Ikegami, Shogo; Department of Cardiology, Keio University School of Medicine, Tokyo 160-8582, Japan.
Hiraide, Takahiro;Maeda, Takashi;Momoi, Mizuki;+5 more

Journal of clinical medicine,2025 Dec 11

Background: Pulmonary arterial hypertension (PAH) leads to right ventricular failure and death. Inhaled treprostinil, a tricyclic benzindene prostacyclin analog, has become available, but evidence regarding its clinical efficacy and quality-of-life (QoL) benefits-particularly in patients already receiving optimized combination vasodilator therapy-remains limited.
Methods: Inhaled treprostinil was introduced to nine patients with PAH already receiving combination therapy with pulmonary vasodilators. Acute hemodynamic effects were assessed during initial right heart catheterization, and long-term effects were evaluated at baseline and 3 months after treatment. Exercise tolerance was assessed by the 6-minute walking distance (6MWD) test and cardiopulmonary exercise testing, while QoL was evaluated using the Kansas City Cardiomyopathy Questionnaire-12 (KCCQ-12).
Results: Mean pulmonary arterial pressure significantly improved both acutely (48.9 ± 17.8 to 43.7 ± 14.5 mmHg, p = 0.036) and at 3 months (46.4 ± 16.1 to 39.8 ± 14.1 mmHg, p = 0.014). Pulmonary vascular resistance tended to decrease, while 6MWD outcomes remained unchanged. QoL improved, with KCCQ-12 overall and clinical summary scores increasing from 59.1 ± 27.4 to 67.1 ± 26.5 and 78.1 ± 26.3 to 87.5 ± 21.2, respectively.
Conclusions: Treprostinil inhalation improved hemodynamics and patient-reported outcomes despite prior combination improved hemodynamics and tended to enhance QoL in patients with PAH receiving combination vasodilator therapy.