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Peripartum cardiomyopathy: Treatment and prognosis - UpToDate
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Peripartum cardiomyopathy: Treatment and prognosis
Authors: Wendy Tsang, MD, Roberto M Lang, MD
Section Editor: Candice Silversides, MD, MS, FRCPC
Deputy Editor: Susan B Yeon, MD, JD, FACC
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Jul 2019. | This topic last updated: May 01, 2019.
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 similar to that employed for other types of HF with left ventricular 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
Treatment of peripartum cardiomyopathy (PPCM) is largely similar to treatment for other types of
heart failure (HF). Additional therapeutic issues for this population may include arrhythmia
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management, anticoagulation therapy, mechanical support, and investigational therapies such as
bromocriptine [4].
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, and aldosterone 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 peripartum cardiomyopathy who have delivered and are not breastfeeding, acute and
chronic HF are managed using standard therapy. (See "Treatment of acute decompensated heart
failure: Components of therapy" and "Overview of the therapy of heart failure with reduced ejection
fraction".)
Arrhythmia management — Arrhythmias are common in patients hospitalized for PPCM [5]. 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 rates between 20 and 25 percent [6,7]. Atrial fibrillation also occurs commonly and was
observed in 3.1 to 11.9 percent of patients with PPCM in various studies [6,8,9]. 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].
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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 [10]. Since up to 20 to 60
percent of women with PPCM have complete recovery of left ventricular ejection fraction (LVEF) to
normal by six months to five years (see 'Recovery of left ventricular function' below), ICD placement
should generally be deferred at least three months and possibly even 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 untested [7,11]. Furthermore, a wearable defibrillator is not
without its risks and concerns. (See "Primary prevention of sudden cardiac death in heart failure and
cardiomyopathy" 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 [12]. Cardiac resynchronization
therapy should generally be deferred until at least three months and possibly even 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".)
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 [13,14]. 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, and management during labor and delivery. 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'). One exception is for patients with PPCM receiving bromocriptine,
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as thromboembolic events (including stroke and myocardial infarction) have been reported as a
complication of bromocriptine use [15]. 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 [16]. (See 'Bromocriptine' below.)
Although data are limited, 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: Anticoagulant therapy to prevent thromboembolism".)
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 PPCM patients 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)
[16]. 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 PPCM patients [17]. 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 [18]. 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 [18].
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Outcomes were reported for 1258 women, which included 99 with PPCM, who had received durable
mechanical circulatory support [19]. 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 due to the fact that 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
"Intermediate- and long-term mechanical circulatory support" and "Practical management of long-term
mechanical circulatory support devices".)
Older studies found that transplantation was performed in up to one-third of women with PPCM [2022]. Contemporary reports demonstrate that transplantation rates vary from 4 to 23 percent of
patients [23-27]. Thus, women with PPCM and significant LV systolic dysfunction should be managed
at a center with transplant capabilities. (See "Indications and contraindications for cardiac
transplantation in adults".)
In addition to the potential maternal and fetal risks related to pregnancy after heart transplantation for
any reason (see "Pregnancy after cardiac 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 485 patients from the UNOS database and found worse
long-term survival in patients transplanted for PPCM compared with all others undergoing
transplantation [27]. 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. 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 suggest not routinely using
bromocriptine for patients with PPCM. Some other experts advocate using bromocriptine routinely in
this setting. The 2018 European Society of Cardiology (ESC) guidelines for management of
cardiovascular diseases during pregnancy included a weak recommendation for bromocriptine use in
this setting, stating that in patients with PPCM, "bromocriptine treatment may be considered to stop
lactation and enhance recovery" of LV function [28]. However, there are conflicting expert opinions
regarding the efficacy of bromocriptine use as well as the role of breastfeeding in this setting (as
noted below). (See 'Breastfeeding' below.)
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This treatment strategy is based upon an experimental observation of prevention of PPCM in mice via
prolactin blockade with bromocriptine [29]. A small randomized pilot study and several observational
reports have suggested a beneficial response to bromocriptine therapy in patients with PPCM [4,3033]. 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 [34].
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 longterm bromocriptine (eight weeks: 5 mg for two weeks followed by 2.5 mg for six weeks) in addition to
standard HF therapy [35]. 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, left ventricular 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. (See 'Maternal outcome'
below.)
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 [28]. (See
'Antithrombotic therapy' above.)
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 [20], 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 [36]. These drugs often
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have significant side effects, and studies in other forms of myocarditis have not shown clear benefit
from immunosuppressive therapy [37]. (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) [38]. 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'.)
DELIVERY
Limited data are available to guide the timing and mode of delivery in peripartum cardiomyopathy
(PPCM). Decisions regarding timing and mode of delivery should be based on combined input from
the cardiology, obstetrics, anesthesiology, and neonatology services [1]. In this regard,
multidisciplinary conferences are often useful.
In women with PPCM with advanced heart failure (HF), we suggest prompt delivery for maternal
cardiovascular indications. Urgent delivery may be required in women with advanced HF with
hemodynamic instability [1]. Planned cesarean delivery is preferred for women with advanced HF
requiring inotropic therapy or mechanical circulatory support [1,39].
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'.)
BREASTFEEDING
Some experts, including the 2010 European Society of Cardiology working group, suggest that
breastfeeding be avoided because of the potential effects of prolactin subfragments [40] (see
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"Peripartum cardiomyopathy: Etiology, clinical manifestations, and diagnosis", section on 'Role of
prolactin'). However, one 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 left ventricular 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 stable should not be discouraged from breastfeeding as long as it is compatible with their
heart failure medications [41].
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 peripartum cardiomyopathy (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 [42] 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 left ventricular (LV) dysfunction or LV ejection fraction
(LVEF) ≤25 percent at diagnosis are at high risk of recurrent PPCM, we suggest avoiding future
pregnancy in such patients [1]. We suggest that the patient or her partner undergo a sterilization
procedure or the patient use a highly effective non-estrogen method of contraception, such as the
etonogestrel implant, a copper intrauterine device (IUD), or levonorgestrel-releasing IUD. Depot
medroxyprogesterone acetate is not as highly effective, so it is considered a second line alternative.
Though the risk of recurrence appears to be less in women with PPCM with recovered LV function
and LVEF >25 percent at diagnosis, such patients should receive counseling, including the option of
avoidance of subsequent pregnancy due to the risk of relapse of PPCM, heart failure (HF), and death.
Estrogen-progestin contraceptives (eg, pills, patch, vaginal ring) may increase fluid retention, which
may worsen HF. In general, estrogen-progestin contraceptives should be avoided, particularly early
after diagnosis and in women with persistent LV dysfunction because of their potential to increase the
risk of thromboembolism [42,43].
THERAPY AFTER RECOVERY OF LEFT VENTRICULAR FUNCTION
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Peripartum cardiomyopathy patients with persistent left ventricular (LV) dysfunction should be
continued on standard heart failure (HF) treatment indefinitely. A subset of patients with peripartum
cardiomyopathy will achieve full recovery of LV function (ejection fraction [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 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, as follows [44]. 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
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The prognosis of peripartum cardiomyopathy (PPCM) includes maternal, obstetric, and neonatal
outcomes, and the effect of subsequent pregnancy.
Maternal outcome — Several studies have evaluated the outcome of women with PPCM [10,2325,45-49]. Whether women with PPCM have a different prognosis than pregnant women with other
forms of cardiomyopathy is not clear. (See "Acquired heart disease and pregnancy", section on
'Cardiomyopathy'.)
Mortality and morbidity — The mortality rate for PPCM has been reported as approximately 10
percent in two years [23], with rates ranging from 6 percent in five years (figure 1) [46] and 11 percent
in three years [10] to as high as 28 percent in a report of 29 black patients [45]. Cardiac
transplantation rates of less than 1 to 2 percent per year have been reported [10,23,24].
Death due to PPCM is usually caused by 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) [50]
●
Left ventricular ejection fraction (LVEF) ≤25 percent [32]
●
Black race [39,45]
●
Indigent status [51]
●
Multiparity [39]
●
Age greater than 30 to 35 years [52,53]
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 [54]. 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 [55]. Complete recovery of LV function (defined as recovery to an LVEF >50 percent)
has been reported in 20 to 60 percent of patients in various series [10,23,45,55-57]. Although nearly
all recovery of LV function occurred within six months of diagnosis in some series [23,45,58], delayed
recovery of LV function has been observed in other studies [9,10,51,59]. In one series of 100 patients,
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42 women showed partial or complete improvement in LVEF occurring over months to five years [10].
In this series, only 4 of 23 women who eventually had complete recovery achieved this within six
months.
Various studies have identified the following predictors of persistent LV dysfunction at follow-up:
●
LVEF ≤30 percent [23]
●
Fractional shortening less than 20 percent and an LV end-diastolic dimension ≥6 cm [60]
●
Elevated cardiac troponin T [61]
●
Black race [10,55]
●
Diagnosis during pregnancy [10]
●
Reduced right ventricular function measured using fractional area change on echocardiography
[62] or volumes on cardiac magnetic resonance imaging [63]
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
[56]. Small preliminary studies of the value of dobutamine stress echocardiography to predict
recovery of LV function have yielded mixed results [64,65].
The impact of preeclampsia or hypertension on prognosis of PPCM is unclear. Some studies have
suggested that PPCM associated with preeclampsia or hypertension may have better outcomes
[32,57,66]; it has been postulated that PPCM may have developed because of these conditions, and
therefore resolution of these conditions would facilitate recovery from PPCM. In contrast, the
Investigations of Pregnancy-Associated Cardiomyopathy (IPAC) study found that hypertension and
preeclampsia were not associated with improved outcomes among patients with PPCM [67].
Obstetric and neonatal outcomes — Data are more limited on obstetric and fetal outcomes. In the
above report of 123 patients, cesarean delivery was performed in 40 percent of patients, largely for
obstetric indications [23]. 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
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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. (See 'Contraception' above.)
Recovered LV function — Among women in whom LV function returns to normal, the risk of
subsequent pregnancy appears lower than for those with persistent LV dysfunction, but elevated
compared with the general population [68,69]. 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 [68]:
●
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 [49]. 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) [68]. 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 [70].
●
A more complex pattern was illustrated in a review of 15 women with PPCM, 14 of whom had
incomplete LV recovery [71]. 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
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improvement and normalization of LV function (LVEF ≥50 percent) within 30 months of the
subsequent pregnancy.
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 heart failure (HF) had higher rates of
recovery and higher LVEFs compared with those who were treated with standard HF therapy alone
[72]. However, further studies are needed in order 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".)
SUMMARY AND RECOMMENDATIONS
●
The management of heart failure (HF) due to peripartum cardiomyopathy (PPCM) is similar to
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'.)
●
Decisions regarding use of implantable cardioverter defibrillator and cardiac resynchronization
therapy in patients with PPCM should include consideration of the natural history of the disease,
including the potential of recovery of ventricular function. (See 'Device therapy' above.)
●
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 suggest not routinely using
bromocriptine for patients with PPCM (Grade 2C). Some other experts advocate using
bromocriptine routinely in this setting. (See 'Bromocriptine' above.)
●
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 PPCM with advanced HF. (See 'Delivery' above.)
●
The limited available data suggest that the risk of recurrence with subsequent pregnancy is
highest among women with persistent left ventricular (LV) systolic dysfunction, although women
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with recovered LV systolic function are also at risk for recurrence. (See 'Subsequent pregnancy'
above.)
●
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) or LV ejection fraction ≤25 percent at diagnosis 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 editorial staff at UpToDate would like to acknowledge Amy Bales, MD, who contributed to earlier
versions of this topic review.
Use of UpToDate is subject to the Subscription and License Agreement.
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Topic 95071 Version 11.0
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GRAPHICS
NYHA and other classifications of cardiovascular disability
Class
NYNA functional
classification [1]
Canadian
Cardiovascular
Society functional
classification [2]
Specific activity
scale [3]
I
Patients with cardiac
disease but without
resulting limitations of
physical activity. Ordinary
physical activity does not
cause undue fatigue,
palpitation, dyspnea, or
anginal pain.
Ordinary physical activity,
such as walking and
climbing stairs, does not
cause angina. Angina with
strenuous or rapid
prolonged exertion at work
or recreation.
Patients can perform to
completion any activity
requiring ≥7 metabolic
equivalents (ie, can carry
24 lb up 8 steps; do
outdoor work [shovel snow,
spade soil]; do recreational
activities [skiing,
basketball, squash,
handball, jog/walk 5 mph]).
II
Patients with cardiac
disease resulting in slight
limitation of physical
activity. They are
comfortable at rest.
Ordinary physical activity
results in fatigue,
palpitation, dyspnea, or
anginal pain.
Slight limitation of ordinary
activity. Walking or climbing
stairs rapidly, walking
uphill, walking or stairclimbing after meals, in
cold, in wind, or when
under emotional stress, or
only during the few hours
after awakening. Walking
more than 2 blocks on the
level and climbing more
than 1 flight of ordinary
stairs at a normal pace and
in normal conditions.
Patients can perform to
completion any activity
requiring ≥5 metabolic
equivalents (eg, have
sexual intercourse without
stopping, garden, rake,
weed, roller skate, dance
fox trot, walk at 4 mph on
level ground), but cannot
and do not perform to
completion activities
requiring ≥7 metabolic
equivalents.
III
Patients with cardiac
disease resulting in marked
limitation of physical
activity. They are
comfortable at rest. Lessthan-ordinary physical
activity causes fatigue,
palpitation, dyspnea, or
anginal pain.
Marked limitation of
ordinary physical activity.
Walking 1 to 2 blocks on
the level and climbing 1
flight in normal conditions.
Patients can perform to
completion any activity
requiring ≥2 metabolic
equivalents (eg, shower
without stopping, strip and
make bed, clean windows,
walk 2.5 mph, bowl, play
golf, dress without
stopping) but cannot and
do not perform to
completion any activities
requiring >5 metabolic
equivalents.
IV
Patients with cardiac
disease resulting in inability
to carry on any physical
activity without discomfort.
Symptoms of cardiac
insufficiency or of the
anginal syndrome may be
present even at rest. If any
physical activity is
undertaken, discomfort is
increased.
Inability to carry on any
physical activity without
discomfort. Anginal
syndrome may be present
at rest.
Patients cannot or do not
perform to completion
activities requiring >2
metabolic equivalents.
Cannot carry out activities
listed above (specific
activity scale III).
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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 16.0
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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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