医学
外显率
疾病
肺动脉高压
发病机制
胚胎血管重塑
怀孕
激素
心力衰竭
生理学
内科学
流行病学
生物信息学
心脏病学
表型
化学
基因
生物
生物化学
遗传学
作者
Céline Cheron,S. Ainslie McBride,Fabrice Antigny,Barbara Girerd,Margot Chouchana,Marie-Camille Chaumais,Xavier Jaïs,Laurent Bertoletti,Olivier Sitbon,Jason Weatherald,Marc Humbert,David Montani
标识
DOI:10.1183/16000617.0330-2020
摘要
Pulmonary arterial hypertension (PAH) is a rare disease characterised by pulmonary vascular remodelling and elevated pulmonary pressure, which eventually leads to right heart failure and death. Registries worldwide have noted a female predominance of the disease, spurring particular interest in hormonal involvement in the disease pathobiology. Several experimental models have shown both protective and deleterious effects of oestrogens, suggesting that complex mechanisms participate in PAH pathogenesis. In fact, oestrogen metabolites as well as receptors and enzymes implicated in oestrogen signalling pathways and associated conditions such as BMPR2 mutation contribute to PAH penetrance more specifically in women. Conversely, females have better right ventricular function, translating to a better prognosis. Along with right ventricular adaptation, women tend to respond to PAH treatment differently from men. As some young women suffer from PAH, contraception is of particular importance, considering that pregnancy in patients with PAH is strongly discouraged due to high risk of death. When contraception measures fail, pregnant women need a multidisciplinary team-based approach. This article aims to review epidemiology, mechanisms underlying the higher female predominance, but better prognosis and the intricacies in management of women affected by PAH.
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