[Peripartum cardiomyopathy: a little known disease]

Peripartum cardiomyopathy (PPCM) is a relatively rare cardiac disease that manifests itself in the final stage of pregnancy and in the first months after delivery in women with no previous history of cardiovascular disease. The incidence of PPCM varies widely across geographic areas and seems to be on the rise as a result of increased awareness and socioeconomic changes. PPCM recognizes a still partially undefined multifactorial etiology. Various pathogenetic hypotheses have been proposed, that range from autoimmune mechanisms to myocarditis to the hormonal hypothesis for aberrant, antiangiogenic and cardiotoxic prolactin (PRL) production, apoptosis, prolonged exposure to tocolytic drugs, malnutrition and genetic predisposition.The diagnosis of PPCM is still made by exclusion of other etiologies. Although some specific biomarkers with pregnancy-related kinetics have been proposed as diagnostic tools, their value remains questionable and they are not yet available in clinical practice.The prognosis of the disease is variable and not always predictable: both complete functional recovery and poor response to therapy and development of dilated cardiomyopathy and chronic heart failure (HF) may occur, although outcomes appears to have improved slightly in recent years.The acute phase of PPCM may require the use of inotropes and vasodilators as well as mechanical circulatory support and in some cases heart transplant may be indicated. Beta1-adrenergic agonists are contraindicated due to the possible induction of permanent damage due to loss of myocytes, with evolution towards chronic HF. The recent demonstration of the cardiotoxic effect of aberrant PRL has led to successful testing of the therapeutic effects of bromocriptine, a 2D dopamine agonist that blocks PRL. This treatment appears specific to PPCM, as it is targeted at 16k Da PRL, its mediator miR-146a and/or vascular endothelial growth factor.The long-term prognosis, once the acute phase is over, is a function of myocardial damage, and varies from complete functional recovery to chronic HF. Subsequent pregnancies always present a risk of recurrence and hence should be avoided. Even in cases with full functional recovery, relapses in the case of a new pregnancy may occur in 20% of cases. Women who wish a further pregnancy must be adequately informed and, in case of pregnancy, should undergo close monitoring. Treatment of chronic HF does not differ from that from other etiologies, according to international guidelines.