Peripartum cardiomyopathy - National Heart, Lung, and Blood Institute and Office of Rare Diseases (National Institutes of Health) workshop recommendations and review

Objective Peripartum cardiomyopathy (PPCM) is a rare life-threatening cardi omyopathy of unknown cause that occurs in the peripartum period in previous ly healthy women. In April 1997, the National Heart, Lung, and Blood Instit ute (NHLBI) and the Office of Rare Diseases of the National Institutes of H ealth (NIH) convened a Workshop on Peripartum Cardiomyopathy to foster a sy stematic review of information and to develop recommendations for research and education. Participants Fourteen workshop participants were selected by NHLBI staff an d represented cardiovascular medicine, obstetrics, immunology, and patholog y. A representative subgroup of 8 participants and NHLBI staff formed the w riting group for this article and updated the literature on which the concl usions were based. The workshop was an open meeting, consistent with NIH po licy. Evidence Data presented at the workshop were augmented by a MEDLINE search for English-language articles published from 1966 to July 1999, using the t erms 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 partici pants agreed on a standardized definition of PPCM, a general clinical appro ach, and the need for a registry to provide an infrastructure for future re search. Conclusions Peripartum cardiomyopathy is a rare lethal disease about which little is known. Diagnosis is confined to a narrow period and requires echo cardiographic evidence of left ventricular systolic dysfunction. Symptomati c patients should receive standard therapy for heart failure, managed by a multidisciplinary team. If subsequent pregnancies occur, they should be man aged in collaboration with a high-risk perinatal center. Systematic data co llection is required to answer important questions about incidence, treatme nt, and prognosis.