catheters in women whose heart
failure is refractory; however, this logic has been
questioned because many of the drugs used to treat
PPCM produce benefits by mechanisms that cannot
be assessed by measurement of short-term changes
in hemodynamic status. Pulmonary artery catheters
may be beneficial in patients with heart failure, but
little information is available on their use in
women with PPCM.
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In antepartum women, fetal heart rate monitor-
ing should be started early because abnormalities in
fetal heart rate tracings are common when maternal
oxygenation and circulation are compromised.35 Med-
ical stabilization of the mothers condition is critical
and may result in resolution of fetal distress and pre-
vent the need for emergency cesarean delivery that
most likely would be poorly tolerated by the mother.35
Women with acute heart failure benefit from
intravenous administration of positive inotropic
agents such as dobutamine and milrinone, none of
which are contraindicated in pregnancy.44 Positive
inotropic agents improve cardiac performance, facil-
itate diuresis, preserve end-organ function, and pro-
mote clinical stability.13,17,39 Dobutamine requires
b-receptors for its inotropic effects, whereas milrinone
does not, an important distinction in planning care
for a patient who is being treated with b-blocking
drugs.39 Furthermore, milrinone has vasodilating
properties for both the systemic and the pulmonary
circulation, a mechanism that may be a marked
benefit over other inotropic agents. In women with
systolic blood pressure less than 90 mm Hg, dobut-
amine may be preferred over milrinone.44 Vasodilatory
drugs such as nitroglycerin and nitroprusside also
may be of benefit.35,39 Nitroprusside should be used
with caution in pregnant women because the toxic
effects of thiocyanate can be harmful to the fetus.20,42
Clinicians should not focus therapy on a spe-
cific blood pressure value that might or might not
indicate hypotension; rather, they should focus on
signs and symptoms associated with poor cardiac
output and hypoperfusion, such as cold clammy
skin, cool upper and lower extremities, decreased
urine output, and altered mental status.39 Inotropic
agents are of greatest value in women who have
relative hypotension and an intolerance or no
response to vasodilators and diuretics.42 Regardless,
if invasive monitoring of hemodynamic status is
used, once the clinical status of the woman has
stabilized, every effort should be made to devise an
oral regimen that can maintain symptomatic improve-
ment and reduce the subsequent risk of any deterio-
ration in her condition.42
Left ventricular thrombus is common in women
with PPCM whose ejection fraction is less than
35%.2,38 Warfarin should be given to postpartum
women whose ejection fraction is 35% or less, and
heparin or a low-molecular-weight heparin should
be given to women who are pregnant and have a
similar ejection fraction.20,35 Anticoagulation therapy
should be continued until left ventricular function
is normal according to echocardiographic find-
ings.13,39 Arrhythmias should be aggressively treated
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to minimize thrombus formation and to optimize
cardiac function.4,10
Immunosuppressive and anti-inflammatory
therapies have not improved the outcome in PPCM
and, in general, are not recommended.45 Because of
the various mechanisms of PPCM, immunosuppre-
sion most likely would not help all women.13,35,45
In a case report, Jahns et al46 stated that bromo-
criptine, a dopamine antagonist that inhibits pro-
lactin secretion, prevented the expected deterioration
in the size of the left ventricle and systolic function
when given in addition to standard heart failure
therapy in a woman with PPCM. The treatment of
STAT3-deficient mice with bromocriptine also pre-
vented the development of PPCM in a study by
Hilfiker-Kleiner et al.16 The results of assessments of
the therapeutic effects of prolactin blockade with
bromocriptine are promising, and trials are being
done in women with PPCM.16,31 In a case study, de
Jong et al47 argue that the benefit of using cabergo-
line, another potent dopamine receptor antagonist
like bromocriptine, is the long half-life, 14 to 21
days, of cabergoline, so a single dose is often enough.
Medical therapy can be unsuccessful in women
with PPCM, and mechanical cardiovascular support
with an intra-aortic balloon pump or ventricular
assist devices may be required.48,49 Left ventricular
assist devices can be a bridge to recovery or to trans-
plantation.13,49-51 Use of short-term extracorporeal
membrane oxygenation has also been of benefit in
women with PPCM whose heart failure was refrac-
tory to medical therapy and who had persistent pul-
monary edema with hypoxemia.48,50 Extracorporeal
membrane oxygenation can also serve as a bridge
to left ventricular assist devices in patients with
refractory cardiogenic shock despite use of an intra-
aortic balloon pump and full inotropic support.51
Women in whom maximal medical manage-
ment is unsuccessful may be candidates for cardiac
transplantation.48 According to one study,14 cardiac
transplantation was necessary in 4% of women with
PPCM. In another study52 in which 69 women under-
went cardiac transplantation for PPCM, the investi-
gators concluded that heart transplantation is a
practical therapeutic option for women with PPCM
who have advanced heart failure and signs and
symptoms unresponsive to medical therapies. The
risk of organ rejection in women with PPCM does
not appear to be higher than the risk in women of
similar age who have a history of pregnancy and
undergo transplantation for other causes. However,
in an earlier study, Koegh et al48 found that the inci-
dence of biopsy-proven early rejection, necessitating
increased cytolytic therapy, was marginally higher in
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Table 1 Management of compensated heart failure in peripartum
cardiomyopathya
Nonpharmaceutical therapies
Low-sodium diet: limit of 2 g sodium per day
Fluid restriction: 2 L/day
Light daily activity: if tolerated (eg, walking)
Oral pharmaceutical therapies
Antepartum management of peripartum cardiomyopathy
b-blocker
Carvedilol (starting dose 3.125 mg twice a day, target dose
25 mg twice a day)
Extended-release metoprol (starting dose 0.125 mg daily, target
dose 0.25 mg daily)
Vasodilator
Hydralazine (starting dose 10 mg 3 times a day, target dose 40 mg
3 times a day)
Digoxin (starting dose 0.125 mg daily, target dose 0.25 mg daily)
Monitor serum levels
Thiazide diuretic (with caution)
Hydrochlorothiazide (12.5-50 mg daily)
May also consider loop diuretic with caution
Low-molecular-weight heparin if ejection fraction <35%
Postpartum management of peripartum cardiomyopathy
Angiotensin-converting enzyme (ACE) inhibitor
Captopril (starting dose 6.25-12.5 mg 3 times a day,
target dose 25-50 mg 3 times a day)
Enalapril (starting dose 1.25-2.5 mg 2 times a day,
target dose 10 mg 2 times a day)
Ramipril (starting dose 1.25-2.5 mg 2 times a day,
target dose 5 mg 2 times a day)
Lisinopril (starting dose 2.5-5 mg daily, target dose
25-40 mg daily)
Angiotensin-receptor blocker (if ACE inhibitor not tolerated)
Candesartan (starting dose 2 mg daily, target dose 32 mg daily)
Valsartan (starting dose 40 mg twice a day, target dose 160 mg
twice a day)
Consider nitrates or hydralazine if woman is intolerant to ACE
inhibitor and angiotensin-receptor blocker
Loop diuretic
Furosemide intravenously or by mouthdosing considerations
should be made on the basis of creatinine clearance
Glomerular filtration rate >60 mL/min per 1.73 m2:
furosemide 20-40 mg every12-24 h
Glomerular filtration rate <60 mL/min per 1.73 m2:
furosemide 20-80 mg every 12-24 h
Vasodilator
Hydralazine (starting dose 37.5 mg 3 or 4 times a day, target dose
40 mg 3 times a day)
Isorbide dinitrate (starting dose 20 mg 3 times a day, target dose
40 mg 3 times a day)
Aldosterone antagonist
Spironolactone (starting dose 12.5 mg daily, target dose
25-50 mg daily)
Eplerenone (starting dose 12.5 mg daily, target dose 25-50 mg daily)
b-blocker as above
Warfarin if ejection fraction <35%
aBased in part on Jessup et al39 and Heart Failure Society of America.42
95
 Table 2 Management of decompensated heart failure in
peripartum cardiomyopathya
Airway
Intubate promptly upon distress for increased work of breathing
to prevent complications with difficult airway later in treatment
Breathing
Provide supplemental oxygen
Maintain continuous pulse oximetry to monitor SaO2
Measure arterial blood gases (if available) every 4-6 h until
breathing is stable
Circulation
Start cardiac and blood pressure monitoring
Insert arterial catheter for accurate blood pressure monitoring
and blood sampling
Obtain central venous access with central venous pressure
monitoring
In antepartum women, obtain fetal monitoring
Pharmacological management of acute heart failure in peripartum
cardiomyopathy
Intravenous loop diuretic (caution is advised in antepartum
women)
Furosemide: dosing considerations should be made on the
basis of creatinine clearance
Glomerular filtration rate >60 mL/min per 1.73 m2:
furosemide 20-40 mg intravenously every 12-24 h
Glomerular filtration rate <60 mL/min per 1.73 m2:
furosemide 20-80 mg intravenous every12-24 h
In severe fluid overload, consider furosemide infusion or
ultrafiltration
Vasodilator
Nitroglycerin infusion 5-10 g/min, titrate to clinical status and
blood pressure
Nitroprusside 0.1-5 g/kg per minute, use with caution in
antepartum women
Positive inotropic agents
Milrinone 0.125-0.5 g/kg per minute
Dobutamine 2.5-10 g/kg per minute
Avoid b-blockers in the acute phase, as they can decrease perfusion
Heparin sodium, alone or with oral warfarin (Coumadin) therapy
Monitor oxygenation with arterial blood gases every 4-6 h until
patients condition is stable
Consider endomyocardial biopsy; if proven viral myocarditis,
consider immunosuppresive medications (eg, azathioprine,
corticosteroids)
Every effort should be made to devise an oral regimen that can
maintain symptomatic improvement and reduce the subsequent
risk of worsening clinical status
If no improvement clinically:
Consider cardiac magnetic resonance imaging
Perform endomyocardial biopsy to detect viral myocarditis
(if not previously completed)
Assist devices:
Intra-aortic balloon pump
Left ventricular assist devices
Extracorporeal membrane oxygenation
Transplantation
If a woman remains refractory to therapy, consult your institutions
guidelines for bromocriptine or cabergoline administration for
suppression of prolactin production
aBased in part on Jessup et al39 and Heart Failure Society of America.42
96 AJCC AMERICAN JOURNAL OF CRITICAL CARE, March 2012, Volume 21, No. 2
Downloaded from http://ajcc.aacnjournals.org/ by AACN on May 8, 2017
women with PPCM than in women with dilated
cardiomyopathy. Table 2 presents guidelines for man-
agement of decompensated heart failure in PPCM.
Outcomes of PPCM
Prognosis of PPCM is positively related to the
recovery of ventricular function.17 Failure of heart
size to return to normal is associated with increased
mortality and morbidity.4 Women with persistent
left ventricular dysfunction are less likely to survive
and recover normal cardiac function than are women
with improved left ventricular function. A fractional
shortening less than 20% and a left ventricular
diastolic dimension of 6 cm or greater at the time
of diagnosis are associated with a more than 3-fold
higher risk for persistent cardiac dysfunction.53
Sliwa et al3 found that ejection fraction was the
strongest predictor of outcome in women with PPCM.
Abboud et al17 reported that 50% of women with
PPCM recover baseline ventricular function within
6 months of delivery. In contrast, Ntusi and Mayosi18
found that only 30% of women with PPCM have
complete recovery of cardiac function; most have
partial recovery. Medical therapy as outlined in the
ACCF/AHA guidelines39 should be continued when
a woman does not recover function. When appropri-
ate, implantation of defibrillators to prevent sudden
cardiac death and use of cardiac revascularization
therapy should be considered.
Reported mortality rates for PPCM vary widely.
In a study by Sliwa et al,3 the mortality rate in 29
women was 32%, whereas in a large population-
based study in Haiti by Fett et al,8 the mortality rate
was 15.8%. In a study of 123 women by Elkayam
et al,5 the rate was 9% at a mean follow-up time of
24 months. Brar et al15 concluded that mortality
rates associated with PPCM were lower than initially
reported at 2.5%, and Mielniczuk et al9 reported a
mortality rate of 1.36% to 2.05%. Earlier diagnosis,
coupled with modern management of heart failure,
most likely has an important influence on the mor-
tality associated with PPCM.9,15 Although rates have
improved, mortality remains extremely high in
women with PPCM.
One of the most frequently cited issues for
women who survive PPCM is whether or not they
can safely become pregnant again.5,14 No clearly
established recommendations for future pregnancies
in these women exist.20 Left ventricular recovery and
function are considered the most reliable prognostic
factors and predictors of survival in subsequent
pregnancies.20 Future pregnancies are not recom-
mended in women with persistent heart failure,
because the heart most likely would not be able to
www.ajcconline.org
tolerate the increased cardiovascular workload asso-
ciated with the pregnancy.5,13 Women whose car-
diomyopathy appears to have resolved are a more
difficult group to counsel.4 Because multiparity has
been associated with PPCM, subsequent pregnan-
cies can increase the risk for recurrent episodes of
PPCM, irreversible cardiac damage and decreased
left ventricular function, worsening of a womans
clinical condition, and even death.4,14
Williams et al35 have suggested that dividing
women into 2 categories (recovered vs nonrecovered
left ventricular function) is most appropriate for
counseling on future pregnancy. Even though the
cardiac function has normalized in the group of
women with recovered cardiac function, the left
ventricular contractile reserve may remain impaired,
and recurrence of PPCM is still possible.4,5 The sub-
set of women with persistent left ventricular systolic
dysfunction should be counseled against subse-
quent pregnancies; the risks are 19% higher for
maternal death than among women with PPCM
whose heart failure has resolved.35
Conclusion
PPCM affects previously healthy women in the
final month of pregnancy and up to 5 months after
delivery.4 The diagnosis is based on 4 criteria.1,4 For
some women, the clinical and echocardiographic
status improve rapidly and sometimes return to
normal. In other women, the clinical condition rap-
idly worsens, no improvement occurs with medical
therapy, and chronic heart failure from persistent
ventricular dysfunction develops.5 No single expla-
nation of the pathogenesis of PPCM is relevant for
all women; the disease has a multifactorial origin.
In acute care, treatment may involve the use of
intravenous vasodilators, inotropic medications, an
intra-aortic balloon pump, ventricular assist devices,
and/or extracorporeal membrane oxygenation.20,50
Survivors of PPCM often recover from left ventricu-
lar dysfunction; however, they may be at risk for
recurrence of heart failure and death in subsequent
pregnancies. Women with chronic left ventricular
dysfunction should be managed according to
ACCF/AHA guidelines.39 Careful assessment of risk
factors in pregnant women could help in the preven-
tion of PPCM. Tools to stratify women by risk who
have recovered from PPCM are needed to predict
the risk of future pregnancies.
eLetters
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FINANCIAL DISCLOSURES
None reported.
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Peripartum Cardiomyopathy: Review and Practice Guidelines
Leah Johnson-Coyle, Louise Jensen and Alan Sobey
Am J Crit Care 2012;21 89-98 10.4037/ajcc2012163
2012 American Association of Critical-Care Nurses
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