Major Finding: In the period 1997–2005, 25% of patients with idiopathic pulmonary arterial hypertension died on the waiting list. None died on the list between 2005 and 2010. After lung transplantation, the 30-day mortality decreased from 24% in the earlier period to 6% in the later period.
Data Source: A review of 2,918 patients at a single institution who were referred for lung transplantation.
Disclosures: Dr. de Perrot reported receiving speaker and teaching honoraria from Actelion.
PHILADELPHIA – Mortality on the waiting list is still a problem, but long-term survival after lung transplantation of patients with pulmonary arterial hypertension has improved significantly over time, a study has shown.
Pulmonary arterial hypertension (PAH) was classified as idiopathic (iPAH) or associated with congenital heart diseases or connective tissue diseases in the study, which was divided into 1997–2004 and 2005–2010 cohorts.
Of 2,918 patients referred to the program from January 1997 to September 2010, 316 (11%) presented with PAH (World Health Organization Group 1). In these patients, PAH was classified as iPAH (123 patients), congenital (77 patients), connective (102 patients), and other (14). The number of referrals was similar between 1997–2004 and 2005–2010. Follow-up was completed until September 2010 for all patients.
Among the 100 PAH patients listed for lung transplantation (LT), 57 had bilateral LT and 22 had heart LT. Eighteen patients on the waiting list died; three are still waiting. Waiting list mortality was higher for patients with connective tissue diseases, Dr. Marc de Perrot said at the meeting. No patient with iPAH has died on the waiting list since 2005; 25% died before that time, he and his associates at Toronto General Hospital found.
After LT, 30-day mortality decreased from 24% in the first cohort to 6% in the second, a significant difference. Ten-year survival was 56% after bilateral LT and 49% after heart LT, a nonsignificant difference. However, 10-year survival was significantly worse for iPAH patients at 42% vs. 70% for the remaining patients (P = .01). Ten-year survival was best for connective tissue disease (69%) and congenital (70%) patients.
“Patients with connective tissue diseases have a high mortality on the waiting list, but enjoy excellent long-term survival after transplant,” Dr. de Perrot concluded.