Peripartum cardiomyopathy: Etiology, clinical manifestations, and diagnosis
- Wendy Tsang, MD
Wendy Tsang, MD
- Assistant Professor
- Toronto General Hospital
- University of Toronto
- Amy C Bales, MD
Amy C Bales, MD
- Assistant Professor of Clinical Medicine
- Midwestern University, Chicago College of Osteopathic Medicine
- Roberto M Lang, MD
Roberto M Lang, MD
- Professor of Medicine
- Pritzker School of Medicine of the University of Chicago
Peripartum cardiomyopathy (PPCM, also called pregnancy-associated cardiomyopathy) is a rare cause of heart failure (HF) that affects women late in pregnancy or in the early puerperium . Although initially described in 1849 , it was not recognized as a distinct clinical entity until the 1930s . Earlier terms for this condition include toxic postpartum HF, Meadows’ syndrome, Zaria syndrome, and postpartum myocardiosis.
This topic will discuss the etiology, clinical manifestations, and diagnosis of PPCM. Treatment and prognosis of PPCM, critical illness during pregnancy and the peripartum period, HF during pregnancy, and issues related to pregnancy in women with acquired or congenital heart disease are discussed separately. (See "Peripartum cardiomyopathy: Treatment and prognosis" and "Critical illness during pregnancy and the peripartum period" and "Management of heart failure during pregnancy" and "Acquired heart disease and pregnancy" and "Pregnancy in women with congenital heart disease: General principles".)
A variety of definitions have been used to identify peripartum cardiomyopathy (PPCM) [1,4-7]. We favor the definition developed by the 2010 European Society of Cardiology (ESC) Working Group on Peripartum Cardiology . The Working Group sought to avoid under-diagnosis of PPCM by adopting a broad definition (which was also included in the 2011 ESC guidelines on management of cardiovascular diseases during pregnancy) [1,7].
The 2010 ESC Working Group defined PPCM as an idiopathic cardiomyopathy with the following characteristics:
●Development of heart failure (HF) toward the end of pregnancy or in the months following delivery
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- Angiogenic imbalance
- Role of prolactin
- Inflammatory cytokines
- Abnormal immune response
- Genetic predisposition
- Hemodynamic factors
- Risk factors
- CLINICAL MANIFESTATIONS
- Timing of presentation
- Symptoms and signs
- Chest radiograph
- Cardiac magnetic resonance imaging
- Cardiac catheterization
- Endomyocardial biopsy
- DIFFERENTIAL DIAGNOSIS
- SUMMARY AND RECOMMENDATIONS