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围生期心肌病。

Peripartum cardiomyopathy.

机构信息

Cardiology Division, Department of Medicine, Massachusetts General Hospital, Boston, MA, 02114, USA.

Harvard Medical School, Boston, MA, 02115, USA.

出版信息

BMJ. 2019 Jan 30;364:k5287. doi: 10.1136/bmj.k5287.

Abstract

Peripartum cardiomyopathy (PPCM) is a rare, often dilated, cardiomyopathy with systolic dysfunction that presents in late pregnancy or, more commonly, the early postpartum period. Although the condition is prevalent worldwide, women with black ancestry seem to be at greatest risk, and the condition has a particularly high incidence in Nigeria and Haiti. Other risk factors include pre-eclampsia, advanced maternal age, and multiple gestation pregnancy. Although the complete pathophysiology of peripartum cardiomyopathy remains unclear, research over the past decade suggests the importance of vasculo-hormonal pathways in women with underlying susceptibility. At least some women with the condition harbor an underlying sarcomere gene mutation. More than half of affected women recover systolic function, although some are left with a chronic cardiomyopathy, and a minority requires mechanical support or cardiac transplantation (or both). Other potential complications include thromboembolism and arrhythmia. Currently, management entails standard treatments for heart failure with reduced ejection fraction, with attention to minimizing potential adverse effects on the fetus in women who are still pregnant. Bromocriptine is one potential disease specific treatment under investigation. In this review, we summarize the current literature on peripartum cardiomyopathy, as well as gaps in the understanding of this condition and future research directions.

摘要

围生期心肌病(PPCM)是一种罕见的、常为扩张型的、以收缩功能障碍为特征的心肌病,可发生于妊娠晚期或更常见于产后早期。尽管该病在全球范围内普遍存在,但具有黑人血统的女性似乎风险最高,该病在尼日利亚和海地的发病率尤其高。其他危险因素包括子痫前期、高龄产妇和多胎妊娠。尽管围生期心肌病的确切病理生理学仍不清楚,但过去十年的研究表明,血管 - 激素途径在具有潜在易感性的女性中很重要。至少有一些患有这种疾病的女性存在潜在的肌节基因突变。虽然超过一半的受影响的女性恢复了收缩功能,但有些女性仍患有慢性心肌病,少数女性需要机械支持或心脏移植(或两者兼有)。其他潜在的并发症包括血栓栓塞和心律失常。目前,管理包括心力衰竭伴射血分数降低的标准治疗,同时注意尽量减少对仍处于妊娠状态的胎儿的潜在不良影响。溴隐亭是正在研究的一种潜在的疾病特异性治疗方法。在这篇综述中,我们总结了围生期心肌病的现有文献,以及对这种疾病的理解和未来研究方向的差距。

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