In a single-centre Japanese cohort of 101 patients with haemodynamically severe pulmonary arterial hypertension treated with parenteral prostacyclin (1999–2023), 22 patients (22%) achieved near-normal haemodynamics and were successfully weaned off prostacyclin, all without adverse events, after a mean of 7.7 years of treatment. Those who de-escalated tended to have started prostacyclin earlier in their disease course and needed lower peak doses, suggesting early, intensive treatment may support haemodynamic recovery. Most remained on dual/triple oral therapy, and improvements were sustained over more than 5 years of follow-up. The authors note this is hypothesis-generating (single-centre, retrospective, immortal-time bias in outcome comparisons) and call for prospective, multicentre validation.
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Citation
Kuronuma K, Quadery SR, Suetomi T, et al. Early parenteral prostacyclin therapy enables near-normal haemodynamics and treatment de-escalation in patients with haemodynamically severe pulmonary arterial hypertension. Eur Respir J 2026; in press (https://doi.org/10.1183/13993003.00855-2026)