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Peripartum cardiomyopathy

Last updated: April 1, 2021

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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. [1]

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. [2][3]
  • Possible contributing factors
    • Genetic predisposition
    • Viral infections
    • Autoimmune processes
    • Nutritional deficiencies

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

Approach [1][2][3]

Diagnostic criteria [1]

All of the following criteria must be fulfilled to confirm a diagnosis of PPCM:

Diagnostic tests [1][3]

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 [2][3]
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.

Overview [2][3][4][5]

Initial management of heart failure

Acute decompensated PPCM [2][3]

Involve specialists early, as both the mother and fetus are at risk of adverse outcomes!

Hemodynamically stable patients [2][3][4]

  • 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 [2][3][4]
Class of drug Pregnancy Lactation
Loop diuretics
  • Use with caution.
  • Monitor the newborn/infant's hydration status and weight gain.
Beta blockers
ACE inhibitors
  • Contraindicated
Mineralocorticoid receptor antagonist
  • Contraindicated
Hydralazine/nitrates
  • Use with caution.
  • Use with caution.
Digoxin
  • Use with caution.
  • Use with caution.
ARBs
  • Contraindicated
  • Avoid.

ARNIs

Ivabradine

Anticoagulation in PPCM [3]

Indications and regimens [3]

Modifications for pregnancy or lactation

Warfarin is contraindicated in pregnancy.

Delivery [1][5]

  • 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. [3]
  • Acute decompensated PPCM (despite optimal medical therapy): Consider urgent cesarean delivery.
  • Stable PPCM: vaginal delivery at term, if feasible [4]

Breastfeeding [5]

  • 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 [2][4][7]

Contraception [3][7]

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

Subsequent pregnancies [2][3][7]

  • 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
  1. Arany Z, Elkayam U. Peripartum Cardiomyopathy. Circulation. 2016; 133 (14): p.1397-1409. doi: 10.1161/circulationaha.115.020491 . | Open in Read by QxMD
  2. Davis MB, Arany Z, McNamara DM, Goland S, Elkayam U. Peripartum Cardiomyopathy. J Am Coll Cardiol. 2020; 75 (2): p.207-221. doi: 10.1016/j.jacc.2019.11.014 . | Open in Read by QxMD
  3. Bauersachs J, König T, Meer P, et al. Pathophysiology, diagnosis and management of peripartum cardiomyopathy: a position statement from the Heart Failure Association of the European Society of Cardiology Study Group on peripartum cardiomyopathy. European Journal of Heart Failure. 2019; 21 (7): p.827-843. doi: 10.1002/ejhf.1493 . | Open in Read by QxMD
  4. Bozkurt B, Colvin M, Cook J, et al. Current Diagnostic and Treatment Strategies for Specific Dilated Cardiomyopathies: A Scientific Statement From the American Heart Association. Circulation. 2016; 134 (23). doi: 10.1161/cir.0000000000000455 . | Open in Read by QxMD
  5. The American College of Obstetricians and Gynecologists. ACOG Practice Bulletin No. 212: Pregnancy and Heart Disease. Obstetrics & Gynecology. 2019; 133 (5): p.e320-e356. doi: 10.1097/aog.0000000000003243 . | Open in Read by QxMD
  6. Kido K, Guglin M. Anticoagulation Therapy in Specific Cardiomyopathies: Isolated Left Ventricular Noncompaction and Peripartum Cardiomyopathy. J Cardiovasc Pharmacol Ther. 2018; 24 (1): p.31-36. doi: 10.1177/1074248418783745 . | Open in Read by QxMD
  7. Sliwa K, Petrie MC, Hilfiker‐Kleiner D, et al. Long‐term prognosis, subsequent pregnancy, contraception and overall management of peripartum cardiomyopathy: practical guidance paper from the Heart Failure Association of the European Society of Cardiology Study Group on Peripartum Cardiomyopathy. European Journal of Heart Failure. 2018; 20 (6): p.951-962. doi: 10.1002/ejhf.1178 . | Open in Read by QxMD
  8. Curtis KM, Tepper NK, Jatlaoui TC, et al. U.S. Medical Eligibility Criteria for Contraceptive Use, 2016. MMWR. Recommendations and Reports. 2016; 65 (3): p.1-103. doi: 10.15585/mmwr.rr6503a1 . | Open in Read by QxMD