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Official reprint from UpToDate®

www.uptodate.com © 2023 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Peripartum cardiomyopathy: Treatment and prognosis

Authors: Wendy Tsang, MD, Roberto M Lang, MD
Section Editor: Candice Silversides, MD, MS, FRCPC
Deputy Editor: Nisha Parikh, MD, MPH

All topics are updated as new evidence becomes available and our peer review process is complete.

Literature review current through: Feb 2023. | This topic last updated: Mar 15, 2023.

INTRODUCTION

Peripartum cardiomyopathy (PPCM, also called pregnancy-associated cardiomyopathy) is a rare

cause of heart failure (HF) that affects women late in pregnancy or in the early puerperium [1].
Although initially described in 1849 [2], it was not recognized as a distinct clinical entity until the
1930s [3]. Earlier terms for this condition include toxic postpartum HF, Meadows’ syndrome,
Zaria syndrome, and postpartum myocardiosis.

Treatment of PPCM is like that employed for other types of HF with left ventricular (LV) systolic
dysfunction. However, modifications to standard therapy are often necessary to ensure the
safety of the mother and the unborn or breastfeeding child. (See "Management of heart failure
during pregnancy", section on 'Management goals'.)

Etiology, clinical manifestations, and diagnosis of PPCM, critical illness during pregnancy and
the peripartum period, HF during pregnancy, and issues related to pregnancy in women with
acquired or congenital heart disease are discussed separately. (See "Peripartum
cardiomyopathy: Etiology, clinical manifestations, and diagnosis" and "Critical illness during
pregnancy and the peripartum period" and "Management of heart failure during pregnancy"
and "Acquired heart disease and pregnancy" and "Pregnancy in women with congenital heart
disease: General principles".)

MANAGEMENT

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Treatment of PPCM is largely like treatment for other types of HF. Additional therapeutic issues
for this population may include arrhythmia management, anticoagulation therapy, mechanical
support, and investigational therapies such as bromocriptine [4]. Patients with PPCM and
severe left ventricular (LV) systolic dysfunction should be cared for at hospitals with advanced
heart failure capabilities.

Heart failure treatment — In women with PPCM and HF, the goals of medical therapy are
similar to those in patients with acute and chronic HF with reduced ejection fraction due to
other causes. These include:

● Supplemental oxygen and assisted ventilation as needed

● Optimization of preload
● Hemodynamic support with inotropes and vasopressors if required
● Relief of symptoms
● When possible, institute chronic therapies that improve long-term outcomes

Due to the unique issues related to pregnancy and the peripartum period, each therapeutic
decision has additional implications. The treatment of HF in pregnant and breastfeeding
patients is discussed in detail separately. Briefly, women with HF during pregnancy should be
treated similarly to other patients with HF. However, angiotensin converting enzyme inhibitors,
angiotensin II receptor blockers, angiotensin receptor-neprilysin inhibitor, sodium-glucose
luminal cotransporter-2 (SGLT2) inhibitors, and mineralocorticoid receptor antagonists are to be
avoided, as they are contraindicated in pregnancy. (See "Management of heart failure during
pregnancy", section on 'Treatment regimens'.)

For women with PPCM who have delivered and are not breastfeeding, acute and chronic HF are
managed using standard therapy. (See "Treatment of acute decompensated heart failure:
Specific therapies" and "Overview of the management of heart failure with reduced ejection
fraction in adults".)

Genetic testing — Genetic counseling and testing may be considered when available,
especially those with a positive family history of dilated cardiomyopathy, sudden death, or
PPCM [5]. This is because approximately 10 to 20 percent of women with HF in the peripartum
period carry mutations known to induce cardiomyopathies [6-11]. Some of these inherited
dilated cardiomyopathies develop during early adulthood and may be difficult to differentiate
from PPCM. Alternatively, patients with these genetic abnormalities may have a predisposition
to developing HF that is triggered by the physiological stress of pregnancy and delivery. A
genetic predisposition to PPCM may also be shared with other diseases such as cancer [12] (See
"Genetics of dilated cardiomyopathy".)

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Arrhythmia management — Arrhythmias are common in patients hospitalized for PPCM [13].
The reported incidence of ventricular arrhythmias has been variable. A large inpatient database
study reported that of 9841 hospitalizations for PPCM, 18.7 percent had an arrhythmia, with
ventricular tachycardia occurring in 4.2 percent and cardiac arrest in 2.2 percent. Much smaller
series have reported cardiac arrest rates between 20 and 25 percent [14,15]. Atrial fibrillation
also occurs commonly and was observed in 3.1 to 11.9 percent of patients with PPCM in various
studies [14,16,17]. Management of arrhythmias during pregnancy is discussed separately. (See
"Supraventricular arrhythmias during pregnancy" and "Ventricular arrhythmias during
pregnancy".)

Device therapy — Decisions regarding use of implantable cardioverter defibrillator (ICD) and
cardiac resynchronization therapy in patients with PPCM should include consideration of the
natural history of these diseases, including the potential of recovery of ventricular function [1].

Specific indications for use of ICD therapy have not been established for PPCM [1], since scant
evidence on use of these devices is available in this patient population [18]. Additionally, up to
20 to 70 percent of women with PPCM have complete recovery of LV ejection fraction (LVEF) to
normal by 3 to 12 months (see 'Recovery of left ventricular function' below) [19]. In women
without significant ventricular arrhythmias, ICD placement should generally be deferred at least
six months following presentation, with the patient receiving optimum medical therapy to
determine whether criteria for placement are present. Whether a wearable defibrillator would
prevent sudden cardiac death in those being monitored for LVEF improvement is unclear
[15,20]. A registry study has demonstrated that wearable defibrillators do deliver appropriate
shocks for ventricular fibrillation within the first months after diagnosis [15]. However, a
wearable defibrillator is not without its risks and concerns. The role of implantable loop
recorders in detecting arrhythmias in this population is also unclear. (See "Primary prevention
of sudden cardiac death in patients with cardiomyopathy and heart failure with reduced LVEF"
and "Secondary prevention of sudden cardiac death in heart failure and cardiomyopathy" and
"Wearable cardioverter-defibrillator", section on 'Newly diagnosed nonischemic
cardiomyopathy'.)

Information on the use of cardiac resynchronization therapy in PPCM is limited, but a limited
observational case series of eight patients suggests that resynchronization in medically
optimized patients resulted in improved systolic function and cardiac remodeling [21]. Cardiac
resynchronization therapy should generally be deferred until at least six months following
presentation, with the patient receiving optimum medical therapy to determine whether criteria
for placement are present. (See "Cardiac resynchronization therapy in heart failure: Indications
and choice of system".)

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Antithrombotic therapy — Patients with PPCM are at high risk for thrombus formation and
thromboembolism due to both the hypercoagulable state of pregnancy and stasis of blood due
to severe LV dysfunction [22,23]. Approximately 7 percent will have a peripheral arterial or
venous thromboembolism within the first 30 days after delivery [24]. However, data are
inconclusive on the utility of antithrombotic therapy (antiplatelet therapy or anticoagulation) to
reduce thromboembolic events or mortality in patients with systolic HF who are in sinus
rhythm. (See "Antithrombotic therapy in patients with heart failure", section on 'Role of
antithrombotic therapy'.)

For pregnant women who require anticoagulation, anticoagulation decisions and choosing a
specific anticoagulation regimen are challenging due to specific risks during various stages of
pregnancy, including the potential teratogenic effects of warfarin in the first trimester, dosing
complexities of the various agents, management during labor and delivery, and the risk of
postpartum hemorrhage. These issues are discussed in detail separately. (See "Use of
anticoagulants during pregnancy and postpartum".)

Our approach to antithrombotic therapy in patients with PPCM is the same as that for other
patients with LV systolic dysfunction (with or without HF). For patients with LV systolic
dysfunction (with or without HF) without LV thrombus or other indications for antithrombotic
therapy, we do not recommend antiplatelet or anticoagulant therapy (see "Antithrombotic
therapy in patients with heart failure", section on 'Our approach'). As an exception, we suggest
anticoagulation in patients with PPCM treated with bromocriptine (which we consider
investigational in this setting, as discussed below), although controlled data are lacking [25].
Thromboembolic events (including stroke and myocardial infarction) have been reported as a
complication of bromocriptine use in patients with PPCM [26]. (See 'Bromocriptine' below.)

However, experts differ in their approach to anticoagulation, and some routinely anticoagulate
patients with PPCM with severe LV systolic dysfunction [5,27].

We suggest anticoagulation for patients with PPCM who have acute intracardiac thrombus or
evidence of systemic embolism. This recommendation is consistent with recommendations for
management of acute ventricular thrombus or thromboembolism in patients with HF generally.
(See "Antithrombotic therapy in patients with heart failure", section on 'Role of antithrombotic
therapy'.)

Standard guidelines for antithrombotic therapy for atrial fibrillation should be followed in
patients with PPCM and atrial fibrillation, including recommending anticoagulation for patients
with PPCM with HF and atrial fibrillation. (See "Atrial fibrillation in adults: Use of oral
anticoagulants".)

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Mechanical circulatory support and cardiac transplantation — Mechanical circulatory

support (MCS) should be considered early in patients who are hemodynamically unstable and
unresponsive to medical therapy with maximal inotropic support. A device can be implanted in
the acute phase either as a "bridge-to-recovery" with subsequent weaning as ventricular
function improves or as a "bridge-to-bridge" with implantation of a more durable device if
continued circulatory support is required. A "bridge-to-transplantation" approach is rarely
required as the initial approach because a high proportion of patients with PPCM will have
some recovery of ventricular function. Thus, a temporary device should always be initially
preferred.

In patients with PPCM, a severely depressed baseline LVEF alone should not be considered an
indication for use of aggressive therapies such as MCS and cardiac transplantation. In PPCM,
lower baseline LVEF is associated with lower likelihood of recovery of LVEF with medical
management as discussed below. However, the baseline LVEF does not adequately predict the
probability of recovery in individual patients. (See 'Maternal outcome' below.)

When MCS is indicated, devices that can be used include intra-aortic balloon counter pulsation
(IABP), venoarterial extracorporeal membrane oxygenation (ECMO), and LV assist device (LVAD)
[25]. The choice of which initial device to implant will depend on patient hemodynamics and
local availability and expertise. Venoarterial ECMO has been associated with an increase in
prolactin levels, which may be detrimental in patients with PPCM [28]. Some experts have
suggested administration of bromocriptine doses up to 10 mg twice daily to suppress prolactin
levels in patients receiving venoarterial ECMO with significantly elevated prolactin levels [5]. As
previously discussed, patients started on bromocriptine should also receive anticoagulation to
reduce their thromboembolic risk. However, we do not use of bromocriptine to suppress
prolactin levels in patients receiving venoarterial ECMO as there is little evidence to support this
treatment [29].

Outcomes were reported for 1258 women, which included 99 with PPCM, who had received
durable mechanical circulatory support [30]. Women with PPCM who received durable
mechanical circulatory support had better survival than women without PPCM, with two-year
survival of 83 percent for the PPCM cohort. These differences were likely because women with
PPCM were younger and had fewer comorbidities. However, rates of recovery of myocardial
function were poor at 6 percent in the PPCM group and 2 percent in those without PPCM.
Indications and use of mechanical circulatory support are discussed separately. (See "Short-
term mechanical circulatory assist devices" and "Treatment of advanced heart failure with a
durable mechanical circulatory support device" and "Management of long-term mechanical
circulatory support devices".)

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Older studies found that transplantation was performed in up to one-third of women with
PPCM [31-33]. Contemporary reports demonstrate that transplantation rates vary from 4 to 23
percent of patients [34-38]. Thus, women with PPCM and significant LV systolic dysfunction
should be managed at a center with transplant capabilities. (See "Heart transplantation in
adults: Indications and contraindications".)

In addition to the potential maternal and fetal risks related to pregnancy after heart
transplantation for any reason (see "Heart transplantation in adults: Pregnancy after
transplantation"), women who have been transplanted for PPCM have worse outcomes
compared with other cardiac transplant recipients. The largest series of cardiac transplantation
for PPCM included 809 PPCM patients of 20,352 women in the UNOS database and found that
PPCM was significantly associated with a higher one-year mortality compared with nonischemic
cardiomyopathy patients [39]. A previous study of 485 patients from the UNOS database found
worse long-term survival in patients transplanted for PPCM compared with all others
undergoing transplantation [38]. Women with PPCM who received a cardiac transplant had
higher mortality, higher incidence of rejection, poorer graft survival, and higher
retransplantation rates. Younger patient age, higher allosensitization, higher pretransplant
acuity, and increased rejection rates are all thought to play a role in these poorer outcomes.

Investigational therapy — The following investigational therapies are not recommended for
PPCM since the efficacy and safety of these approaches have not been established.

Bromocriptine — The role of bromocriptine therapy in PPCM is controversial. There are

conflicting expert opinions regarding bromocriptine use as well as the role of breastfeeding in
patients with PPCM (see 'Breastfeeding' below). While preliminary data have suggested a
benefit from bromocriptine in patients with PPCM, further trials are pending to establish
efficacy. Until additional data are available, we suggest not routinely using bromocriptine for
patients with PPCM.

There are regional differences in bromocriptine use for PPCM. In North America, use of
bromocriptine in this setting continues to be viewed as experimental, as noted in a 2020 review
by North American PPCM experts [27]. In the North American IPAC study, only 1 of 100 patients
was treated with bromocriptine [19]. In contrast, some European experts suggest
bromocriptine use, as reflected by the 2018 European Society of Cardiology (ESC) guidelines for
management of cardiovascular diseases during pregnancy, which included a weak
recommendation for bromocriptine use for PPCM [40]. The 2019 position statement from the
Heart Failure Association of the European Society of Cardiology Study Group on PPCM also
included a weak recommendation for bromocriptine use [5]. A meta-analysis found that 87.5

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percent of European PPCM patients in literature are treated with bromocriptine or cabergoline
[41].

Use of bromocriptine in this setting is based upon an experimental observation of prevention of

PPCM in mice via prolactin blockade with bromocriptine [42]. A small randomized pilot study
and several observational reports have suggested beneficial responses to bromocriptine
therapy in patients with PPCM including enhanced recovery of left and right ventricular function
[4,43-48]. However, available data are insufficient to recommend routine use of bromocriptine
treatment for PPCM. Of note, the drug stops the production of breast milk, making
breastfeeding impossible.

In a randomized open-label study performed in South Africa, 20 women with newly diagnosed
PPCM were randomly assigned to receive either standard care plus bromocriptine (2.5 mg twice
daily for two weeks followed by 2.5 mg daily for six weeks) or standard care alone [4]. The 10
women receiving bromocriptine demonstrated significantly greater improvement in LVEF as
compared with the 10 women receiving standard care only (27 to 58 percent versus 27 to 36
percent). One patient in the bromocriptine group died as compared with four in the standard
care group. Fewer patients in the bromocriptine group reached the composite end point of
death, New York Heart Association functional class III or IV HF ( table 1), or LVEF <35 percent
at six months, as compared with patients in the standard care group (one versus eight). The
generalizability of these results is unclear given the small sample size, the higher than expected
mortality rate in the standard care group, and differences in characteristics of PPCM in patients
in Africa as compared with those elsewhere [49].

A subsequent multicenter trial performed in Germany enrolled 63 women with PPCM with LVEF
≤35 percent who were randomly assigned to short-term bromocriptine (one week of 2.5 mg
daily) or long-term bromocriptine (eight weeks: 5 mg for two weeks followed by 2.5 mg for six
weeks) in addition to standard HF therapy [50]. Improvement in LVEF as assessed by cardiac
magnetic resonance imaging at six months was similar in the two groups (28 to 49 percent in
the one-week group and 27 to 51 percent in the eight-week group). The frequency of full
recovery (LVEF ≥50 percent) was nominally but not significantly higher in the eight-week group
compared with the one-week group (68 versus 52 percent). None of the patients required heart
transplantation, LV assist devices (LVAD), or died during the study period. Thus, the patients in
this trial had better outcomes than observed in prior series of PPCM, but a placebo control
group was not included in the study. Results from the NIH-funded North American
Bromocriptine trial, which will randomize patients to eight weeks of bromocriptine or placebo,
are pending. (See 'Maternal outcome' below.)

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As noted above, we suggest anticoagulation in patients with PPCM treated with bromocriptine
(when this investigational therapy is used) given the risk of thromboembolic complications [40].
(See 'Antithrombotic therapy' above.)

The 2019 position statement from the Heart Failure Association of the European Society of
Cardiology Study Group on PPCM suggested that cabergoline be used as an alternative to
bromocriptine if bromocriptine is not available [5]. We do not use cabergoline in this setting
since there are limited supportive data [51,52].

Immunosuppressive agents — Immunosuppressive therapy is not recommended for PPCM

[1]. Although immunosuppressive therapy has been reported in patients with PPCM and biopsy-
proven myocarditis in an observational study [31], its efficacy is unclear. Empiric
immunosuppression, in the absence of evidence of a responsive form of myocarditis (eg, giant
cell myocarditis), is not recommended since most reported cases have nonspecific biopsy
findings [53]. These drugs often have significant side effects, and studies in other forms of
myocarditis have not shown clear benefit from immunosuppressive therapy [54]. (See
"Treatment and prognosis of myocarditis in adults", section on 'Immunosuppressive therapy'.)

Intravenous immune globulin — Intravenous immune globulin (IVIG) has been tried in
patients with myocarditis or recent-onset dilated cardiomyopathy with no clear evidence of
clinical benefit. A retrospective study of six women with PPCM treated with IVIG and 11
historical controls found a greater increase in LVEF at six months in patients treated with IVIG
compared with controls (26 versus 13 percent) [55]. However, the efficacy of this approach has
not been confirmed in this setting or other types of myocarditis. (See "Treatment and prognosis
of myocarditis in adults", section on 'Intravenous immune globulin'.)

Other investigational therapies — Administration of antisense therapy against microRNA-

146a is an attractive potential option, as it would allow lactation. It has been tested in mouse
models and was found to be effective in lessening the development of systolic dysfunction but
did not fully reverse PPCM [56]. Treatment in humans has not been tried to date.

A case report described use of apheresis to remove circulating soluble fms-like tyrosine kinase-
1 (sFlt-1) in a woman with severe PPCM requiring prolonged biventricular assist device support
[57]. The safety and efficacy of apheresis in this setting has not been determined.

DELIVERY

Limited data are available to guide the timing and mode of delivery in PPCM. Decisions
regarding timing and mode of delivery should be based on combined input from the cardio-
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obstetric team including cardiology, obstetrics, anesthesiology, and neonatology services [1]. In
this regard, multidisciplinary conferences are necessary.

Ideally, stabilizing the mother prior to delivery is preferred. In hemodynamically stable women
with no absolute indication for cesarean delivery, vaginal delivery with close hemodynamic
monitoring and epidural analgesia is recommended [5] (see "Neuraxial analgesia for labor and
delivery (including instrumented delivery)"). In women with PPCM with advanced HF and
hemodynamic instability despite medical therapy, we suggest prompt delivery for maternal
cardiovascular indications. Urgent delivery may be required in women with advanced HF with
hemodynamic instability [5]. Planned cesarean delivery with central neuraxial anesthesia is
preferred for women with advanced HF requiring inotropic therapy or mechanical circulatory
support [1,5,58]. (See "Cesarean birth: Preoperative planning and patient preparation" and
"Anesthesia for cesarean delivery".)

For other women, the risks and benefits of early delivery should be considered and discussed
with the patient. The 2010 European Society of Cardiology working group statement advised
that early delivery is not required if the maternal and fetal conditions are stable [1]. However,
patient-specific issues, including gestational age, cervical status, fetal status, and the potential
cardiovascular impact of continuing pregnancy should be considered in timing delivery. As for
women with other types of cardiac conditions, cesarean delivery in patients with stable
cardiovascular status is generally reserved for obstetrical indications (eg, failure of progression
of labor, placenta previa, fetal intolerance of labor). (See "Acquired heart disease and
pregnancy", section on 'Mode and timing of delivery'.)

POSTPARTUM

Breastfeeding — Some experts, including the 2018 European Society of Cardiology guidelines
for the management of cardiovascular diseases during pregnancy, suggest that prevention of
lactation may be considered in patients with severe HF because of the potential effects of
prolactin subfragments and the high metabolic demands of lactation and breastfeeding [40].
(See "Peripartum cardiomyopathy: Etiology, clinical manifestations, and diagnosis", section on
'Role of prolactin'.)

However, a study designed to examine predictors of ventricular recovery found that in 37 of 55

patients who chose to breastfeed, none had adverse maternal effects and that rate of recovery
of LV function was significantly higher in lactating women. Overall, given the benefits of
breastfeeding and this report, some experts have recommended that women who are clinically

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stable should not be discouraged from breastfeeding as long as it is compatible with their HF
medications [59].

If a decision is made to proceed with breastfeeding, we suggest avoiding angiotensin II

receptor blockers due to lack of safety data. (See "Management of heart failure during
pregnancy", section on 'Avoid angiotensin inhibition'.)

Contraception — Women with PPCM or history of PPCM should receive counseling regarding
risk of recurrence and family planning and contraception options.

Direct evidence is lacking on the safety of contraceptives in women with PPCM [60] and limited
data are available on the risk of recurrence, so our approach, which is consistent with the
Centers for Disease Control and Prevention guidelines, is based upon indirect evidence. (See
'Prognosis' below.)

Since women with PPCM with persistent LV dysfunction are at high risk of recurrent PPCM, we
suggest avoiding future pregnancy in such patients [5,40]. We suggest that the patient or her
partner undergo a sterilization procedure or that the patient use a reliable form of
contraception that does not contain estrogen, such as the etonogestrel implant, a copper
intrauterine device (IUD), or levonorgestrel-releasing IUD. Depot medroxyprogesterone acetate
is not as reliable, so it is considered a second-line alternative.

Though the risk of recurrence appears to be lower in women with PPCM with recovered LV
function, such patients should receive counseling, including the option of avoidance of
subsequent pregnancy, due to the risk of relapse of PPCM.

Progestin-containing contraceptives (eg, pills, patch, vaginal ring) may increase fluid retention,
which may worsen HF. Estrogen-containing contraceptives should be avoided in women with
persistent significant LV dysfunction because of their potential to increase the risk of
thromboembolism [60,61].

THERAPY AFTER RECOVERY OF LEFT VENTRICULAR FUNCTION

Patients with PPCM with persistent LV dysfunction should be continued on standard HF

treatment indefinitely. A subset of patients with PPCM will achieve full recovery of LV function
(LVEF >50 percent). It must be emphasized that LV dysfunction can re-occur despite initial full
recovery and this recurrence risk is not limited to occurring during subsequent pregnancies. For
patients with recovery of LV function, there are limited studies examining the relationship
between medication withdrawal and clinical outcomes and there are no major societal

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guidelines regarding this management pathway. Clinical markers (ie, contractile reserve on
stress echocardiography) or biomarkers that may predict outcomes in this group are under
study.

In those patients who demonstrate persistent normal LV function (LVEF >50 percent) for a
period of at least six months, we suggest stepwise weaning of the HF regimen with close
clinical follow-up (eg, every three to four months) and with echocardiographic monitoring (eg,
every six months) to ensure stability of LV function during and for at least one to two years after
weaning of HF medications to ensure stability.

An example of such a protocol is described in a review by PPCM experts, as follows [62]. If LV

structure and function have recovered and remain normal for six months, mineralocorticoid
receptor antagonist (eg, spironolactone) is withdrawn with continuation of beta blockade and
angiotensin converting enzyme (ACE) inhibitors/angiotensin II receptor blockers (ARBs). If, six
months after stopping the mineralocorticoid receptor antagonist, LV function remains normal
and the patient remains free of clinical HF symptoms, withdrawal of the ACE inhibitor/ARB is
suggested. The patient then continues on beta blockade alone. If there is no decline in LV
function, the patient is then weaned from beta blocker therapy, preferably over a period of two
to four weeks to avoid rebound phenomena, and again with close clinical monitoring and
echocardiographic follow-up. Loop diuretics or thiazides may be discontinued at any time (even
before full recovery of LV function) if the patient is free of congestive symptoms; recurrence of
congestive symptoms would prompt reintroduction of these medications.

A decline in LV systolic function as documented by echocardiographic assessment, or the

recurrence of HF symptoms at any point in the process of weaning HF medications, would
dictate a reinstitution of standard HF therapy.

These recommendations are based on expert opinion only; there is a paucity of data in this area
to guide clinicians. Therefore, it is imperative that if HF therapies are withdrawn, the patient
should be followed clinically and by echocardiography to ensure stability, as described above.

PROGNOSIS

The prognosis of PPCM includes maternal, obstetric, and neonatal outcomes, and the effect of
subsequent pregnancy.

Maternal outcome — Several studies have evaluated the outcome for women with PPCM
[18,19,34-36,63-67]. (See "Acquired heart disease and pregnancy", section on
'Cardiomyopathy'.)
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Mortality and morbidity — The mortality rate for PPCM varies with region [68,69]. Mortality
rates range from 1.4 to 3.4 percent within 30 days of diagnosis [70]. A systematic review
reported all-cause mortality of 8 percent at six months, with higher rates of 11.5 percent in
Asia/Pacific, 10.9 percent in Africa, and 6.6 percent in the Middle East, and with the lower rates
of 2.9 percent in North America and 0.7 percent in Europe [41]. Twelve-month mortality in this
meta-analysis was 9.8 percent, with a range from 2.3 percent in Europe to 15.2 percent in Africa.
Mid-term outcomes are reportedly 10 percent in two years [34], 6 percent in five years
( figure 1) [64], and 11 percent in three years [18]. Cardiac transplantation rates of less than 1
to 2 percent per year have been reported [18,34,35].

Death due to PPCM is usually caused by ventricular arrhythmias, progressive pump failure,
sudden death, or thromboembolic events. The following adverse prognostic factors have been
identified in various studies:

● Worse New York Heart Association functional class ( table 1) [71]

● LVEF ≤25 percent [45]
● Being from a Black population [58,63]
● Indigent status [72]
● Multiparity (ie, having given birth two or more times) [58]
● Age greater than 30 to 35 years [73,74]

PPCM is associated with significant extracardiac morbidity including brain injury. In a study of
182 women with PPCM, 46 had major adverse events (MAE) including death, cardiac
transplantation, mechanical circulatory support, cardiopulmonary arrest, fulminant pulmonary
edema, thromboembolic complications, and defibrillator or pacemaker implantation [75]. In
half of the patients with an MAE, the MAE preceded diagnosis of PPCM. One-third of patients
who had an MAE other than death or cardiac transplantation had residual brain damage as a
result of cerebrovascular accident or cardiopulmonary arrest.

Recovery of left ventricular function — Partial or complete recovery of LV function is

common among patients with PPCM and appears to be more frequent than with other types of
dilated cardiomyopathy [76]. In a systematic review, complete recovery of LV systolic function
(LVEF >50 percent) was 44 percent within six months overall, with a lower rate of 13.6 percent in
the Middle East and a higher rate of 56.8 percent in Europe [41]. At one year, 58.7 percent had
recovered their LV function. In the North American IPAC Study, 72 percent had recovery of LV
systolic function (LVEF ≥0.50) at one year [19]. Although recovery of LV systolic function often
occurs within six months of diagnosis [34,63,77], delayed recovery of LV function is also
reported [17,18,72,78]. In one series of 100 patients, 42 women showed partial or complete
improvement in LVEF occurring over months to five years [18]. In this series, only 4 of 23
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women who eventually had complete recovery achieved this within six months. It has been
observed that women with recovered LVEF may have subtle systolic and diastolic dysfunction
and reduced maximal exercise capacity [75,79].

Various studies have identified the following predictors of persistent LV dysfunction at follow-
up:

● LVEF ≤30 percent [34]

● Fractional shortening less than 20 percent and an LV end-diastolic dimension ≥6 cm [80]
● Elevated cardiac troponin T [81]
● Low blood pressure [82]
● Being from a Black population [18,76]
● Diagnosis during pregnancy [18]
● Reduced right ventricular function measured using fractional area change on
echocardiography [83] or volumes on cardiac magnetic resonance imaging [84]

While recovery of LV function in patients with PPCM is related to the degree of dysfunction at
the time of diagnosis, baseline LVEF has limited sensitivity for prediction of improvement in
individual patients [85]. Small preliminary studies of the value of dobutamine stress
echocardiography to predict recovery of LV function have yielded mixed results [86,87].

Obstetric and neonatal outcomes — Data are more limited on obstetric and fetal outcomes.
In a report of 123 patients, cesarean delivery was performed in 40 percent of patients, largely
for obstetric indications [34]. Preterm birth (<37 weeks) occurred in 25 percent, the mean birth
weight was 3.1 kg (range 1.4 to 5.0 kg), and 5.9 percent of infants were small for date. There
were two stillbirths, one neonatal death, and four newborns had congenital anomalies.

Subsequent pregnancy — Women with PPCM or history of PPCM should receive counseling
regarding the risk of recurrence with subsequent pregnancies [1]. The available data on risk of
recurrence of PPCM come from several small studies, which suggest that the risk of
complications is high, particularly among women who do not have full recovery of LV function.
Termination of pregnancy may not prevent relapse. Although limited data are available, we
suggest that patients with PPCM with persistent LV dysfunction (LVEF <50 percent) or LVEF ≤25
percent at diagnosis should be advised to avoid a subsequent pregnancy due to the risk of HF
progression and death [1]. Other patients with PPCM should also be advised of the risk of
recurrence. Also, a small study suggested that reproductive success in women with PPCM is
reduced [88]. (See 'Contraception' above.)

Recovered LV function — Among women in whom LV function returns to normal, the risk of
LV dysfunction during subsequent pregnancy appears lower than for those with persistent LV
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dysfunction, but elevated compared with the general population [89-91]. In a series of 28
women who recovered to an LVEF ≥50 percent after the initial episode, the following results in
subsequent pregnancies were noted [89]:

● There were no deaths

● There was a reduction in the mean LVEF (56 to 49 percent), and the LVEF fell by more than
20 percent in six women (21 percent)
● Six patients developed HF symptoms

The persistent risk in such women may be related to subtle residual dysfunction that is not
detected on resting evaluations. Support for this hypothesis comes from a report of seven
women with a history of PPCM who regained normal resting LV size and performance [67].
Contractile reserve, assessed by dobutamine challenge, was significantly impaired compared
with matched controls.

In summary, some women who recover LV function after an initial episode of PPCM will have
significant decline in LV function during a subsequent pregnancy. Women with PPCM with
normalized LV function should be counseled about the potential risks of recurrence and
carefully monitored for signs of ventricular dysfunction if they choose to become pregnant
again.

Persistent LV dysfunction — The potential risks of subsequent pregnancy in women who

have persistent LV systolic dysfunction appear to be substantial, as illustrated by the following
observations:

● In a series of 16 women with PPCM with persistent LV dysfunction who had subsequent
pregnancies, three died (19 percent) [89]. In addition, there was a further reduction in the
mean LVEF (36 to 32 percent), HF symptoms developed in seven patients, premature
delivery in six, and therapeutic abortion in four.

● In a report of six women who had subsequent pregnancies after PPCM, two who had
persistent LV dysfunction died three months postpartum due to HF [92].

● A more complex pattern was illustrated in a review of 15 women with PPCM, 14 of whom
had incomplete LV recovery [93]. Subsequent pregnancy resulted in worsening HF in eight
women (53 percent) and one death from worsening HF 10 months postpartum. Seven
women did not develop worsening HF during the second pregnancy; these women all had
continued improvement and normalization of LV function (LVEF ≥50 percent) within 30
months of the subsequent pregnancy.

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Therapy to improve LV function — A study of 34 patients with PPCM with a subsequent

pregnancy found that patients who treated immediately after delivery with at least four weeks
of bromocriptine therapy in addition to standard therapy for HF had higher rates of recovery
and higher LVEFs compared with those who were treated with standard HF therapy alone [94].
However, further studies are needed to determine the efficacy and safety of this approach.

SOCIETY GUIDELINE LINKS

Links to society and government-sponsored guidelines from selected countries and regions
around the world are provided separately. (See "Society guideline links: Heart failure in adults"
and "Society guideline links: Cardiomyopathy" and "Society guideline links: Management of
cardiovascular diseases during pregnancy".)

SUMMARY AND RECOMMENDATIONS

● Management – The management of heart failure (HF) due to peripartum cardiomyopathy

(PPCM) is like that of HF due to other causes that occur during pregnancy with special
attention to particular risks during pregnancy, including fetal risks. (See 'Heart failure
treatment' above and "Management of heart failure during pregnancy", section on
'Management goals'.)

● Bromocriptine – The role of bromocriptine therapy in PPCM is controversial. While

preliminary data have suggested a benefit from bromocriptine in patients with PPCM,
further trials are needed to establish safety and efficacy. Until additional data are
available, we do not suggest routinely using bromocriptine for patients with PPCM (Grade
2C). Some other experts advocate using bromocriptine routinely in this setting. If
bromocriptine is used, anticoagulation should also be started to prevent thromboembolic
events. (See 'Bromocriptine' above.)

● Delivery – Decisions regarding the timing and mode of delivery in PPCM should be made
based upon combined input from cardiology, obstetrics, anesthesiology, and neonatology
services. Prompt delivery is suggested in women with advanced HF and hemodynamic
instability despite medical therapy. (See 'Delivery' above.)

● Subsequent pregnancy – The risk of recurrence with subsequent pregnancy is highest

among women with persistent left ventricular (LV) systolic dysfunction, although women
with recovered LV systolic function are also at risk for recurrence. (See 'Subsequent
pregnancy' above.)
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All women with PPCM should receive counseling on the potential risk of recurrence with
future pregnancies. We suggest that women with a history of PPCM who have persistent
LV dysfunction (LV ejection fraction <50 percent) be advised to avoid pregnancy due to the
risk of HF progression and death. (Grade 2C). (See 'Subsequent pregnancy' above and
'Contraception' above.)

ACKNOWLEDGMENT

The UpToDate editorial staff acknowledges Amy Bales, MD, who contributed to earlier versions
of this topic review.

Use of UpToDate is subject to the Terms of Use.

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Topic 95071 Version 24.0

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GRAPHICS

NYHA and other classifications of cardiovascular disability

Canadian
NYHA functional Cardiovascular Specific activity
Class [1]
classification Society functional scale [3]
[2]
classification

I Patients with cardiac Ordinary physical Patients can perform

disease but without activity, such as to completion any
resulting limitations of walking and climbing activity requiring ≥7
physical activity. stairs, does not cause metabolic equivalents
Ordinary physical angina. Angina with (ie, can carry 24 lb up 8
activity does not cause strenuous or rapid steps; do outdoor work
undue fatigue, prolonged exertion at [shovel snow, spade
palpitation, dyspnea, work or recreation. soil]; do recreational
or anginal pain. activities [skiing,
basketball, squash,
handball, jog/walk 5
mph]).

II Patients with cardiac Slight limitation of Patients can perform

disease resulting in ordinary activity. to completion any
slight limitation of Walking or climbing activity requiring ≥5
physical activity. They stairs rapidly, walking metabolic equivalents
are comfortable at uphill, walking or stair- (eg, have sexual
rest. Ordinary physical climbing after meals, in intercourse without
activity results in cold, in wind, or when stopping, garden, rake,
fatigue, palpitation, under emotional weed, roller skate,
dyspnea, or anginal stress, or only during dance foxtrot, walk at 4
pain. the few hours after mph on level ground)
awakening. Walking but cannot and do not
more than 2 blocks on perform to completion
the level and climbing activities requiring ≥7
more than 1 flight of metabolic equivalents.
ordinary stairs at a
normal pace and in
normal conditions.

III Patients with cardiac Marked limitation of Patients can perform

disease resulting in ordinary physical to completion any
marked limitation of activity. Walking 1 to 2 activity requiring ≥2
physical activity. They blocks on the level and metabolic equivalents
are comfortable at climbing 1 flight in (eg, shower without
rest. Less-than- normal conditions. stopping, strip and
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ordinary physical make bed, clean

activity causes fatigue, windows, walk 2.5
palpitation, dyspnea, mph, bowl, play golf,
or anginal pain. dress without
stopping) but cannot
and do not perform to
completion any
activities requiring >5
metabolic equivalents.

IV Patients with cardiac Inability to carry on Patients cannot or do

disease resulting in any physical activity not perform to
inability to carry on any without discomfort. completion activities
physical activity Anginal syndrome may requiring >2 metabolic
without discomfort. be present at rest. equivalents. Cannot
Symptoms of cardiac carry out activities
insufficiency or of the listed above (specific
anginal syndrome may activity scale III).
be present even at
rest. If any physical
activity is undertaken,
discomfort is
increased.

NYHA: New York Heart Association.

References:

1. The Criteria Committee of the New York Heart Association. Nomenclature and Criteria for Diagnosis of Diseases of the
Heart and Great Vessels, 9 th ed, Little, Brown & Co, Boston 1994. p.253.
2. Campeau L. Grading of angina pectoris. Circulation 1976; 54:522.
3. Goldman L, Hashimoto B, Cook EF, Loscalzo A. Comparative reproducibility and validity of systems for assessing
cardiovascular functional class: Advantages of a new specific activity scale. Circulation 1981; 64:1227.

Graphic 52683 Version 19.0

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Outcome with a cardiomyopathy is related to the etiology

In a study of 1230 patients with a cardiomyopathy of various etiologies, the

adjusted Kaplan-Meier estimates of survival are related to the underlying
cause of cardiomyopathy; only idiopathic cardiomyopathy and
cardiomyopathy due to causes for which survival was significantly different
from that in patients with idiopathic cardiomyopathy are shown. The best
outcome is in those with a peripartum cardiomyopathy, and the worst
outcome is in those with an infiltrative cardiomyopathy or that due to HIV
infection.

Data from Felker CM, Thompson RE, Hare JM, et al. N Engl J Med 2000; 342:1077.

Graphic 75646 Version 2.0

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Contributor Disclosures
Wendy Tsang, MD No relevant financial relationship(s) with ineligible companies to disclose. Roberto M
Lang, MD Grant/Research/Clinical Trial Support: Philips Medical Imaging and Healthcare [Cardiac
imaging]. Consultant/Advisory Boards: Philips Medical Imaging and Healthcare [Cardiac imaging].
Speaker's Bureau: Philips Medical Imaging and Healthcare [Cardiac imaging]. All of the relevant financial
relationships listed have been mitigated. Candice Silversides, MD, MS, FRCPC No relevant financial
relationship(s) with ineligible companies to disclose. Nisha Parikh, MD, MPH No relevant financial
relationship(s) with ineligible companies to disclose.

Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are
addressed by vetting through a multi-level review process, and through requirements for references to be
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Conflict of interest policy

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