Long-term effects of inhaled treprostinil in patients with pulmonary arterial hypertension: The TReprostinil sodium Inhalation Used in the Management of Pulmonary arterial Hypertension (TRIUMPH) study open-label extension

摘要: Background Inhaled treprostinil improved functional capacity as add-on therapy in the short-term management of patients with pulmonary arterial hypertension (PAH). This study investigated long-term effects inhaled concurrently receiving oral background therapy. Methods A total 206 (81% women) completing 12-week double-blind phase Treprostinil Sodium Inhalation Used Management Pulmonary Arterial Hypertension (TRIUMPH) transitioned into an open-label extension. Patients were assessed every 3 months for changes 6-minute walk distance (6MWD), Borg dyspnea score, New York Heart Association (NYHA) class, quality life (QOL) scores, and signs symptoms PAH. Results primarily NYHA class III (86%), a mean baseline 6MWD 349 ± 81 meters. median change 28, 31, 32, 18 meters continuing was observed at 6, 12, 18, 24 months, respectively. effect more prominent those originally allocated to active phase. Survival rates remaining on 97%, 94%, 91% In addition, 82%, 74%, 69% maintained treatment benefit evidenced by lack clinical worsening months. The most common adverse events known prostanoid (headache [34%], nausea [21%], vomiting [10%]) or due route administration (cough [53%], pharyngolaryngeal pain [13%], chest [13%]). Conclusions Long-term demonstrated persistent PAH who remained up