PT - JOURNAL ARTICLE AU - M. M. Hoeper AU - I. Markevych AU - E. Spiekerkoetter AU - T. Welte AU - J. Niedermeyer TI - Goal-oriented treatment and combination therapy for pulmonary arterial hypertension AID - 10.1183/09031936.05.00075305 DP - 2005 Nov 01 TA - European Respiratory Journal PG - 858--863 VI - 26 IP - 5 4099 - http://erj.ersjournals.com/content/26/5/858.short 4100 - http://erj.ersjournals.com/content/26/5/858.full SO - Eur Respir J2005 Nov 01; 26 AB - Combination therapy may improve outcome in patients with severe pulmonary arterial hypertension (PAH). PAH patients were treated according to a goal-oriented therapeutic strategy. Patients who did not reach the treatment goals with monotherapy received combination treatment according to a predefined strategy, including bosentan, sildenafil and inhaled iloprost. Intravenous iloprost and lung transplantation were reserved for treatment failures. End points were overall survival, transplantation-free survival, and survival free from transplantation and intravenous prostanoid treatment. Between January 2002 and December 2004, 123 consecutive patients with PAH were treated according to the novel approach. Survival at 1, 2 and 3 yrs was 93.0, 83.1 and 79.9%, respectively, which was significantly better than the survival of a historical control group, as well as the expected survival. Compared to the historical control group, the use of combination treatment also significantly improved the combined end point of death, lung transplantation and need for intravenous iloprost treatment. In conclusion, a therapeutic approach utilising combinations of bosentan, sildenafil and inhaled iloprost in conjunction with a goal-oriented treatment strategy provides acceptable long-term results in patients with severe pulmonary arterial hypertension, and reduces the need for intravenous prostaglandin treatment and lung transplantation.