Peripartum cardiomyopathy: a systematic literature review

INTRODUCTION: Peripartum cardiomyopathy (PPCM) is a rare but potentially fatal disease defined by heart failure towards the end of pregnancy or in the months following delivery. We aim to raise awareness of the condition and give the clinician an overview of current knowledge on the mechanisms of pathophysiology, diagnostics and clinical management. MATERIAL AND METHODS: Systematic literature searches were performed in PubMed and Embase up to June 2016. Cohorts of more than 20 women with PPCM conducted after 2000 were selected to report contemporary outcomes and prognostic data. Guidelines and reviews that provided comprehensive overviews were included, too. RESULTS: New research on the pathophysiological mechanisms of PPCM points towards a two-hit multifactorial cause involving genetic factors and an antiangiogenic hormonal environment of late gestation with high levels of prolactin and sFlt-1. The prevalence of concomitant preeclampsia is high (often 30-45%) and symptoms can be similar, posing diagnostic difficulties. Most women (71-98%) present postpartum. Echocardiography is essential for diagnosis, and cardiac magnetic resonance imaging may provide new insights to pathophysiology and prognosis. Management is multidisciplinary and involves advanced heart failure therapy. Treatment, timing and mode of delivery in pregnant women depend on disease severity. The risk of relapse in subsequent pregnancies is >20%, and women are often advised against a new pregnancy. CONCLUSIONS: PPCM has a huge impact on cardiovascular health and reproductive life perspective. New insights into genetics, molecular pathophysiological mechanisms and clinical studies have resulted in potential disease-specific therapies, but many questions remain unanswered.