Congenital heart disease-associated pulmonary arterial hypertension: Preliminary results from a novel registry

Abstract

Background/Aims: Pulmonary arterial hypertension (PAH) frequently accompanies childhood congenital heart disease (CHD) and may persist into adult life. The advent of specific PAH therapies for PAH prompted formation of a national Australian and New Zealand registry in 2010 to document the incidence, demographics, presentation and outcomes for these patients. Methods: This multicentre, prospective, web-based registry enrols patients with CHD-associated PAH being followed in a tertiary centre. The inclusion criteria stipulated patient age ≥16years, a measured mean pulmonary arterial pressure >25mmHg at rest or echocardiographical evidence of PAH or a diagnosis of Eisenmenger syndrome, and foll..