Peripartum cardiomyopathy - National Heart, Lung, and Blood Institute and Office of Rare Diseases (National Institutes of Health) workshop recommendations and review
Peripartum cardiomyopathy - National Heart, Lung, and Blood Institute and Office of Rare Diseases (National Institutes of Health) workshop recommendations and review
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DOI：
10.1001/jama.283.9.1183
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发表时间：
2000-03-01
影响因子：
120.7
通讯作者：
Baughman, KL
中科院分区：
文献类型：
--
作者：
Pearson, GD;Veille, JC;Baughman, KL
围产期心肌病（PPCM）是一种罕见的危及生命的心肌病，发生在围产期的健康妇女，病因不明。1997年4月，美国国家心脏、肺和血液研究所（NHLBI）和美国国立卫生研究院（NIH）罕见疾病办公室召开了一次关于围产期心肌病的研讨会，以促进信息的系统回顾，并为研究和教育提出建议。一个由8名参与者和NHLBI工作人员组成的代表性小组组成了本文的写作小组，并更新了结论所依据的文献。研讨会是一个开放的会议，符合NIH的政策。证据数据在研讨会上提出了一个MEDLINE搜索1966年至1999年7月发表的英文文章，使用围产期心肌病，心肌病，妊娠。文章的流行病学，发病机制，病理生理学，诊断，治疗和预后的PPCM被included.Recommendation过程讨论后的数据，研讨会与会者同意的标准化定义的PPCM，一般的临床方法，并需要一个注册表，以提供一个基础设施，为未来的research.Conclusions围产期心肌病是一种罕见的致死性疾病，知之甚少。诊断仅限于一个狭窄的时期，需要超声心动图证据的左心室收缩功能障碍。有症状的患者应接受标准的心力衰竭治疗，由多学科团队管理。如果随后怀孕发生，他们应该与高风险的围产期中心合作管理。需要系统的数据收集来回答有关发病率、治疗和预后的重要问题。
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.