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Article History

Received: 11 November 2025

Accepted: 3 February 2026

First Online: 12 March 2026

Declarations

:

: H. James Ford reports grants from United Therapeutics, Merck, Gossamer Bio, Johnson & Johnson, Cereno and Enzyvant, consultancy fees from United Therapeutics, Merck, Enzyvant, Liquidia, Johnson & Johnson and Gossamer Bio, and a prior leadership role with the Pulmonary Hypertension Association. Kelly M. Chin served as a scientific committee member for Johnson & Johnson; received research grants/support from Johnson & Johnson, Altavant, Acceleron, United Therapeutics, Pfizer, Merck, Gossamer Bio; received support for travel to meetings from Johnson & Johnson; and received consultancy fees from Johnson & Johnson, Altavant, Acceleron, United Therapeutics, Gossamer Bio and Merck. Fenling Fan served as a scientific committee member for Johnson & Johnson. Ekkehard Grünig has received fees for lectures and/or consultations from Bayer/MSD, Ferrer, GEBRO, GSK, Johnson & Johnson and OMT. Research grants to his institution have been received from Acceleron, BayerHealthCare, MSD, Bellerophon, GossamerBio, GSK, Johnson & Johnson, Novartis, OMT, Pfizer, REATA and United Therapeutics outside the submitted work. Michael Friberg , Jakob A. Hauser and Hany Rofael are employees of Johnson & Johnson. Matthieu Pannaux was an employee of a company contracted by Johnson & Johnson at the time of the work. Current affiliation: Sanofi, Vitry-Sur-Seine, France. Pavel Jansa has received fees and grants from Johnson & Johnson, AOP Orphan, Bayer Healthcare, MSD and Arena Pharmaceuticals Inc.

: The A DUE study (including the OL period) was conducted in compliance with the Declaration of Helsinki. Ethical approval was received from the institutional review boards / independent ethics committees at each site (see Supplement). As previously described, an Independent Data Monitoring Committee monitored safety during the DB period of the study and an Independent Liver Safety Data Review Board assessed any hepatic adverse events of special interest (AESIs) that required further evaluation throughout the study [ ]. A clinical events committee (CEC) reviewed and confirmed or rejected all reported potential mortality and morbidity (M/M) events in a blinded fashion. Written informed consent was obtained from all patients.