Background: Pulmonary hypertension is a serious disease that, in its terminal phase, requires lung transplantation. Patients and methods: A retrospective study was undertaken of 15 patients with pulmonary hypertension who underwent lung transplantation between 1994 and 2004. Clinical data recorded before the procedure and during follow-up were reviewed. Results: Pulmonary hypertension Was reported as idiopathic in 8 patients (53%) and related to consumption of toxic oil in 2. The remaining causes were documented as chronic peripheral pulmonary embolism, histiocytosis X, venoocclusive disease, scleroderma, and simple corrected congenital heart defect in I patient each. The mean values of the hemodynamic variables were 100, 50, and 67 torn Hg for systolic, diastolic, and mean pulmonary artery pressure, respectively; 2.63 L/min for cardiac output; and 20.9 Wood units for total pulmonary resistance. The mean time between diagnosis of pulmonary hypertension and lung transplantation was 5.9 years (range, 0.4-20 y). Seven patients were in functional class III and 8 in functional class IV. The mean 6-minute walk distance was 204 m (range, 0-360 m). Four patients (26%) died during the during the perioperative period and 9 (60%), 7 (46%), and 6 (40%) were still alive at 1, 3, and 5 years, respectively. Conclusions: Double-lung transplantation is a therapeutic option that, in certain cases, has similar outcomes to those achieved with the most aggressive medical treatment for pulmonary hypertension. (c) 2008 SEPAR. Published by Elsevier Espana, S.L. All rights reserved.