Long-term survival of patients with pulmonary arterial hypertension recovering to World Health Organization functional class I or II: a historical comparison between intravenous epoprostenol and oral agents

摘要

Background Intravenous epoprostenol is the only drug proved in a randomized study to reduce mortality in patients with idiopathic pulmonary arterial hypertension (PAH). However, administration of this drug has procedural difficulties and a risk of sepsis. Oral drugs provide simple treatment, but their benefit for survival has not been proven. A recovery of patients with PAH to World Health Organization functional class (WHO-FC) I or II may predict favorable survival. Methods Survival analyses were performed on a historical cohort of 41 patients with PAH. The patients were 43?±?22?years old, 23 had idiopathic or heritable PAH, and 18 had connective tissue disease-associated PAH. The baseline was defined as the initial visit to a medical facility. Results The median duration of follow-up was 1276?days (108 to 5389?days) and 21 patients died during this period. The estimated survival times for patients who received intravenous epoprostenol and did and did not recover to WHO-FC I or II were 4371?±?577?days and 1172?±?404?days, respectively. These times for patients who were not treated with intravenous epoprostenol and did and did not recover to WHO-FC I or II were 4717?±?554?days and 925?±?230?days, respectively. A Cox proportional hazard analysis gave a hazard ratio for death after recovery to WHO-FC I or II of 0.07 (P? Conclusions Patients with PAH who achieve recovery to WHO-FC I or II without use of intravenous epoprostenol have similar survival to those who reach the same WHO-FC with use of intravenous epoprostenol. Benign survival of patients with PAH who have recovered to WHO-FC I or II may extend for several years after onset of the disease.