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 (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 — Treatment of PPCM is largely similar to 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].
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 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 — Some experts perform genetic testing in women with PPCM, especially those with a positive family history of dilated cardiomyopathy or PPCM [5]. This is because approximately 15 to 20 percent of women with peripartum HF carry mutations known to induce cardiomyopathies [6-10]. 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. (See "Genetics of dilated cardiomyopathy".)
Arrhythmia management — Arrhythmias are common in patients hospitalized for PPCM [11]. 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 [12,13]. Atrial fibrillation also occurs commonly and was observed in 3.1 to 11.9 percent of patients with PPCM in various studies [12,14,15]. 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 [16]. 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) [17]. 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 unclear [13,18]. A registry study has demonstrated that wearable defibrillators do deliver appropriate shocks for ventricular fibrillation within the first months after diagnosis [13]. However, a wearable defibrillator is not without its risks and concerns. (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 [19]. 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 and choice of system".)
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 [20,21]. 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 [22]. Thromboembolic events (including stroke and myocardial infarction) have been reported as a complication of bromocriptine use in patients with PPCM [23]. (See 'Bromocriptine' below.)
However, some experts routinely anticoagulate patients with PPCM with severe LV systolic dysfunction [5,24].
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 in adults: Use of oral anticoagulants".)
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) [22]. 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 [25]. 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 [26].
Outcomes were reported for 1258 women, which included 99 with PPCM, who had received durable mechanical circulatory support [27]. 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 "Treatment of advanced heart failure with a durable mechanical circulatory support device" and "Management of long-term mechanical circulatory support devices".)
Older studies found that transplantation was performed in up to one-third of women with PPCM [28-30]. Contemporary reports demonstrate that transplantation rates vary from 4 to 23 percent of patients [31-35]. 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 485 patients from the UNOS database and found worse long-term survival in patients transplanted for PPCM compared with all others undergoing transplantation [35]. 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 needed to establish safety and 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 [24]; in the prior North American IPAC study, only 1 of 100 patients was treated with bromocriptine [17]. In contrast, some European experts suggest bromocriptine use in this setting, 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 [36]. 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].
Use of bromocriptine in this setting is based upon an experimental observation of prevention of PPCM in mice via prolactin blockade with bromocriptine [37]. 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 [4,38-43]. 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 [44].
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 [45]. 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. (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 [36]. (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 [46,47].
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 [28], 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 [48]. These drugs often have significant side effects, and studies in other forms of myocarditis have not shown clear benefit from immunosuppressive therapy [49]. (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) [50]. 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 [51]. 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 [52]. 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-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 [1,5,53]. (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'.)
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 [36]. (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 stable should not be discouraged from breastfeeding as long as it is compatible with their HF medications [54].
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 [55] 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 [5,36]. 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 [55,56].
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 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 [57]. 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 [16,17,31-33,58-62]. (See "Acquired heart disease and pregnancy", section on 'Cardiomyopathy'.)
Mortality and morbidity — The mortality rate for PPCM varies with region [63,64]. It has been reported as approximately 10 percent in two years [31], with rates ranging from 6 percent in five years (figure 1) [59] and 11 percent in three years [16] to as high as 28 percent in a report of 29 Black patients [58]. In the IPAC study, there were 4 deaths among 91 women at one year postpartum [17]. Cardiac transplantation rates of less than 1 to 2 percent per year have been reported [16,31,32].
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) [65]
●LVEF ≤25 percent [40]
●Being from a Black population [53,58]
●Indigent status [66]
●Multiparity (ie, having given birth two or more times) [53]
●Age greater than 30 to 35 years [67,68]
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 [69]. 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 [70]. Complete recovery of LV function (defined as recovery to an LVEF >50 percent) has been reported in 20 to 70 percent of patients in various series [16,17,31,58,70-72]. Although nearly all recovery of LV function occurred within six months of diagnosis in some series [31,58,73], delayed recovery of LV function has been observed in other studies [15,16,66,74]. In one series of 100 patients, 42 women showed partial or complete improvement in LVEF occurring over months to five years [16]. In this series, only 4 of 23 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 [69,75].
Various studies have identified the following predictors of persistent LV dysfunction at follow-up:
●LVEF ≤30 percent [31]
●Fractional shortening less than 20 percent and an LV end-diastolic dimension ≥6 cm [76]
●Elevated cardiac troponin T [77]
●Being from a Black population [16,70]
●Diagnosis during pregnancy [16]
●Reduced right ventricular function measured using fractional area change on echocardiography [78] or volumes on cardiac magnetic resonance imaging [79]
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 [71]. Small preliminary studies of the value of dobutamine stress echocardiography to predict recovery of LV function have yielded mixed results [80,81].
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 [31]. 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 [82]. (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 dysfunction, but elevated compared with the general population [83-85]. 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 [83]:
●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 [62]. 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) [83]. 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 [86].
●A more complex pattern was illustrated in a review of 15 women with PPCM, 14 of whom had incomplete LV recovery [87]. 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.
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 [88]. 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" and "Society guideline links: Management of cardiovascular diseases during pregnancy".)
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 the timing and mode of delivery in PPCM should be made based upon combined input from the cardio-obstetric team including cardiology, obstetrics, anesthesiology, and neonatology services. Prompt delivery is suggested in women with PPCM with advanced HF and hemodynamic instability despite medical therapy. (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. If bromocriptine is used, anticoagulation should also be started to prevent thromboembolic events. (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 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.)
●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.
