TY - JOUR T1 - Sex and gender in pulmonary arterial hypertension JF - European Respiratory Review JO - EUROPEAN RESPIRATORY REVIEW DO - 10.1183/16000617.0330-2020 VL - 30 IS - 162 SP - 200330 AU - Céline Cheron AU - Susan Ainslie McBride AU - Fabrice Antigny AU - Barbara Girerd AU - Margot Chouchana AU - Marie-Camille Chaumais AU - Xavier Jaïs AU - Laurent Bertoletti AU - Olivier Sitbon AU - Jason Weatherald AU - Marc Humbert AU - David Montani Y1 - 2021/12/31 UR - http://err.ersjournals.com/content/30/162/200330.abstract N2 - 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.Pulmonary arterial hypertension affects women more than men, although women have a better prognosis. Oestrogens might be the key to explain these gender differences. https://bit.ly/36KK1yX ER -