1. Phenotypes in pulmonary hypertension.
- Author
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Weatherald J, Hemnes AR, Maron BA, Mielniczuk LM, Gerges C, Price LC, Hoeper MM, and Humbert M
- Subjects
- Humans, Prognosis, Pulmonary Arterial Hypertension physiopathology, Pulmonary Arterial Hypertension diagnosis, Pulmonary Arterial Hypertension classification, Phenotype, Hypertension, Pulmonary classification, Hypertension, Pulmonary physiopathology, Hypertension, Pulmonary diagnosis, Hypertension, Pulmonary therapy
- Abstract
The clinical classification of pulmonary hypertension (PH) has guided diagnosis and treatment of patients with PH for several decades. Discoveries relating to underlying mechanisms, pathobiology and responses to treatments for PH have informed the evolution in this clinical classification to describe the heterogeneity in PH phenotypes. In more recent years, advances in imaging, computational science and multi-omic approaches have yielded new insights into potential phenotypes and sub-phenotypes within the existing clinical classification. Identification of novel phenotypes in pulmonary arterial hypertension (PAH) with unique molecular profiles, for example, could lead to new precision therapies. Recent phenotyping studies have also identified groups of patients with PAH that more closely resemble patients with left heart disease (group 2 PH) and lung disease (group 3 PH), which has important prognostic and therapeutic implications. Within group 2 and group 3 PH, novel phenotypes have emerged that reflect a persistent and severe pulmonary vasculopathy that is associated with worse prognosis but still distinct from PAH. In group 4 PH (chronic thromboembolic pulmonary disease) and sarcoidosis (group 5 PH), the current approach to patient phenotyping integrates clinical, haemodynamic and imaging characteristics to guide treatment but applications of multi-omic approaches to sub-phenotyping in these areas are sparse. The next iterations of the PH clinical classification are likely to reflect several emerging PH phenotypes and improve the next generation of prognostication tools and clinical trial design, and improve treatment selection in clinical practice., Competing Interests: Conflict of interest: J. Weatherald reports grants from Janssen, Bayer, Merck and AstraZeneca; consulting fees and lecture honoraria from Janssen and Merck; payment for expert testimony from Sprigings Intellectual Property Law; travel support from Janssen; advisory board participation with Janssen, Acceleron and Université Laval; and a leadership role on the medical advisory committee for Pulmonary Hypertension Association of Canada, outside the submitted work. A.R. Hemnes reports grants from NIH; consulting fees from Gossamer Bio, United Therapeutics, Bayer, Janssen, Merch and Tenax Therapeutics; advisory board participation with NIH/NHLBI; leadership roles with the Nashville Ballet and Pulmonary Vascular Research Institute; and stock or stock options with Tenax Therapeutics, outside the submitted work. B.A. Maron reports grants from NIH/NHLBI; consulting fees from Actelion and Tenax Therapeutics; and the following patents: PCT/US2019/059890, #9605047 and PCT/US2020/066886, outside the submitted work. L.M. Mielniczuk reports consulting fees from Bayer, Merck and Janssen; lecture honoraria from Janssen and Merck; and leadership roles as Chair of Pulmonary Hypertension Association of Canada and Vice President of Canadian Heart Failure Society, outside the submitted work. C. Gerges reports support for the present manuscript from OrphaCare; outside the submitted work, C. Gerges reports lecture honoraria from AOPHealth, AstraZeneca, Janssen and Ferrer; and travel support from AOPHealth, AstraZeneca, Cordis, Janssen and MSD. L.C. Price reports grants from Ferrer; lecture honoraria from Janssen, Ferrer and MSD; travel support from Janssen, Ferrer Pharmaceuticals and Altavant; and advisory board participation with Janssen, outside the submitted work. M.M. Hoeper reports consulting fees from Acceleron, Actelion, Altavant, AOP Health, Bayer, Ferrer, Gossamer Bio, Janssen, Keros and MSD; lecture honoraria from Acceleron, Actelion, AOP Health, Bayer, Ferrer, Janssen and MSD; and US patent application #63466014 (Methods of improving lung diffusion capacity in a patients with pulmonary arterial hypertension, application filed by MSD), outside the submitted work. M. Humbert reports grants from Acceleron, AOP Orphan, Janssen, Merck and Shou Ti; consulting fees from Acceleron, Aerovate, Altavant, AOP Orphan, Bayer, Chiesi, Ferrer, Janssen, Merck, MorphogenIX, Shou Ti and United Therapeutics; lecture honoraria from Janssen and Merck; and advisory board participation with Acceleron, Altavant, Janssen, Merck and United Therapeutics, outside the submitted work., (Copyright ©The authors 2024. For reproduction rights and permissions contact permissions@ersnet.org.)
- Published
- 2024
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