Elsevier

The Lancet

Volume 368, Issue 9536, 19–25 August 2006, Pages 687-693
The Lancet

Seminar
Peripartum cardiomyopathy

https://doi.org/10.1016/S0140-6736(06)69253-2Get rights and content

Summary

Peripartum cardiomyopathy (PPCM) is a disorder in which initial left ventricular systolic dysfunction and symptoms of heart failure occur between the late stages of pregnancy and the early postpartum period. It is common in some countries and rare in others. The causes and pathogenesis are poorly understood. Molecular markers of an inflammatory process are found in most patients. Clinical presentation includes usual signs and symptoms of heart failure, and unusual presentations relating to thromboembolism. Clinicians should consider PPCM in any peripartum patient with unexplained disease. Conventional heart failure treatment includes use of diuretics, β blockers, and angiotensin-converting enzyme inhibitors. Effective treatment reduces mortality rates and increases the number of women who fully recover left ventricular systolic function. Outcomes for subsequent pregnancy after PPCM are better in women who have first fully recovered heart function. Areas for future research include immune system dysfunction, the role of viruses, non-conventional treatments such as immunosuppression, immunoadsorption, apheresis, antiviral treatment, suppression of proinflammatory cytokines, and strategies for control and prevention.

Section snippets

Epidemiology

Although it seems likely that women of reproductive age all over the world have some risk of developing PPCM, good data about incidence are unavailable because so few population-based registries exist. Recent reports suggest an estimated incidence of one case per 299 livebirths in Haiti,11 one case per 1000 livebirths in South Africa,12 and one case per 2289 livebirths13 to one case per 4000 livebirths in the USA.2 These more recent data from the USA suggest a higher incidence than reported in

Aetiology

The cause and mechanism of pathogenesis of PPCM remain unknown, and many hypotheses have been proposed (figure 1).24, 25, 26, 27, 28, 29 Early suggestions that nutritional disorders, such as deficiencies in selenium and other micronutrients, might contribute could not be confirmed in studies of Haitian patients with PPCM,27 although unidentified nutritional factors might exist.

Because of immune-system changes related to pregnancy, associations with autoimmune mechanisms and inflammation have

Clinical presentation

The most common presentation of PPCM is with symptoms and signs of systolic heart failure.1, 8, 9, 12 Clinical examination of 97 patients seen in South Africa12 over 4 years revealed a displaced hypodynamic apical impulse in 72% and gallop rhythm in 92% of the patients. Functional mitral regurgitation was present in 43%. Left ventricular hypertrophy by electrocardiographic voltage criteria was present in 66% and ST-T wave abnormalities in 96% of the patients. Additional symptoms and signs

Management and prognosis

The medical management of patients with PPCM is similar to that for other forms of heart failure, and has been reviewed in detail.56 Treatment aims to reduce afterload and preload, and to increase contractility. Angiotensin-converting enzyme (ACE) inhibitors are usually used to reduce afterload by vasodilation if PPCM occurs after pregnancy. Because of potential toxic effects on the fetus, hydralazine (with or without nitrates) replaces ACE inhibitors during pregnancy. β blockers are used,

Implications for research

Reliable population-based information about incidence and prevalence of PPCM is essential to the development of national and international health policies for prevention and control of this condition. These should include a genetic epidemiological design to estimate the contribution of genetic susceptibility factors to this condition. While investigation continues on the molecular basis of PPCM, controlled trials are also needed for potential new treatments—including apheresis,

Search strategy and selection criteria

We aimed to summarise the results of a MEDLINE search (January, 1960, to January, 2006) on the current understanding of the epidemiology, aetiology, clinical profile, and management of PPCM. We used the search terms “peripartum cardiomyopathy”, “postpartum cardiomyopathy”, “heart failure in pregnancy”, and “heart failure postpartum”. We also searched the reference lists of articles identified by this strategy and selected those we judged relevant. Several review articles and book chapters

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