Sex and gender in pulmonary arterial hypertension.
Eur Respir Rev
; 30(162)2021 Dec 31.
Article
in En
| MEDLINE
| ID: mdl-34750113
ABSTRACT
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.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Pulmonary Arterial Hypertension
/
Hypertension, Pulmonary
Type of study:
Diagnostic_studies
/
Prognostic_studies
Aspects:
Determinantes_sociais_saude
Limits:
Female
/
Humans
/
Male
/
Pregnancy
Language:
En
Journal:
Eur Respir Rev
Year:
2021
Document type:
Article