Long-term outcome of patients with Eisenmenger syndrome receiving pulmonary arterial hypertension-targeted therapy.

ABSTRACT:

Background

Eisenmenger syndrome (ES) is a severe and fatal complication of uncorrected congenital heart disease characterised by the development of pulmonary arterial hypertension (PAH) due to chronic left-to-right shunting. Despite the increasing use of PAH-targeted therapies, evidence of their long-term effects on the survival of patients with ES remains limited.

Methods

We reviewed 101 consecutive adult patients with ES (69% female; mean age, 34±18 years) who were referred to our centre between December 1972 and December 2023. We investigated the clinical, haemodynamic and treatment data from medical records. The primary outcome measure was transplantation-free survival. The effect of PAH-targeted therapy on transplantation-free survival was analysed using a time-dependen

SUBMITTER: Matama H 

PROVIDER: S-EPMC12636896 | biostudies-literature | 2025 Nov

REPOSITORIES: biostudies-literature