BACKGROUND AND OBJECTIVE: There are few published data on the efficacy of i.v. iloprost in pulmonary arterial hypertension (PAH). We present long-term outcomes in PAH patients receiving i.v. iloprost in a large UK referral centre. METHODS: Eighty patients with idiopathic PAH (iPAH, n = 46) or PAH associated with connective tissue disease (CTD-PAH, n = 34) were identified as receiving domiciliary i.v. iloprost between January 1999 and April 2015. Baseline characteristics, doses achieved, functional class at follow-up and survival data were retrieved from hospital databases. RESULTS: Median maximum dose achieved was 4.6 ng/kg/min in the iPAH group and 5.0 ng/kg/min in CTD-PAH patients. Exercise capacity significantly improved in the first 6 months of therapy in IPAH patients. Overall 1-, 3- and 5-year survival was 78%, 64% and 52% in iPAH (P = 0.002) and 64%, 26% and 21% in CTD-PAH. Independent predictors of survival were age and exercise capacity. CONCLUSION: We report improved survival to that previously reported in iPAH patients treated with domiciliary i.v. iloprost. This may be in part related to higher administered doses. Patients with CTD-PAH had poorer survival, reinforcing the need for early transplantation referral in suitable patients.
BACKGROUND AND OBJECTIVE: There are few published data on the efficacy of i.v. iloprost in pulmonary arterial hypertension (PAH). We present long-term outcomes in PAH patients receiving i.v. iloprost in a large UK referral centre. METHODS: Eighty patients with idiopathic PAH (iPAH, n = 46) or PAH associated with connective tissue disease (CTD-PAH, n = 34) were identified as receiving domiciliary i.v. iloprost between January 1999 and April 2015. Baseline characteristics, doses achieved, functional class at follow-up and survival data were retrieved from hospital databases. RESULTS: Median maximum dose achieved was 4.6 ng/kg/min in the iPAH group and 5.0 ng/kg/min in CTD-PAHpatients. Exercise capacity significantly improved in the first 6 months of therapy in IPAH patients. Overall 1-, 3- and 5-year survival was 78%, 64% and 52% in iPAH (P = 0.002) and 64%, 26% and 21% in CTD-PAH. Independent predictors of survival were age and exercise capacity. CONCLUSION: We report improved survival to that previously reported in iPAH patients treated with domiciliary i.v. iloprost. This may be in part related to higher administered doses. Patients with CTD-PAH had poorer survival, reinforcing the need for early transplantation referral in suitable patients.
Authors: Sean Gaine; Kelly Chin; Gerry Coghlan; Richard Channick; Lilla Di Scala; Nazzareno Galiè; Hossein-Ardeschir Ghofrani; Irene M Lang; Vallerie McLaughlin; Ralph Preiss; Lewis J Rubin; Gérald Simonneau; Olivier Sitbon; Victor F Tapson; Marius M Hoeper Journal: Eur Respir J Date: 2017-08-17 Impact factor: 16.671
Authors: Mikaela Wardle; Amanda Nair; Sarah Saunders; Iain Armstrong; Athanasios Charalampopoulos; Charlie Elliot; Abdul Hameed; Neil Hamilton; John Harrington; Carol Keen; Robert Lewis; Ian Sabroe; A A Roger Thompson; Robert M Kerry; Robin Condliffe; David G Kiely Journal: Pulm Circ Date: 2022-03-25 Impact factor: 2.886
Authors: Clara Hjalmarsson; Barbro Kjellström; Kjell Jansson; Magnus Nisell; David Kylhammar; Mohammad Kavianipour; Göran Rådegran; Stefan Söderberg; Gerhard Wikström; Dirk M Wuttge; Roger Hesselstrand Journal: ERJ Open Res Date: 2021-08-02