The following are key points to remember from this state-of-the-art review on peripartum cardiomyopathy (PPCM):

1. PPCM is an idiopathic condition with left ventricular (LV) systolic dysfunction (ejection fraction [EF] <45%) towards the end of pregnancy or following delivery, when no other cause of heart failure (HF) is found.
2. In the United States, incidence varies from 1 in 1,000 to 1 in 4,000. Risk factors for PPCM include African ancestry, pre-eclampsia and hypertension, multifetal pregnancies, and older maternal age.
3. Etiology of PPCM is poorly understood. Existing studies suggest that PPCM is a vascular disease, triggered by peripartum hormonal changes with prolactin breakdown products having vasculotoxic effects. Genetic studies have identified pathogenic variants in the titin gene in afflicted patients supporting a two hit hypothesis.
4. Diagnosis is often delayed, as symptoms overlap those of normal pregnancy. Echocardiography is needed for diagnosis, and intracardiac thrombi should be ruled out when EF is severely reduced due to high risk for thromboembolism.
5. Predictors of adverse outcomes include EF at the time of diagnosis (EF <30% is associated with worse outcomes), African ancestry, obesity, pre-eclampsia, LV dilatation, right ventricular systolic dysfunction, and LV thrombus. PPCM is associated with higher recovery rates compared to other forms of HF, occurring as late as 2 years.
6. Management has to ensure fetal safety. During pregnancy and lactation, loop diuretics, beta-blockers, digoxin, and hydralazine/nitrates can be used. Renin-angiotensin-aldosterone inhibitors are contraindicated during pregnancy, but during lactation, enalapril, captopril, and spironolactone can be used.
7. Anticoagulation is endorsed by the American Heart Association if EF is <30% during late pregnancy and up to 8 weeks postpartum. Warfarin is contraindicated during pregnancy, but low molecular weight heparin can be used. Both of these agents are safe during lactation. Novel oral anticoagulants have not been studied during pregnancy and are best avoided.
8. The role of bromocriptine as a therapeutic agent is currently experimental. If used, therapeutic anticoagulation is recommended, as it is prothrombotic and it suppresses lactation.
9. PPCM is the most common cause of cardiogenic shock during or shortly after pregnancy. Nitroprusside should be avoided due to possible cyanide toxicity, and outcomes may be worse with dobutamine. Temporary mechanical support has been used successfully and should be considered early. Cardiac transplant in this population has been associated with higher rejection rates and lower survival.
10. Early delivery or termination of pregnancy should be considered in case of hemodynamic instability. Stable patients are delivered vaginally unless there are obstetric reasons for a cesarean section. Postpartum risk of decompensation should be anticipated.
11. Since prolactin has been implicated in the pathogenesis of PPCM, 2010 European guidelines on PPCM advise against breastfeeding. However, a small study in United States and data from an observational registry suggest that breastfeeding is safe.
12. Data on risk for arrhythmias are conflicted. It is reasonable to consider wearable defibrillators for women with new-onset PPCM and severe LV dysfunction until recovery or until an implantable cardioverter-defibrillator is indicated.
13. Contraception should be discussed as soon as feasible. Progesterone-releasing subcutaneous implants or Mirena intrauterine devices are first-line choices and estrogen should be avoided.
14. If LV dysfunction persists, medications should be continued indefinitely. In those with LV recovery, available observational data support continued therapy indefinitely. If HF medications are stopped, they should be weaned in a stepwise manner with frequent clinical and echocardiographic assessments.
15. Appropriate counseling should be provided for patients considering additional pregnancies. If LV dysfunction persists, women must be counseled about worse maternal and fetal outcomes. Women who recover to EF >50% still have an increased risk for HF, which may persist after pregnancy.
16. During subsequent pregnancies, women with PPCM should be closely followed with serial clinical assessments, echocardiograms, and B-type natriuretic peptide levels from prior to contraception until after delivery. Angiotensin-converting enzyme inhibitors/angiotensin-receptor blockers and aldosterone antagonists should be discontinued prior to conception and restarted after delivery.

Clinical Topics: Anticoagulation Management, Heart Failure and Cardiomyopathies, Noninvasive Imaging, Prevention, Acute Heart Failure, Heart Failure and Cardiac Biomarkers, Echocardiography/Ultrasound, Hypertension

Keywords: Anticoagulants, Cardiomyopathies, Echocardiography, Heart Failure, Hypertension, Intrauterine Devices, Lactation, Maternal Age, Natriuretic Peptide, Brain, Obesity, Peripartum Period, Postpartum Period, Pre-Eclampsia, Pregnancy, Prenatal Care, Secondary Prevention, Shock, Cardiogenic, Thromboembolism, Vascular Diseases, Ventricular Dysfunction, Left

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