Background Despite the introduction of targeted therapies in pediatric pulmonary arterial
hypertension (PAH), prognosis remains poor. For the definition of treatment strategies and …

Objectives: In order to describe survival and treatment strategies in pediatric pulmonary
arterial hypertension (PAH) in the current era of PAH-targeted drugs and to identify …

To be able to design goal-oriented treatment strategies in paediatric pulmonary arterial
hypertension (PAH), we aimed to identify treatment goals by investigating the prognostic …

Rationale: There are currently no data supporting specific dosing and weaning strategies for
parenteral prostanoid therapy in children with pulmonary arterial hypertension (PAH) …

Rationale: The development of evidence-based treatment guidelines for pediatric pulmonary
arterial hypertension (PAH) is hampered by lack of pediatric clinical trials. Trial design is …

Congenital heart disease (CHD) is a frequent cause of pediatric pulmonary arterial
hypertension (PAH), with diverse etiology and outcome. We aimed to describe phenotypic …

Objectives Paediatric pulmonary arterial hypertension (PAH) after neonatal arterial switch
operation (ASO) for transposition of the great arteries (TGA) is a clinically recognised entity …

Pulmonary arterial hypertension (PAH) is a severe and progressive intrinsic disease of the
precapillary lung vasculature. Since the introduction of PAH-targeted drugs, survival of PAH …

Muscle strength is decreased in adults with pulmonary arterial hypertension (PAH). We aim
to investigate muscle strength in children with PAH in relation to a cohort of healthy children …

Conclusions–In the era of PAH-targeted drugs, 36% of children with PAH were treated with
IV/SC prostanoids. Most children had severe PAH and started early after diagnosis. Near …