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Clinical Cardiology
March 1, 2000

Peripartum Cardiomyopathy: National Heart, Lung, and Blood Institute and Office of Rare Diseases (National Institutes of Health) Workshop Recommendations and Review

Author Affiliations

Author Affiliations: Division of Heart and Vascular Diseases, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Md (Dr Pearson); Department of Obstetrics and Gynecology, Bowman Gray School of Medicine, Winston-Salem, NC (Dr Veille); Division of Cardiology, University of Southern California, Los Angeles (Dr Rahimtoola); Division of Cardiology, George Washington University School of Medicine and Health Sciences, Washington, DC (Dr Hsia); Emeritus Professor of Cardiology, Imperial College Medical School, London, England (Dr Oakley); Division of Cardiovascular Medicine, Medical College of Wisconsin, Milwaukee (Dr Hosenpud); Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, Ga (Dr Ansari); and Division of Cardiology, Johns Hopkins University School of Medicine, Baltimore, Md (Dr Baughman).


Clinical Cardiology Section Editors: Bruce Brundage, MD, University of California, Los Angeles, School of Medicine; Margaret A. Winker, MD, Deputy Editor, JAMA.

JAMA. 2000;283(9):1183-1188. doi:10.1001/jama.283.9.1183

Objective Peripartum cardiomyopathy (PPCM) is a rare life-threatening cardiomyopathy of unknown cause that occurs in the peripartum period in previously healthy women. In April 1997, the National Heart, Lung, and Blood Institute (NHLBI) and the Office of Rare Diseases of the National Institutes of Health (NIH) convened a Workshop on Peripartum Cardiomyopathy to foster a systematic review of information and to develop recommendations for research and education.

Participants Fourteen workshop participants were selected by NHLBI staff and represented cardiovascular medicine, obstetrics, immunology, and pathology. A representative subgroup of 8 participants and NHLBI staff formed the writing group for this article and updated the literature on which the conclusions were based. The workshop was an open meeting, consistent with NIH policy.

Evidence Data presented at the workshop were augmented by a MEDLINE search for English-language articles published from 1966 to July 1999, using the terms peripartum cardiomyopathy, cardiomyopathy, and pregnancy. Articles on the epidemiology, pathogenesis, pathophysiology, diagnosis, treatment, and prognosis of PPCM were included.

Recommendation Process After discussion of data presented, workshop participants agreed on a standardized definition of PPCM, a general clinical approach, and the need for a registry to provide an infrastructure for future research.

Conclusions Peripartum cardiomyopathy is a rare lethal disease about which little is known. Diagnosis is confined to a narrow period and requires echocardiographic evidence of left ventricular systolic dysfunction. Symptomatic patients should receive standard therapy for heart failure, managed by a multidisciplinary team. If subsequent pregnancies occur, they should be managed in collaboration with a high-risk perinatal center. Systematic data collection is required to answer important questions about incidence, treatment, and prognosis.