Peripartum cardiomyopathy

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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.

Cardiomyopathy characterized by the development of idiopathic HFrEF in the months after or before delivery. ^[2]

• Incidence: 1–4 in 4,000 deliveries ^[3][4]
• At-risk group: African Americans (account for > 40% of cases) ^[4][5]
• Risk factors ^[4][5]
  • Multiple gestation pregnancy
  • Age > 30 years
  • Hypertensive disorders of pregnancy
  • Prolonged use of beta-adrenergic agonists for tocolysis
  • Previous PPCM

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. ^[3][4]
• Possible contributing factors
  • Genetic predisposition
  • Viral infections
  • Autoimmune processes
  • Nutritional deficiencies

• Symptom onset
  • Most common in the first month after delivery
  • Can occur during pregnancy, usually in the 3^rd trimester
• Common manifestation: signs and symptoms of heart failure ^[2][4]
• Less common presentations
  • Cardiogenic shock
  • Thromboembolic event ^[3][4]

A high index of suspicion is essential for diagnosing PPCM as symptoms often overlap with normal pregnancy or the postpartum state!

Approach ^[2][3][4]

• 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).
  • Confirm systolic heart failure (HFrEF) on echocardiography.
  • Rule out other causes of heart failure or underlying heart disease.
  • Patients presenting with acute heart failure: See “Diagnostics” in “Acute heart failure.”
• In addition, obtain a biophysical profile to assess fetal well-being.

Diagnostic criteria ^[2]

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% ^[3]

Diagnostic tests ^[2][4]

• Echocardiography: indicated in all patients with suspected PPCM
  • Characteristic finding: reduced left ventricular ejection fraction < 45%
  • Other findings include varying degrees of:
    • Left and right ventricular dilatation
    • Biatrial enlargement
    • Functional mitral regurgitation and tricuspid regurgitation
    • Pulmonary hypertension
    • Left ventricular thrombus ^[4]
• Supportive findings of PPCM on other diagnostic tests
  • Chest x-ray: cardiomegaly, x-ray signs of pulmonary edema
  • Cardiac biomarkers: elevated BNP and troponin I ^[5]
  • ECG: variable findings (normal, sinus tachycardia, tachyarrhythmias)

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.

The differential diagnoses listed here are not exhaustive.

Overview ^[3][4][5][6]

• 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 ^[3][4]

• Urgent consults: critical care, cardiology, and obstetrics
• Urgent stabilization
  • Initiate management of acute heart failure.
  • 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.
• Further management
  • 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 ^[3][4][5]

• 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 “Use of heart failure medications during pregnancy and lactation.”

Anticoagulation in PPCM ^[4]

Indications and regimens ^[4]

• Confirmed thromboembolic event
  • Therapeutic anticoagulation is recommended.
  • Dosage and duration depend on the thromboembolic event (see, e.g., “Anticoagulation for PE”, “Anticoagulation for DVT”).
• LVEF ≤ 30–35%
  • It is unclear if prophylactic or therapeutic doses of anticoagulants should be used. ^[3][4][5]
  • Duration of anticoagulation: no consensus; usually through the rest of pregnancy and for 6–8 weeks postpartum ^[2][7]

Modifications for pregnancy or lactation

• Pregnancy: Use UFH or LMWH.
• Lactation: Use UFH, LMWH, or warfarin.

Warfarin is contraindicated in pregnancy.

Delivery ^[2][6]

• Important considerations
  • 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. ^[4]
• Acute decompensated PPCM (despite optimal medical therapy): Consider urgent cesarean delivery.
• Stable PPCM: vaginal delivery at term, if feasible ^[5]

Breastfeeding ^[6]

• 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 ^[3][5][8]

• 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
  • High-risk patients with LVEF < 35%: Consider a wearable cardioverter-defibrillator while awaiting a response to medical therapy.
  • Persistent, severe LV dysfunction despite ≥ 6 months of optimal medical therapy: Consider ICD with or without cardiac resynchronization therapy. ^[3][5]
• Heart transplantation: may be required if LV function remains poor despite medical therapy

• Urgently consult cardiology, obstetrics, neonatology, and intensive care.
• Confirm systolic heart failure (HFrEF) on echocardiography.
• Obtain BMP, electrolytes, CXR, ECG, cardiac biomarkers.
• Initiate management of acute heart failure (ensure appropriate use of heart failure medications during pregnancy and lactation).
  • Persistent hemodynamic instability despite optimal medical therapy
    • Consider urgent delivery.
    • Consider delaying breastfeeding until clinically stable.
  • Patient has been stabilized
    • Initiate medications for treatment of heart failure.
    • Co-manage with cardiology and obstetrics.
• Consider anticoagulation for PPCM.
• Evaluate for potential underlying causes of heart failure (e.g., preeclampsia, thyroid abnormalities).

Contraception ^[4][8]

• Rationale
  • 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. ^[4]
  • Estrogen-containing contraceptives are best avoided in the early postpartum period and in patients with severely reduced ejection fraction. ^[4][9]
• Options ^[4]
  • First-line: progestin-releasing IUDs, subdermal progestin implants, vasectomy, or tubal ligation
  • Second-line: depot medroxyprogesterone acetate
  • Third-line: progesterone-only pills
  • Fourth-line: barrier contraceptives
  • See “Contraception” for more information.

Patients must receive advice on contraception and the risk of further pregnancies before discharge!

Subsequent pregnancies ^[3][4][8]

• Preconception counseling
  • Persistent LV dysfunction (i.e., LVEF < 50%): Strongly advise against pregnancy because of the high risk of deterioration.
  • Recovered LV function (i.e., LVEF > 50%): Shared decision-making is advised in consultation with cardiology and obstetrics specialists. ^[4]
• Management of subsequent pregnancies
  • Closely monitor patients for PPCM recurrence during pregnancy and for at least one year postpartum.
  • Termination of pregnancy should be discussed in high-risk patients. ^[8]

• 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