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Eur J Heart Fail. 2019 Jul;21(7):827-843. doi: 10.1002/ejhf.1493. Epub 2019 Jun 27.

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.

Author information

1
Department of Cardiology and Angiology, Hannover Medical School, Hannover, Germany.
2
Department of Cardiology, University Medical Center Groningen, Groningen, The Netherlands.
3
Department of Cardiology, Institute of Cardiovascular and Medical Sciences, Glasgow University, Glasgow, UK.
4
Department of Medicine, College of Medicine, University of Lagos, Nigeria.
5
Department of Cardiology, Beirut Cardiac Institute, Lebanon.
6
Department of Cardiology and Cardiovascular Research Institute Basel (CRIB), University Hospital Basel, University of Basel, Switzerland.
7
Royal Brompton Hospital and Imperial College London, London, UK.
8
Department of Medicine, Karolinska Institutet and Heart and Vascular Theme, Karolinska University Hospital, Stockholm, Sweden.
9
Heart Failure Unit, Cardiology, G. da Saliceto Hospital, Piacenza, Italy.
10
Department of Cardiology, CARIM School for Cardiovascular Diseases, Faculty of Health, Medicine and Life Sciences, Maastricht University, Maastricht, The Netherlands.
11
Department of Cardiovascular Sciences, Centre for Molecular and Vascular Biology, Leuven, Belgium.
12
The Netherlands Heart Institute, Nl-HI, Utrecht, The Netherlands.
13
Institute of Emergency for Cardiovascular Disease, University of Medicine Carol Davila, Bucharest, Romania.
14
Division of Cardiology and Metabolism, Department of Cardiology (CVK), Berlin-Brandenburg Center for Regenerative Therapies (BCRT), German Centre for Cardiovascular Research (DZHK) Partner Site Berlin, Charité Universitätsmedizin Berlin, Berlin, Germany.
15
Department of Cardiology, Medical University, Clinical Military Hospital, Wroclaw, Poland.
16
University of Belgrade Faculty of Medicine and Heart Failure Center, Belgrade University Medical Center, Belgrade, Serbia.
17
Department of Obstetrics, Imperial College School of Medicine, Chelsea and Westminster Hospital, London, UK.
18
Department of Anesthesiology and Critical Care Medicine, AP-HP, Saint Louis Lariboisière University Hospitals, University Paris Diderot, Paris, France.
19
Hatter Institute for Cardiovascular Research in Africa, Department of Cardiology and Medicine, University of Cape Town, Cape Town, South Africa.

Abstract

Peripartum cardiomyopathy (PPCM) is a potentially life-threatening condition typically presenting as heart failure with reduced ejection fraction (HFrEF) in the last month of pregnancy or in the months following delivery in women without another known cause of heart failure. This updated position statement summarizes the knowledge about pathophysiological mechanisms, risk factors, clinical presentation, diagnosis and management of PPCM. As shortness of breath, fatigue and leg oedema are common in the peripartum period, a high index of suspicion is required to not miss the diagnosis. Measurement of natriuretic peptides, electrocardiography and echocardiography are recommended to promptly diagnose or exclude heart failure/PPCM. Important differential diagnoses include pulmonary embolism, myocardial infarction, hypertensive heart disease during pregnancy, and pre-existing heart disease. A genetic contribution is present in up to 20% of PPCM, in particular titin truncating variant. PPCM is associated with high morbidity and mortality, but also with a high probability of partial and often full recovery. Use of guideline-directed pharmacological therapy for HFrEF is recommended in all patients respecting contraindications during pregnancy/lactation. The oxidative stress-mediated cleavage of the hormone prolactin into a cardiotoxic fragment has been identified as a driver of PPCM pathophysiology. Pharmacological blockade of prolactin release using bromocriptine as a disease-specific therapy in addition to standard therapy for heart failure treatment has shown promising results in two clinical trials. Thresholds for devices (implantable cardioverter-defibrillators, cardiac resynchronization therapy and implanted long-term ventricular assist devices) are higher in PPCM than in other conditions because of the high rate of recovery. The important role of education and counselling around contraception and future pregnancies is emphasised.

KEYWORDS:

Heart failure; Peripartum cardiomyopathy; Pregnancy

PMID:
31243866
DOI:
10.1002/ejhf.1493

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