10-year survival of pulmonary arterial hypertension associated with connective tissue disease: insights from a multicentre PAH registry.

ABSTRACT:

Objectives

To report the 10-year survival rate and prognostic factors of pulmonary arterial hypertension associated with CTD (CTD-PAH) patients, to compare treatment and survival between patients enrolled before and after 2015, and to validate the discrimination of the recommended four-strata model in predicting 10-year survival at follow-up in Chinese CTD-PAH patients.

Methods

This study was derived from a Chinese national multicentre prospective registry study from 2009 to 2019. Medical records were collected at baseline and follow-up, including PAH-targeted therapy and binary therapy (both CTD and PAH-targeted therapy).

Results

A total of 266 CTD-PAH patients were enrolled and the 10-year survival rate was 59.9% (median follow-up time: 4.85 years). Underlying CTD (

SUBMITTER: Chen X 

PROVIDER: S-EPMC10629783 | biostudies-literature | 2023 Nov

REPOSITORIES: biostudies-literature