Objective To analyze the outcomes of patients with pulmonary arterial hypertension referred for lung transplantation and determine the changes over time. Methods All patients with pulmonary arterial hypertension referred for lung transplantation in our program from January 1997 to September 2010 were reviewed. Pulmonary arterial hypertension was classified as idiopathic (n = 123) or associated with congenital heart disease (n = 77), connective tissue disease (n = 102), or chronic thromboembolic disease (n = 14). Results After completing their assessment, 61 patients (19%) were found to be unsuitable for lung transplantation, 38 (12%) refused lung transplantation, 65 (21%) were too early to be listed, and 48 (15%) died before their assessment (n = 34) or being listed (n = 14). Of the 100 patients listed for lung transplantation, 57 underwent bilateral lung transplantation, 22 underwent heart–lung transplantation, 18 died while waiting, and 3 were still waiting. The waiting list mortality was the greatest for patients with connective tissue disease–pulmonary arterial hypertension (34% vs 11% in the remaining patients, P  = .005). The number of patients admitted to the hospital to be bridged to lung transplantation increased from 7% in the 1997–2004 cohort to 25% in the 2005–2010 cohort ( P  = .02). After lung transplantation, the 30-day mortality decreased from 24% in the 1997–2004 group to 6% in the 2005–2010 group ( P  = .007). The 10-year survival was worse for those with idiopathic pulmonary arterial hypertension (42% vs 70% for the remaining patients, P  = .01). The long-term survival reached 69% at 10 years in the patients with connective tissue disease pulmonary arterial hypertension. Conclusions Lung transplantation is an option for about one third of the patients with pulmonary arterial hypertension referred for lung transplantation. The 30-day mortality after lung transplantation improved significantly over time, but the long-term survival remained similar between the two cohorts. Patients with connective tissue disease–pulmonary arterial hypertension have a high mortality on the waiting list but excellent long-term survival.