Long-term outcome of children with newly diagnosed pulmonary arterial hypertension: results from the global TOPP registry.

ABSTRACT:

Background and aims

The Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension (TOPP) registry is a global network established to gain insights into the disease course and long-term outcomes of paediatric pulmonary arterial hypertension (PAH). Previously published cohorts in paediatric PAH are obscured by survival bias due to the inclusion of both prevalent (previously diagnosed) and incident (newly diagnosed) patients. The current study aims to describe long-term outcome and its predictors in paediatric PAH, exclusively of newly diagnosed patients.

Methods and results

Five hundred thirty-one children with confirmed pulmonary hypertension, aged ≥3 months and <18 years, were enrolled in the real-world TOPP registry at 33 centres in 20 countries, from 2008 to 2015.

SUBMITTER: Ploegstra MJ 

PROVIDER: S-EPMC10785586 | biostudies-literature | 2024 Jan

REPOSITORIES: biostudies-literature