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- heart failure
- cardiomyopathy dilated
- tissue Doppler
- peripheral vascular disease
- endomyocardial fibrosis
- arrhythmic right ventricular dysplasia
- cardiomyopathy restrictive
Peripartum cardiomyopathy (PPCM) is a relatively rare idiopathic form of heart failure that affects women during the last months of pregnancy or the first months after delivery. The aetiology and pathophysiological mechanisms of this disease are poorly characterised and incompletely understood. Diagnosis remains a challenge, as PPCM symptoms vary and may mimic those commonly experienced by women during pregnancy and postpartum due to normal physiological changes that occur during this period. The clinical course varies between complete recovery to rapid progression to end stage heart failure and even death. Standard heart failure treatment, with adjustments for women who are pregnant or lactating, is the treatment of choice. Disease specific therapeutic strategies, including prolactin blockade, show promise. National and international registries and collaborative research efforts are warranted to characterise this disease better and to develop novel treatments that can improve outcomes.
Heart failure during pregnancy has been recognised since the mid 19th century, but it was not until 1971 that Demakis and colleagues proposed diagnostic criteria for PPCM.w1 These diagnostic criteria have subsequently been revised by several groups1 2 w2 (table 1).
The common elements in all of the proposed criteria are that PPCM is a relatively rare idiopathic form of heart failure with left ventricular (LV) systolic dysfunction that occurs during the peripartum period.
The true incidence of PPCM is unknown, as clinical presentation varies and no systematic epidemiological studies have been conducted. Current estimates, ranging between 18 per 100 000 and 333 per 100 000 births, are primarily based on case series from single centres (figure 1).