Efectos a largo plazo (5 años) de bosentán en pacientes con hipertensión arterial pulmonar

Introduction and objectives: Bosentan has proven efficacy in pulmonary hypertension in the short term. Little is known about its effects beyond 2 to 3 years. Our objective was to analyze the efficacy and safety of bosentan in the long term (5 years) in patients treated in our center. Methods: This retrospective study sequentially analyzed clinical, functional, and laboratory parameters in a series of patients treated initially with bosentan as monotherapy from 2002 to 2009 in a single hospital. Treatment success was defined as survival without clinical worsening that required additional pulmonary vasodilators. Results: We included 20 patients (70% women, mean age 4614 years, 65% congenital heart disease), with a median follow-up of 64 months. One patient required withdrawal of bosentan due to adverse effects. At 4 months, significant improvements were achieved in hemodynamic, clinical and functional parameters. Clinical and functional benefits persisted at 5-year follow-up. Overall 5-year survival after beginning bosentan therapy was 95% (84%-100%). Treatment success at 1, 2, 3, 4 and 5 years was 95% (84%-100%), 83% (65%-100%), 78% (58%-98%), 61% (38%-84%), and 41% (16%-66%), respectively. The group with better outcomes had NT-proBNP levels at 1 year <400 pg/mL (P=.013). Conclusions: In our series, treatment success with bosentan in monotherapy was maintained in 78% at 3-year follow-up and 41% at 5-year follow-up. The group with long-term success showed significantly lower NT-proBNP levels at 1-year follow-up. Survival at 5 years in our series was 95%.