PHILADELPHIA – Although mortality on the waiting list is still a problem, long-term survival after lung transplantation of patients with pulmonary arterial hypertension has improved significantly over time, a study has shown.
In the study, pulmonary arterial hypertension (PAH) was classified as idiopathic (iPAH) or associated with congenital heart diseases or connective tissue diseases. Patients were divided into 1997-2004 and 2005-2010 cohorts.
Out of 2,918 patients referred to the program between January 1997 and 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 underwent bilateral LT and 22 had heart LT. Eighteen patients on the waiting list died, and three are still waiting. The waiting list mortality was higher for patients with connective tissue diseases, Dr. Marc de Perrot said at the annual meeting of the American Association for Thoracic Surgery.
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, the 30-day mortality decreased from 24% in the first cohort to 6% in the second, a significant difference. The 10-year survival was 56% after bilateral LT and 49% after heart LT, a nonsignificant difference.
However, the 10-year survival was significantly worse for iPAH patients at 42% vs. 70% for the remaining patients (P = .01). The 10-year survival was best for connective tissue disease (69%) and congenital (70%) patients.
Lung transplantation is a viable option for about a third of the patients presenting with PAH, according to Dr. de Perrot. He added that extracorporeal life support may help reduce the waiting list mortality, particularly for iPAH patients. Overall, the 30-day mortality for patients after lung transplantation has improved significantly over time.
"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.
Dr. de Perrot reported receiving speaker and teaching honoraria from Actelion.