TY - JOUR T1 - Long-term efficacy of bosentan in treatment of pulmonary arterial hypertension in children JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/09031936.00053510 SP - erj00535-2010 AU - A.A. Hislop AU - S. Moledina AU - H. Foster AU - I. Schulze-Neick AU - S. G Haworth Y1 - 2011/01/01 UR - http://erj.ersjournals.com/content/early/2010/12/22/09031936.00053510.abstract N2 - To evaluate a 5 year experience using bosentan in children with pulmonary arterial hypertension (PAH) a retrospective, observational study was made of children in the UK Pulmonary Hypertension Service for Children given bosentan as monotherapy or in combination from February 2002 until May 2008 and followed up for at least 6 months.Detailed studies were made of 101 children with idiopathic PAH (42) and PAH associated with congenital heart disease (59). Before treatment, WHO Functional Class, 6-minute walk distance (6MWD), height, weight and haemodynamic data were determined. Evaluations were analysed after 6 months and annually to a maximum of 5 years. Median duration of treatment was 31.5 months. Initial improvement in WHO and 6MWD was maintained for up to 3 years. Height and weight increased but the z-scores did not improve. After 3 years, bosentan was continued as monotherapy in only 21% of children with IPAH, but in 69% of repaired cases and 56% of those with Eisenmenger Syndrome. The Kaplan-Meier survival estimates for the 101 patients were 96%, 89%, 83% and 60% at 1, 2, 3 and 5 years. A treatment regime which includes bosentan is safe and appears effective in slowing disease progression in children with PAH. ER -