Long-term course of pulmonary arterial hypertension in adults with congenital heart disease under targeted therapy: a retrospective analysis of a single tertiary center.

ABSTRACT:

Background

Pulmonary arterial hypertension (PAH), a common complication in adults with congenital heart disease (CHD), leads to significant morbidity and mortality. Targeted PAH medication is available, but PAH-CHD patient data are limited. Several questions regarding indication, treatment escalation, and combination therapy remain unanswered. The aim of this study was therefore to evaluate PAH-specific treatment in adults with PAH-CHD to better understand PAH-specific therapy management.

Methods

In this cross-sectional study we retrospectively examined clinical, demographic, and cardiac-catheterization data and medical management for PAH-CHD, and analyzed clinical course and midterm outcome.

Results

Over up to 14 years (median, 6.2 years), 103 PAH-CHD patients (66% f

SUBMITTER: Strack K 

PROVIDER: S-EPMC9622403 | biostudies-literature | 2022 Oct

REPOSITORIES: biostudies-literature