Version May 2024
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].
This topic will discuss the etiology, clinical manifestations, and diagnosis of PPCM. Treatment and prognosis 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: Treatment and prognosis".)
●(See "Critical illness during pregnancy and the peripartum period".)
●(See "Management of heart failure during pregnancy".)
●(See "Acquired heart disease and pregnancy".)
●(See "Pregnancy in women with congenital heart disease: General principles".)
DEFINITION — A variety of definitions have been used to identify PPCM [1,4-7]. The definition developed by the 2010 European Society of Cardiology (ESC) Working Group on Peripartum Cardiology is the most widely used and has been included in the 2018 ESC guidelines on management of cardiovascular diseases during pregnancy and in the position statement from the Heart Failure Association of the European Society of Cardiology Study Group on PPCM [1,7,8]. The definition is broad, as the Working Group sought to avoid underdiagnosis of PPCM, and all three conditions must be met [8]:
●Development of HF in the last month of pregnancy (or toward the end of pregnancy) or within five months following delivery.
●Absence of another identifiable cause for the HF.
●Left ventricular (LV) systolic dysfunction with an LV ejection fraction (LVEF) of less than 45 percent, with or without LV dilation.
The last criterion was added to prevent the inclusion of patients with disorders with higher LVEF that mimic systolic HF [6,9]. Such disorders include accelerated hypertension, diastolic dysfunction, systemic infection, pulmonary embolism, or complications of late pregnancy (eg, preeclampsia or amniotic fluid embolus).
Some patients with higher LVEF (between 45 and 50 percent) may be diagnosed with PPCM if they have typical clinical features; in such patients, the diagnosis should be made after all other causes have been excluded [8]. Prior definitions also exclude cardiomyopathy that presented as HF before the last month of pregnancy [4-6], although the disease process is likely the same.
●One study of 123 females suggested that PPCM and earlier forms of pregnancy cardiomyopathy are likely the same disease [10]. One hundred women met the traditional criteria for PPCM, presenting at a mean of 38 weeks, and 23 presented earlier at a mean of 32 weeks. There were no differences between the two groups in terms of age, race, associated conditions, LVEF (29 versus 27 percent), the rate and time of recovery, and maternal outcomes.
EPIDEMIOLOGY
Global differences in incidence — A large prospective international registry of 411 women from 43 countries has demonstrated that PPCM occurs globally, affecting women from all ethnicities on all continents [11]. However, incidence rates vary widely depending on geographical location. Published incidences of PPCM range widely (figure 1) [1,5,9,12-23]:
●1:100 in Zaria, Nigeria
●1:300 in Haiti
●1:1000 in South Africa
●1:1000 to 1:4000 in the United States
●1:2400 in Canada
●1: 5719 in Sweden
●1:10,149 in Denmark
●1:20,000 live births in Japan
The incidence data for many other countries such as those in the Middle East, Asia, South America, and Australia are not well described. Also, the true incidence of PPCM may be higher, as milder forms may be missed.
The wide range in reported incidence may reflect an overestimation in studies that rely solely on clinical criteria to make the diagnosis (see 'Diagnosis' below). There may also be differences in search criteria and a lack of chart review when large national registries are used. Also, socioeconomic disparities between countries may contribute to some of these observed differences.
A genetic predisposition may contribute to geographical variability. The higher prevalence in Haiti supports the notion that females in the African diaspora have a higher risk of developing PPCM. Nonracial regional variations in PPCM also exist, as demonstrated in a large study that reported differences in women from the Kanto region compared with other areas in Nigeria [24,25].
The incidence of PPCM is also increasing [19]. This may be due to improved diagnosis and recognition but also increasing maternal age, preeclampsia, multiparity, multiple gestations, and maternal cardiovascular risk factors.
Risk factors — The mean age at PPCM presentation is 31 years, and the mean parity is 3 [11].
The following are factors associated with increased risk of PPCM:
●Age greater than 30 years [3,4,10,12].
●African descent [26].
●Pregnancy with multiple gestation [10,27].
●Prior or concurrent preeclampsia, eclampsia, or postpartum hypertension [28].
●Maternal cocaine abuse [29].
●Long-term (>4 weeks) oral tocolytic therapy with beta adrenergic agonists such as terbutaline [30].
●Parity ≥4 [31].
Although multiparity is associated with PPCM [31], studies have shown that the majority of patients who develop PPCM do so during the first or second pregnancy [10,32,33].
Some of the above risk factors (preeclampsia, hypertensive disorders in pregnancy, and cocaine cardiomyopathy) are themselves etiologies of HF in late pregnancy. Early studies of patients with PPCM have often excluded women with preeclampsia to avoid misclassification of patients. However, high incidences of preeclampsia are seen in patients with PPCM, suggesting that preeclampsia is associated with predisposition to PPCM through a shared pathophysiologic mechanism [11,28,34,35].
Diabetes has been reported as a risk factor for PPCM, but this relationship may be confounded by other risk factors such as hypertensive disorders during pregnancy, which is a more established risk factor for PPCM and is also often associated with diabetes [22].
There are conflicting data as to whether selenium deficiency is [36,37] or is not [38] a risk factor for PPCM [10,31-33].
Race-ethnic differences — While there are differences in incidence and severity among ethnic groups, a prospective worldwide registry found that mode of presentation and mean age were similar across ethnic, socioeconomic, and geographic backgrounds [11].
●United States – In the United States, African Americans have a higher prevalence of PPCM compared with White Americans and may also have more severe disease. In a series comparing 52 African Americans and 104 White Americans with PPCM, African American patients were younger, had a higher prevalence of gestational hypertension, and lower rates of recovery of ventricular function, which resulted in a higher rate of the combined endpoint of mortality and cardiac transplantation [39,40]. However, socioeconomic disparities may contribute to this observed difference.
●Canada – A retrospective study found that Aboriginal Canadian women with PPCM presented with lower LVEFs and larger LVs than other Canadian women with PPCM [11,41].
PATHOGENIC FACTORS — Whereas no single unifying cause for PPCM has been identified, several contributing pathogenic factors have been identified. Experimental research suggests that in the setting of pregnancy-related maternal cardiovascular changes, these multiple factors result in a common final pathway with enhanced oxidative stress, cleavage of prolactin to an angiostatic N-terminal 16 kDA prolactin fragment, and impaired vascular endothelial growth factor (VEGF) signaling because of upregulated soluble fms-like tyrosine kinase [1,9,34,42].
Angiogenic imbalance — Data from studies in mice and humans suggest that PPCM may be caused by systemic angiogenic imbalance via a lack of PGC-1α, a key regulator of pro-angiogenic VEGF. [28,34,43,44]. Hypertensive disorders in pregnancy may also contribute to the development of PPCM via angiogenic imbalance, particularly if preeclampsia is present.
Role of prolactin — Altered prolactin processing is believed to be involved in the pathogenesis of PPCM. The 16 kDa prolactin cleavage fragment (16K PRL) causes endothelial damage and myocardial dysfunction, possibly by inducing endothelial cell microRNA-146a expression [42]. Women with PPCM have elevated levels of microRNA-146a compared with healthy postpartum women or women with other cardiomyopathies [42].
Studies in mice suggest that PPCM can result from reduction in the transcription factor STAT3, which also increases one form of 16K PRL [45]. Reduced STAT3 may not be specific to PPCM, as it is seen in both dilated cardiomyopathy (DCM) and PPCM [46].
The potential role of prolactin as a target in the treatment of PPCM is discussed separately. (See "Peripartum cardiomyopathy: Treatment and prognosis", section on 'Bromocriptine or antiprolactin therapy'.)
Pregnancy-related hemodynamic changes — Normal pregnancy is considered a hemodynamic cardiovascular "stress test" such that an uncomplicated pregnancy is analogous to completing a normal stress test.
●The most marked hemodynamic stress occurs toward the end of pregnancy, during delivery, and early postpartum.
●Maternal cardiovascular physiologic changes include increased LV volumes, LV mass, and circulating blood volume; dilutional anemia; and shunting of up to 25 percent of circulating blood to the fetus and placenta.
●It has been hypothesized that women with specific genetic and/or other biologic susceptibilities may develop PPCM at the end of pregnancy or early postpartum, when there is more marked cardiovascular stress.
Other biologic factors
Genetic predisposition — Evidence from several studies supports the hypothesis that PPCM may develop as the combined result of pregnancy-related factors and a susceptible genetic background. The following observations support a genetic underpinning of PPCM:
●PPCM and DCM clustering – PPCM and DCM have been noted to cluster in families, and DCM-associated mutations have been identified in some patients with PPCM [47-49].
●Genetic variants – Variants in eight genes were shared in individuals with PPCM and DCM, suggesting shared genetics between these two conditions. The identified genetic variants were more common in PPCM and DCM than in a control population with no cardiomyopathy [50].
Two-thirds of identified truncating variants were in a gene (gene name TTN) that encodes the sarcomere protein titin that is known to be associated with DCM. The presence of a TTN-truncating variant was significantly correlated with lower LVEF at one-year follow-up. Thus, some patients with PPCM and these TTN-truncating variants may be presenting with an initial manifestation of familial DCM.
●Family history and worse LV recovery – Studies have reported that women with a family history of DCM have poorer recovery rates than those without a family history [47,49].
Interplay between genetics and race-ethnicity — A specific genetic variant linked to hypertension is more common in Black females and may underlie lower LVEF recovery among Black females with PPCM. The guanine nucleotide-binding protein beta-3 subunit (GNB3) has a polymorphism called C825T [51] that is associated with an increased risk of hypertension, low plasma renin, and cardiac remodeling [51]. It has a prevalence of 50 percent in Black individuals compared with 10 percent in White individuals. In a study of 97 women, the GNB3 TT genotype was associated with lower LVEF at 6 and 12 months in women with PPCM.
Immunologic factors
Inflammatory cytokines — Inflammatory cytokines may play a role in the pathogenesis and progression of cardiomyopathy and HF. The cytokines that are elevated in PPCM compared with controls include tumor necrosis factor alpha and interleukin-6 [52,53]. In addition, Fas/Apo-1, an apoptosis signaling receptor, and C-reactive protein are associated with more severe disease [53]. (See "Pathophysiology of heart failure with reduced ejection fraction: Hemodynamic alterations and remodeling", section on 'Other factors'.)
Maternal immune response to fetal microchimerism — The available data are both mixed and insufficient to establish whether abnormal maternal immunological response is the cause of PPCM. It has been suggested that a maternal immunologic response to a fetal antigen (also called fetal microchimerism) can lead to PPCM. Fetal cells may escape into the maternal circulation and remain there without being rejected due to weak immunogenicity of the paternal haplotype of the chimeric cells [54]. If these cells lodge in the cardiac tissue, they can trigger a pathologic autoimmune response [5].
There are high titers of autoantibodies (to cardiac myosin, adenine nucleotide translocator, and branched chain alpha ketoacid dehydrogenase) in PPCM compared with controls [5,55]. However, in a study of humoral immunity in 39 Nigerian women with PPCM, there was no difference in the levels of serum immunoglobulins, circulating immune complexes, or cardiac muscle antibodies between subjects and controls [56].
Infectious and environmental factors
Myocarditis — Though some investigators have suggested myocarditis as a possible cause of PPCM [57-61], the role of myocarditis in PPCM is uncertain [62].
Observations from small case series suggest a possible role for myocarditis:
●One study showed healing myocarditis in endomyocardial biopsies in 5 of 11 patients with PPCM. Absence of myocarditis on biopsy was associated with improvement in HF and LV remodeling [58].
●In two other series of 14 and 18 patients, respectively, myocarditis was present in 29 and 78 percent of patients with PPCM, respectively [59,60]. In comparison, myocarditis was present in 9 percent of 55 patients with non-pregnancy-related idiopathic cardiomyopathy [59]. Among 26 patients with PPCM who had evidence of interstitial inflammation, viral genomes were noted in eight (31 percent) [61]. Viral genomes have also been noted in other forms of myocarditis. (See "Myocarditis: Causes and pathogenesis", section on 'Viral or "idiopathic" myocarditis'.)
●In a nonrandomized study, three patients with PPCM and myocarditis were treated with prednisone and azathioprine and showed clinical improvement with no inflammatory infiltrate on repeat biopsy [57].
One study not supportive of myocarditis as a PPCM etiology:
●A retrospective review of endomyocardial biopsy specimens from 34 patients fulfilling the clinical criteria for a diagnosis of PPCM found a lower incidence of myocarditis (9 percent) than that reported in other studies [62]. This incidence was comparable to that found in an age- and sex-matched control population undergoing transplantation for idiopathic DCM (9.1 percent).
Study limitations that limit our ability to conclude whether myocarditis is a PPCM pathogenic factor include the following:
●Inclusion of patients outside the accepted time frame of PPCM.
●Variability among patient populations.
●Limitations of endomyocardial biopsy as a means of diagnosis of myocarditis.
The timing of biopsy in relation to the onset of symptoms may also be important, since the incidence of inflammation is greater in patients who are biopsied soon after presentation [60,62]. Other limitations include sampling error (since myocardial involvement may be patchy) and variability in histologic criteria for myocarditis (ie, whether patients with borderline myocarditis were included together with those with active myocarditis as defined by the Dallas criteria) [5].
CLINICAL MANIFESTATIONS
Timing of presentation — PPCM is less commonly seen before 36 weeks of gestation, and affected patients usually present during the first month postpartum [10,63,64]. Most women with PPCM are diagnosed early after delivery during readmission after discharge [8]. No significant differences in demographics, presentation, or hospital outcomes are noted between those who present during pregnancy or after delivery.
Pregnant women with other types of cardiac disease (eg, ischemic, valvular, or myopathic) may present earlier in the antepartum period, coincident with increases in the hemodynamic burden imposed by the gravid state during the second trimester, though they may also present during the third trimester or postpartum [65]. Thus, although late presentation during pregnancy can be helpful to identify women with PPCM, the entire clinical picture should be considered. (See "Maternal adaptations to pregnancy: Cardiovascular and hemodynamic changes" and "Acquired heart disease and pregnancy".)
Symptoms and signs — Presentation of PPCM is variable and similar to other forms of systolic HF due to cardiomyopathy [1]. Patients most commonly complain of dyspnea; other typical symptoms include cough, orthopnea, paroxysmal nocturnal dyspnea, pedal edema, and hemoptysis. Initial diagnosis may be delayed since symptoms such as nonspecific fatigue, shortness of breath, and pedal edema are similar to those that occur in normal pregnancy but more pronounced [4]. (See "Heart failure: Clinical manifestations and diagnosis in adults".)
Physical signs can include an elevated jugular venous pressure, a displaced apical impulse, a third heart sound, and a murmur of mitral regurgitation [66]. (See "Heart failure: Clinical manifestations and diagnosis in adults", section on 'Physical examination'.)
Signs and symptoms of systemic or pulmonary thromboembolism may be present. Case series have reported varying rates of thromboembolism [9,67-69], and further data are needed to quantify the risk of this complication [1]. Patients with PPCM and LVEF <35 percent are at risk for developing LV thrombus. As an example, LV thrombus was identified by echocardiography in 16 of 100 patients with PPCM (with mean LVEF of 26 percent) in one series [53]. (See "Epidemiology and pathogenesis of acute pulmonary embolism in adults" and "Overview of the evaluation of stroke".)
DIAGNOSIS
Criteria for diagnosis — As noted above, the diagnosis of PPCM is based upon three clinical criteria [4-6]: development of HF toward the end of pregnancy or in the months following delivery, absence of another identifiable cause of HF, and LV systolic dysfunction with an LVEF generally <45 percent [1]. (See 'Definition' above.)
Diagnostic investigations should be directed to timely diagnosis and treatment.
Diagnostic testing — An electrocardiogram (ECG) and echocardiogram should be performed in patients who are suspected of having PPCM. (See "Approach to diagnosis and evaluation of acute decompensated heart failure in adults".)
Electrocardiogram — An ECG should be performed in all patients with suspected PPCM to detect other conditions in the differential diagnosis such as myocardial infarction and pulmonary embolism. (See 'Differential diagnosis' below.)
ECG abnormalities may be found in up to 50 percent of patients with PPCM, but a normal ECG does not exclude PPCM [70]. ECG findings in patients with PPCM are nonspecific and include sinus tachycardia (or rarely atrial fibrillation) and nonspecific ST- and T-wave abnormalities. Anterior precordial Q waves and prolonged PR intervals and QRS duration are occasionally present [4,13]. (See "Approach to diagnosis and evaluation of acute decompensated heart failure in adults", section on 'Electrocardiogram'.)
Echocardiography — An echocardiogram should be performed in all patients in whom the diagnosis of PPCM is being considered. The echocardiogram generally reveals a global reduction in LV systolic function with LVEF nearly always <45 percent [1]. The LV is frequently but not always dilated (image 1 and image 2) [1]. Doppler assessment of right ventricular systolic pressures can usually also be performed, making right heart catheterization unnecessary in most patients.
Other possible echocardiographic findings include left atrial enlargement, LV or left atrial thrombus (image 1), dilated right ventricle, right ventricular hypokinesis, mitral and tricuspid regurgitation, and rarely small pericardial effusion [71]. (See "Approach to diagnosis and evaluation of acute decompensated heart failure in adults", section on 'Echocardiography'.)
Chest radiograph — A chest radiograph is not necessary to make a diagnosis of HF or PPCM, and it exposes the patient to ionizing radiation (table 1). If, despite a thorough physical examination, the diagnosis of pulmonary edema is uncertain, and a chest radiograph is deemed necessary to make that diagnosis, it can be considered and discussed with the pregnant patient, and fetal-shielding should be used. (See "Diagnostic imaging in pregnant and lactating patients".)
Chest radiograph typically shows enlargement of the cardiac silhouette with evidence of pulmonary venous congestion and/or interstitial edema, and, on occasion, pleural effusions. (See "Heart failure: Clinical manifestations and diagnosis in adults", section on 'Chest radiograph'.)
Plasma brain natriuretic peptide (BNP) or N-terminal pro-BNP (NT-proBNP) — measurement is suggested in the evaluation of patients with suspected HF, especially when the diagnosis is uncertain. Women presenting with PPCM typically have elevated BNP and NT-proBNP levels that are higher than those seen in healthy women during pregnancy or postpartum [72]. Measurement of BNP levels during pregnancy is discussed further separately. (See "Heart failure: Clinical manifestations and diagnosis in adults" and "Acquired heart disease and pregnancy", section on 'Brain natriuretic peptide' and "Natriuretic peptide measurement in heart failure".)
Other less frequently used tests
●Laboratory tests
•Viral and bacterial cultures, as well as viral titers (eg, Coxsackie B) are generally not indicated. The results of these tests are nonspecific and thus without proven value in patients with myocarditis. (See "Clinical manifestations and diagnosis of myocarditis in adults", section on 'Identifying the cause of myocarditis'.)
•Plasma concentrations of proangiogenic and antiangiogenic factors including placenta growth factor, fms-like-tyrosine-kinase 1 receptor, and their ratios have been proposed to be used to distinguish patients with PPCM. These studies are still very preliminary [34,44].
●Cardiac magnetic resonance imaging (CMR) – CMR is not generally required to make the diagnosis of PPCM, but it can be helpful to assess LV systolic function and LV volumes, particularly if echocardiography is technically suboptimal. Experience with CMR in PPCM is limited, and its role is still being evaluated [73-80]. The safety of these techniques in pregnancy and the importance of risk versus benefit in decision-making are discussed separately. (See "Diagnostic imaging in pregnant and lactating patients", section on 'Magnetic resonance imaging' and "Diagnostic imaging in pregnant and lactating patients", section on 'Use of gadolinium'.)
Case reports and small series have noted variable presence of late gadolinium enhancement (LGE) in patients with PPCM [75-80]. The presence and persistence of LGE may be associated with poor recovery of cardiac function [79]; improving LGE may be associated with cardiac recovery [76], while lack of LGE may be associated with presence or absence of cardiac recovery [78]. However, a later study could not confirm these findings [81]. (See "Clinical utility of cardiovascular magnetic resonance imaging", section on 'Late gadolinium enhancement'.)
●Cardiac catheterization – Invasive cardiac tests are usually not used to diagnose PPCM [82].
•Right heart catheterization – This is rarely needed because assessment of cardiac pressures can usually be made with physical examination and Doppler echocardiography. It may be helpful in critically ill patients who need more complete assessment or ongoing evaluation of their hemodynamic state.
•Left heart catheterization and angiography – Unlike new onset HF in the non-pregnant person, investigation for coronary artery disease as the primary cause of the cardiomyopathy is usually not necessary. Left heart catheterization with coronary angiography is only indicated in selected patients in whom coronary artery disease is highly suspected. (See "Non-ST-elevation acute coronary syndromes: Selecting an approach to revascularization", section on 'Signs of ongoing myocardial dysfunction or infarction'.)
Diagnostic coronary angiography exposes the patient to ionizing radiation (equivalent to approximately 100 or more chest radiographs) (table 1), and therefore it is important to carefully consider the appropriate timing of testing, to discuss the risks of fluoroscopy with the patient, and to employ fetal shielding if the procedure must be performed during pregnancy. (See "Diagnostic imaging in pregnant and lactating patients".)
•Endomyocardial biopsy – Similar to new onset HF in the non-pregnant patient, endomyocardial biopsy is generally not required in patients with suspected PPCM. There are no pathognomonic findings in PPCM. As noted above, a variable proportion of patients have evidence of myocarditis. Other histologic findings in PPCM can include myocardial hypertrophy and/or degeneration, fibrosis, and interstitial edema [4,12].
The decision as to whether or not to perform an endomyocardial biopsy should be left to the discretion of the physician and patient. The <1 percent risk of serious complications may not be reasonable given low clinical benefit from the findings.
Recommendations for endomyocardial biopsy in general are discussed separately. (See "Endomyocardial biopsy".)
Genetic testing — We often consider genetic counseling and testing in those with a positive family history of dilated cardiomyopathy, sudden death, or PPCM. This is because approximately 10 to 20 percent of women with HF in the peripartum period carry mutations known to induce cardiomyopathies [35,47-50,83]. 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. People with a genetic predisposition to PPCM may also have a genetic predisposition to other diseases such as cancer [84]. (See "Genetics of dilated cardiomyopathy".)
DIFFERENTIAL DIAGNOSIS — PPCM is a diagnosis of exclusion. Some pre-existing cardiac lesions may manifest during pregnancy due to pregnancy-associated hemodynamic changes (see "Acquired heart disease and pregnancy", section on 'Physiology of normal pregnancy'). As noted in the 2010 European Society of Cardiology (ESC) working group statement on PPCM, the following conditions should be considered in the differential diagnosis [1,85]:
●Pre-existing cardiomyopathy – Any cardiomyopathy may be unmasked during pregnancy including idiopathic dilated cardiomyopathy (DCM), familial DCM, or HIV/AIDS cardiomyopathy (which often presents without ventricular dilatation). In patients with a pre-existing cardiomyopathy, HF is more likely to manifest antepartum or early in pregnancy. In contrast, PPCM most commonly presents postpartum. (See "Acquired heart disease and pregnancy", section on 'Cardiomyopathy'.)
●Pre-existing valvular disease – Either acquired or valvular disease can be unmasked by pregnancy. These conditions often present in the antenatal period in contrast to PPCM, which generally presents in late pregnancy or postpartum. Valvular heart disease is diagnosed by physical examination and echocardiography. (See "Pregnancy and valve disease".)
•Mitral stenosis, most commonly from rheumatic heart disease, is more commonly seen in women from endemic regions.
•Aortic stenosis (usually from bicuspid or other congenital disease), aortic regurgitation (often from a bicuspid aortic valve), and mitral regurgitation are other valve lesions that cause HF during pregnancy.
•PPCM can also lead to functional valvular disease from chamber dilation, annular dilation, and leaflet tethering, which can impair leaflet coaptation. Mitral regurgitation can result from these changes in PPCM.
●Pre-existing undetected congenital heart disease – Aside from bicuspid valve disease, the most common congenital lesions that may be first diagnosed during pregnancy are atrial septal defects, ventricular septal defects, and patent ductus arteriosus. The clinical presentation and echocardiography are helpful in diagnosing these lesions. (See "Pregnancy in women with congenital heart disease: Specific lesions".)
●Diastolic HF due to hypertensive heart disease – This diagnosis is suggested by a prior history of severe hypertension and consistent findings on echocardiography. (See "Heart failure with preserved ejection fraction: Clinical manifestations and diagnosis", section on 'Evaluation'.)
●Myocardial infarction – Although myocardial infarction is rare in women of childbearing age, some studies have suggested an increased risk during pregnancy and during the early postpartum period. Causes of myocardial infarction during pregnancy include coronary artery dissection, coronary artery disease, coronary embolus/thrombosis (in a normal coronary artery), and coronary artery spasm. Risk factors include older maternal age, hypertension, diabetes mellitus, and obesity. Clinical manifestations include anginal chest pain, ECG changes, elevations in cardiac biomarkers, and regional wall motion abnormalities on echocardiography. (See "Acute myocardial infarction and pregnancy".)
●Pulmonary embolus – Pregnancy and the early postpartum period are associated with increased risk of venous thrombosis and pulmonary embolism, but diagnosis of pulmonary embolus can be challenging. The presence of dyspnea without evidence of HF favors the diagnosis of pulmonary embolus over PPCM. Pulmonary embolus can be diagnosed by lung scintigraphy or computed tomographic pulmonary angiography, as discussed separately. (See "Pulmonary embolism in pregnancy: Clinical presentation and diagnosis", section on 'Differential diagnosis'.)
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
●Definition – Peripartum cardiomyopathy (PPCM) is defined as the development of systolic heart failure (HF) toward the end of pregnancy or in the months following pregnancy, with left ventricular ejection fraction (LVEF) generally less than 45 percent in the absence of another identifiable cause of HF. (See 'Definition' above and 'Differential diagnosis' above.)
●Epidemiology – The prevalence of PPCM is highly country and region specific, ranging from 1 in 100 in Haiti, 1 in 1000 to 4000 in the United States, and 1 in 20,000 in Japan. Risk factors for PPCM include greater age, parity, multiple gestation, African descent, and a history of preeclampsia, eclampsia, or postpartum hypertension. (See 'Epidemiology' above.)
●Pathogenic factors – The etiology of PPCM is unknown, but the final common pathway includes angiogenic imbalance and altered prolactin processing. Possible causes include genetic, inflammatory, hormonal, hemodynamic, infectious, and autoimmune factors. (See 'Pathogenic factors' above.)
●Clinical manifestations – The clinical presentation of PPCM is variable and similar to new onset systolic HF due to cardiomyopathy in the non-pregnant person. Onset during late pregnancy and early postpartum are unique to PPCM. Common findings include dyspnea, lower extremity edema, increased jugular venous pressure, and crackles on lung exam. (See 'Clinical manifestations' above.)
●Diagnostic test findings – An electrocardiogram (ECG), echocardiogram, and brain natriuretic peptide (BNP) are performed in most patients. A chest radiograph showing pulmonary edema exposes the patient and fetus to ionizing radiation and is rarely necessary to confirm a diagnosis of PPCM. Other tests are less useful.
•ECG – This is usually nonspecific in PPCM showing sinus tachycardia but can help distinguish from other conditions such as acute myocardial infarction or right heart strain from a pulmonary embolus. (See 'Electrocardiogram' above.)
•Echocardiogram – This shows global reduction in LV systolic function, with LVEF nearly always <45 percent. The LV is frequently but not always dilated. (See 'Echocardiography' above.)
●Differential diagnosis – PPCM is a diagnosis of exclusion. Other causes of HF should be considered including other types of pre-existing cardiomyopathy, valvular heart disease, congenital heart disease, hypertension-related diastolic HF, myocardial infarction, and pulmonary embolism.
آیا می خواهید مدیلیب را به صفحه اصلی خود اضافه کنید؟
