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Article Dans Une Revue European Respiratory Review Année : 2021

Sex and gender in pulmonary arterial hypertension

Susan Ainslie Mcbride
  • Fonction : Auteur
Barbara Girerd
  • Fonction : Auteur
Margot Chouchana
  • Fonction : Auteur
Marie-Camille Chaumais
Xavier Jaïs
  • Fonction : Auteur
Olivier Sitbon
Jason Weatherald
Marc Humbert
David Montani

Résumé

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.

Dates et versions

hal-04508470 , version 1 (18-03-2024)

Identifiants

Citer

Céline Cheron, Susan Ainslie Mcbride, Fabrice Antigny, Barbara Girerd, Margot Chouchana, et al.. Sex and gender in pulmonary arterial hypertension. European Respiratory Review, 2021, 30 (162), pp.200330. ⟨10.1183/16000617.0330-2020⟩. ⟨hal-04508470⟩
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