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

1. Women with PPCM present with heart failure and left ventricular (LV) dysfunction late in pregnancy, during delivery, or in the first postpartum months. Women often have nonspecific symptoms and few physical signs; therefore, a low threshold of suspicion of heart failure is crucial.
2. Studies of gene mutations suggest a substantial overlap in genetic susceptibility between PPCM and other forms of inherited cardiomyopathy. Genetic counseling and testing may be considered when available.
3. Echocardiography is the main diagnostic modality to confirm LV dysfunction, identify complications such as intracardiac thrombi, and exclude alternative diagnoses such as congenital heart disease and primary valvular disease (other than functional mitral regurgitation, which may commonly occur in PPCM). Right ventricular dysfunction is an independent predictor of worse outcome.
4. If available, cardiac magnetic resonance imaging may be considered to exclude other etiologies such as myocarditis, LV noncompaction, or infiltrative diseases, and to supplement echocardiography if needed.
5. Factors associated with increased risk of complications at the time of diagnosis include non-Caucasian race, electrocardiogram QT-interval prolongation, LV ejection fraction <30%, LV end-diastolic diameter of >60 mm, biventricular dysfunction, and delay of diagnosis.
6. Treatment depends on the severity of heart failure and whether the patient is pregnant or post-delivery. A dedicated pregnancy heart team/task force for pregnant patients with heart failure can ensure rapid decision making and management.
7. During pregnancy, angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, and angiotensin receptor-neprilysin inhibitors are contraindicated due to fetal toxicity. Hydralazine and nitrates may be used instead.
8. Beta-blockers are indicated in all stable patients with marked systolic dysfunction during pregnancy and in all patients postpartum. Diuretics should be used as needed to reduce congestion.
9. The 2018 European Society of Cardiology guidelines for the management of cardiovascular disease during pregnancy state that bromocriptine may be considered in women with newly diagnosed PPCM, along with at least prophylactic anticoagulation to reduce thromboembolic risk. The North American Bromocriptine trial, funded by the National Institutes of Health, will further investigate bromocriptine versus placebo.
10. Outcomes vary depending on geographical regions. Globally, the average mortality rate is 2.4% at 1 month and 6% at 6 months. Early mortality is related to acute heart failure, malignant arrhythmias, cardiogenic shock, and thromboembolic events.

Clinical Topics: Anticoagulation Management, Arrhythmias and Clinical EP, Heart Failure and Cardiomyopathies, Noninvasive Imaging, Valvular Heart Disease, Implantable Devices, Genetic Arrhythmic Conditions, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Acute Heart Failure, Echocardiography/Ultrasound, Magnetic Resonance Imaging, Mitral Regurgitation

Keywords: Adrenergic beta-Antagonists, Anticoagulants, Arrhythmias, Cardiac, Bromocriptine, Cardiomyopathies, Diuretics, Echocardiography, Electrocardiography, Genetic Counseling, Genetic Predisposition to Disease, Heart Failure, Magnetic Resonance Imaging, Mitral Valve Insufficiency, Myocarditis, Peripartum Period, Postpartum Period, Pregnancy, Shock, Cardiogenic, Stroke Volume, Thromboembolism, Ventricular Dysfunction, Right, Women

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