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Pathophysiology and epidemiology of peripartum cardiomyopathy

Key Points

  • Peripartum cardiomyopathy (PPCM) is defined as idiopathic systolic dysfunction in peripartum women

  • To make a diagnosis of PPCM, other possible causes of heart failure in peripartum women, such as genetic forms of dilated cardiomyopathy, need to be excluded

  • The incidence and prognosis of PPCM vary according to socioeconomic and genetic factors

  • The aetiology of PPCM is unknown; risk factors might include pre-eclampsia, twin pregnancies, and African ethnicity

  • A possible pathophysiological mechanism for PPCM is the production of a 16 kDa fragment of prolactin; blocking prolactin is, therefore, a potential therapeutic target

Abstract

Cardiovascular diseases are a major cause of complications in pregnancy worldwide, and the number of patients who develop cardiac problems during pregnancy is increasing. Peripartum cardiomyopathy (PPCM) is a potentially life-threatening heart disease that emerges towards the end of pregnancy or in the first months postpartum, in previously healthy women. Symptoms and signs of PPCM are similar to those in patients with idiopathic dilated cardiomyopathy. The incidence varies geographically, most likely because of socioeconomic and genetic factors. The syndrome is associated with a high morbidity and mortality, and diagnosis is often delayed. Various mechanisms have been investigated, including the hypothesis that unbalanced peripartum or postpartum oxidative stress triggers the proteolytic cleavage of the nursing hormone prolactin into a potent antiangiogenic, proapoptotic, and proinflammatory 16 kDa fragment. This theory provides the basis for the discovery of disease-specific biomarkers and promising novel therapeutic targets. In this Review, we describe the latest understanding of the epidemiology, pathophysiology, and novel treatment strategies for patients with PPCM.

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Figure 1: Time of onset of symptoms of peripartum cardiomyopathy according to country.
Figure 2: Pathophysiological mechanisms in PPCM.

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Acknowledgements

We thank Sylvia Dennis (Hatter Institute for Cardiovascular Research in Africa, University of Cape Town, South Africa) for proofreading the manuscript. The authors are supported by the Deutsche Forschungs Gesellschaft (DFG), the Bundesministeriums für Bildung und Forschung (BMBF), the National Research Foundation South Africa, and the Medical Research Foundation South Africa.

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Both authors researched data for the article, contributed substantially to discussion of its content, wrote the manuscript, and reviewed and edited it before submission.

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Correspondence to Denise Hilfiker-Kleiner.

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Hilfiker-Kleiner, D., Sliwa, K. Pathophysiology and epidemiology of peripartum cardiomyopathy. Nat Rev Cardiol 11, 364–370 (2014). https://doi.org/10.1038/nrcardio.2014.37

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