Journal article
A binational registry of adults with pulmonary arterial hypertension complicating congenital heart disease
G Strange, M Rose, F Kermeen, C O'Donnell, A Keogh, E Kotlyar, L Grigg, A Bullock, P Disney, N Dwyer, H Whitford, D Tanous, C Frampton, R Weintraub, DS Celermajer
Internal Medicine Journal | Published : 2015
DOI: 10.1111/imj.12821
Abstract
Background: The management of children with congenital heart disease (CHD) has improved over recent decades and several patients surviving with CHD into adulthood are increasing. In developed countries, there are now as many adults as there are children living with CHD. Pulmonary arterial hypertension (PAH) occurs in ~5% of patients with CHD. Aim: We aimed to understand the characteristics and outcomes of this emerging population. Methods: We collected data retrospectively and prospectively from 12 contributing centres across Australia and New Zealand (2010-2013). Patients were included if they had been diagnosed with PAH and CHD and had been seen once in an adult centre after 1 January 2000..
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Funding Acknowledgements
The Registry was funded through an unrestricted grant from Actelion Pharmaceuticals Australia.