Pulmonary arterial compliance as a long-term prognostic indicator in pulmonary arterial hypertension associated with adult congenital heart disease: results from a National multicenter prospective registry.

ABSTRACT:

Background

Pulmonary arterial compliance (PAC) reflects the pulsatile load and predicts outcome in pulmonary arterial hypertension (PAH). The prognostic role of PAC in the heterogeneous patient population of PAH associated with congenital heart disease (PAH-CHD) is poorly defined. This study aimed to explore the prognostic value of PAC in patients with PAH-CHD.

Methods

Adult patients diagnosed with PAH-CHD were collected from a PAH multicenter prospective registry between August 2009 and December 2019. The primary endpoint was all-cause mortality. Multivariable Cox regression and restricted cubic spline (RCS) analysis were used to evaluate the association between PAC and the primary endpoint. Subgroup and interaction analysis between PAC and shunts or defect characteristics

SUBMITTER: Li Q 

PROVIDER: S-EPMC12522511 | biostudies-literature | 2025 Oct

REPOSITORIES: biostudies-literature