Management of pulmonary arterial hypertension in children.
In this review we discuss the new anti- Pulmonary Arterial Hypertension [PAH] drugs and the available data on their use in paediatric PAH. Treatment of patients with PAH, children and adults, is aimed at a reduction of symptoms, survival and improvement of haemodynamics as well as exercise capacity. PAH may reflect significant different disease conditions in infants and children when compared to PAH in adults. In contrast to adult PAH, characterized mainly by idiopathic PAH and PAH associated with connective tissue disease, more than half of the cases of PAH in children are associated with congenital heart disease. Therefore, efficacy of PAH drugs in these diseases can not be extrapolated from that in adults with PAH.
Department of Paediatric Cardiology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, The Netherlands. M.T.R.Roofthooft@BKK.UMCG.nl
M T R
MT
Roofthooft
R L E
RL
van Loon
R M F
RM
Berger
D000465:Algorithms; D002648:Child; D065128:Endothelin Receptor Antagonists; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy*; D058986:Phosphodiesterase 5 Inhibitors / Q000627:therapeutic use
D065128:Endothelin Receptor Antagonists; D058986:Phosphodiesterase 5 Inhibitors