Abstract
Background: Recent progress in the development of pulmonary hypertension (PH)-specific pharmaceutical agents has improved mortality and morbidity remarkably. Today, these PH-specific drugs have become a standard treatment for PH. Methods and Results: We herein summarize the treatment options and longitudinal clinical outcomes of 21 patients with PH who received PH-specific drugs at the present institution. Sixteen patients began treatment with a single PH-specific drug; 9 of them needed additional PH-specific drugs, but the other 7 were still taking the same drug at the last follow-up. Five patients began treatment with a combination of 2 or 3 PH-specific drugs, and their drugs were not discontinued. Most patients (17/21) were taking a phosphodiesterase type 5 (PDE5) inhibitor at the last follow-up. During the 6.5±4.4 years' follow-up, 5 patients died, but only 1 death was related to PH. At 5 and 10 years, the estimated PH-related death-free and lung transplantation-free survival rate was 100% (95% CI: 100-100%) and 87.5% (95% CI: 38.7-98.1%), respectively. The estimated 5- and 10-year estimated overall survival rates were 77.9% (95% CI: 50.8-91.3%) and 68.2% (95% CI: 37.4-86.2%), respectively. Conclusions: PDE5 inhibitors played a central role in the treatment options. The long-term prognosis of PH was favorable at the present institution.