Background: Pulmonary arterial hypertension (PAH) is associated with decreased functional capacity, right ventricular failure and often early death. The recent introduction of advanced therapies has brought new opportunities for patients with PAH. In The Netherlands, bosentan is distributed in a controlled fashion through a single pharmacy. This allows for the registration and prospective follow-up of all patients in a central database. The aim of this study was to evaluate the 6-year survival rates of all Dutch PAH patients treated with bosentan. Methods: We analyzed the 6-year survival characteristics of the entire Dutch cohort of idiopathic PAH (iPAH; n = 209), collagen vascular disease-associated PAH (CVD-PAH; n = 161), congenital heart disease-associated PAH (CHD-PAH; n = 224) and inoperable chronic thromboembolic pulmonary hypertension (iCTEPH; n = 175) patients, who treated with bosentan between 2002 and 2009. Results: A total of 913 patients were treated with bosentan during the study period. Altogether, 769 patients were included for analysis. Survival rates (for all indications) at 1, 3 and 6 years, respectively, were as follows: overall, 90.7% (n = 486), 78.4% (n = 261) and 57.9% (n = 34); for iPAH, 86.9%, 71.8% and 54.6%; for CVD-PAH, 85.7%, 65.1% and 37.3%; for CHD-PAH, 93.5%, 87.9% and 64.2%; and for iCTEPH, 95.3%, 84.5% and 68.7%. Multivariate analysis showed iCTEPH, female gender, younger age, treatment initiation after 2006 and treatment at an expert center to all be independently and significantly correlated with better outcome. Conclusions: This is the first study on a national scale to show 6-year survival rates of bosentan-treated patients for three major PAH and iCTEPH subgroups. Survival rates in the respective subgroups from the Dutch bosentan cohort are comparable to earlier reported rates from selected patient cohorts.

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Keywords congenital heart disease, connective tissue disease, idiopathic pulmonary hypertension, inoperable chronic thromboembolic pulmonary hypertension, survival
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Bresser, P.J.C., Vis, J.C., Berger, R.M., Vliegen, H.W., Vonk, M.C., Snijder, R., … Mulder, B.J.M.. (2011). Predictors of long-term survival in pulmonary hypertension treated with bosentan. The Journal of Heart and Lung Transplantation. doi:10.1016/j.healun.2011.01.709