RT Journal Article
SR Electronic
T1 Sex and gender in pulmonary arterial hypertension
JF European Respiratory Review
JO EUROPEAN RESPIRATORY REVIEW
FD European Respiratory Society
SP 200330
DO 10.1183/16000617.0330-2020
VO 30
IS 162
A1 Cheron, Céline
A1 McBride, Susan Ainslie
A1 Antigny, Fabrice
A1 Girerd, Barbara
A1 Chouchana, Margot
A1 Chaumais, Marie-Camille
A1 Jaïs, Xavier
A1 Bertoletti, Laurent
A1 Sitbon, Olivier
A1 Weatherald, Jason
A1 Humbert, Marc
A1 Montani, David
YR 2021
UL http://err.ersjournals.com/content/30/162/200330.abstract
AB 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