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Early management decisions in complex congenital heart disease include assessing and maintaining adequate pulmonary blood flow whilst preventing increased pulmonary blood flow that may lead to pulmonary vascular remodelling and the development of pulmonary arterial hypertension. Such decision-making has now largely prevented the development of Eisenmenger syndrome in patients in the developed world, but there remain a large number of adult patients with congenital heart disease and pulmonary arterial hypertension (CHD-PAH). The CHAMPION (Congenital Heart disease And pulMonary arterial hyPertension: Improving Outcomes through education and research Networks) group was formed to highlight the clinical needs of these patients and develop clinical research and education in this area, using a network approach. A highly attended and acclaimed educational event was held in late 2017, where experts in the field discussed the various aspects of CHD-PAH and presented cases in a variety of clinical scenarios, supported by facilitated discussion, with the aim of highlighting appropriate management strategies. This supplement provides a detailed overview of CHD-PAH, including informative cases with discussion of the relevant management strategies.

Pulmonary arterial hypertension (PAH) related to congenital heart disease (CHD): introducing the CHAMPION supplement