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Peripartum cardiomyopathy (PPCM) is the idiopathic development of heart failure with reduced ejection fraction (HFrEF) at the late stages of pregnancy or during the postpartum period. African American women are at an increased risk of developing PPCM and account for > 40% of all cases. PPCM typically manifests with signs and symptoms of heart failure, which can overlap with physiological signs of third-trimester pregnancy and the postpartum state. The diagnosis is based on a left ventricular ejection fraction (LVEF) of < 45% seen on echocardiography and the exclusion of other causes of heart failure. Management of PPCM is multidisciplinary and the early involvement of cardiologists, obstetricians, and neonatologists is recommended. The treatment of heart failure is similar to that of HFrEF due to other causes with necessary modifications to ensure that medications are safe for use during pregnancy and lactation. Patients with extremely low ejection fraction are at risk of developing a thromboembolic event and may require anticoagulation. Urgent delivery should be considered in hemodynamically unstable patients who do not respond to medical therapy. Response to optimal medical therapy is usually good. However, as there is a significant risk of recurrence in subsequent pregnancies, patients should be counseled on contraception.
- Incidence: 1–4 in 4,000 deliveries 
- At-risk group: African Americans (account for > 40% of cases) 
- Risk factors 
Epidemiological data refers to the US, unless otherwise specified.
- Idiopathic: The pathophysiology of PPCM is thought to be multifactorial, involving hormonal, metabolic, oxidative, and angiogenic imbalances. 
Possible contributing factors
- Genetic predisposition
- Viral infections
- Autoimmune processes
- Nutritional deficiencies
- Symptom onset
- Common manifestation: 
Less common presentations
A high index of suspicion is essential for diagnosing PPCM as symptoms often overlap with normal pregnancy or the postpartum state!
- PPCM is a diagnosis of exclusion.
- The diagnostic approach is the same as that for heart failure in nonpregnant patients (see “Diagnostics” in “Congestive heart failure” for details).
- In addition, obtain a biophysical profile to assess fetal well-being.
Diagnostic criteria 
All of the following criteria must be fulfilled to confirm a diagnosis of PPCM:
- New-onset systolic heart failure in the months following or preceding delivery
- Absence of other discernible causes of heart failure
- Absence of preexisting heart disease
- LVEF < 45% 
Diagnostic tests 
- Echocardiography: indicated in all patients with suspected PPCM
- Supportive findings of PPCM on other diagnostic tests
The symptoms of PPCM are nonspecific and overlap with a number of conditions that cause or mimic heart failure in the peripartum period (see table below). Some symptoms resemble the normal features of the third trimester of pregnancy, resulting in a delayed or missed diagnosis of PPCM.
|Differential diagnosis of peripartum cardiomyopathy |
|Conditions that can cause heart failure in the peripartum period||Conditions that can mimic acute heart failure in the peripartum period|
The differential diagnoses listed here are not exhaustive.
- Acute and long-term treatment of PPCM is similar to that of HFrEF due to other causes.
- Ensure medications are safe to use during pregnancy or lactation (see “Usage of heart failure medications during pregnancy and lactation”).
- Multidisciplinary care, involving cardiology, obstetric, neonatology, and intensivist expertise is recommended.
- Patients are at increased risk of thromboembolic events and may require anticoagulation (see “Anticoagulation in PPCM”).
Initial management of heart failure
Acute decompensated PPCM 
- Urgent consults: critical care, cardiology, and obstetrics
- Initiate .
- Ensure medications are safe for use in pregnancy or lactation (see “Use of heart failure medications during pregnancy and lactation”).
- No response to inotropes: Consider mechanical circulatory support.
- Identify and treat associated complications.
- Hemodynamically unstable despite optimal medical therapy: Consider urgent cesarean delivery.
- Initiate prophylactic measures to reduce the risk of complications in high-risk patients.
- Consider delaying breastfeeding until clinically stable.
Involve specialists early, as both the mother and fetus are at risk of adverse outcomes!
Hemodynamically stable patients 
- Management is similar to that in nonpregnant patients (see “Treatment of heart failure” for details and drug dosages).
- Ensure medications are safe for use in pregnancy or lactation (see table below).
|Usage of heart failure medications during pregnancy and lactation |
|Class of drug||Pregnancy||Lactation|
|ACE inhibitors|| |
|Mineralocorticoid receptor antagonist|| |
|Hydralazine/nitrates|| || |
|Digoxin|| || |
|ARBs|| || |
Anticoagulation in PPCM 
Indications and regimens 
Confirmed thromboembolic event
- Therapeutic anticoagulation is recommended.
- Dosage and duration depend on the thromboembolic event (see, e.g., “ ”, “ ”).
- LVEF ≤ 30–35%
Modifications for pregnancy or lactation
- The optimal timing and method of delivery should be determined in joint consultation with obstetrics and cardiology.
- Closely monitor hemodynamic and respiratory status during and following delivery. 
- Acute decompensated PPCM (despite optimal medical therapy): Consider urgent cesarean delivery.
- Stable PPCM: vaginal delivery at term, if feasible 
- Acute decompensated PPCM: Consider delaying breastfeeding until clinically stable.
- Stable PPCM: Breastfeeding is likely safe and should not be discouraged.
Modify heart failure medications as needed for breastfeeding patients. See “Usage of heart failure medications in pregnancy and lactation.”
Ongoing management of heart failure 
- Close follow-up: serial echocardiograms to assess for LV recovery
Medical treatment of heart failure
- Persistent LV dysfunction: life-long therapy recommended
- Recovered LV function: optimal duration of therapy unknown; consider continuing therapy indefinitely
- Device therapy
- Heart transplantation: may be required if LV function remains poor despite medical therapy
Acute management checklist
- Urgently consult cardiology, obstetrics, neonatology, and intensive care.
- Confirm systolic heart failure (HFrEF) on echocardiography.
- Obtain BMP, electrolytes, CXR, ECG, cardiac biomarkers.
- Initiate (ensure appropriate ).
- Consider .
- Evaluate for potential underlying causes of heart failure (e.g., preeclampsia, thyroid abnormalities).
Contraception and subsequent pregnancies
- The risk of PPCM recurrence and mortality with subsequent pregnancies is substantial.
- Contraception counseling is essential and should be done at the time of diagnosis or before hospital discharge.
- The choice of contraception is guided by efficacy, risk of adverse effects, and patient preference. 
- Estrogen-containing contraceptives are best avoided in the early postpartum period and in patients with severely reduced ejection fraction. 
- Options 
Subsequent pregnancies 
- Preconception counseling
- Management of subsequent pregnancies
- Higher rate of clinical and myocardial function recovery compared to other forms of HFrEF
- Good response to optimal medical therapy (LVEF recovery can occur within 3–6 months of diagnosis)
- Complete recovery less likely in patients with LVEF < 30%
- Significant risk of recurrence for all patients in subsequent pregnancies