Outcome of patients with pulmonary arterial hypertension referred for lung transplantation: a 14-year single-center experience

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.