Survival comparison in adults with congenital systemic to pulmonary shunt and borderline elevated pulmonary vascular resistance versus Eisenmenger syndrome.

ABSTRACT: Pulmonary arterial hypertension (PAH) associated with congenital heart disease (PAH-CHD) is a consequence of unrepaired large systemic-to-pulmonary shunts. The long-term data of adult patients who have PAH-CHD with elevated pulmonary vascular resistance (PVR) are limited. We aimed to investigate the survival of adults who had PAH-CHD with predominantly left-to-right (L-R) shunts with (1) borderline-to-high PVR and (2) treat-and-repair compared with those with Eisenmenger syndrome (ES). From 1995 to 2021, 99 adults with ES (age 34.1 ± 11.2 years) and 118 adults with PAH-CHD with predominantly L-R shunts (age 39.1 ± 13.7 years) were eligible. The PVR in the ES group was 21.0 ± 13.1 WU. Most ES patients (97%) received pulmonary vasodilator therapy. Among the 118 patients with PAH-CHD with pre

SUBMITTER: Sengsim J 

PROVIDER: S-EPMC11612139 | biostudies-literature | 2024 Dec

REPOSITORIES: biostudies-literature