Patients with pulmonary arterial hypertension
Pulmonary arterial hypertension therapies (targeting endothelin-1, nitric oxide, prostacyclin, and bone morphogenetic protein/activin signalling pathways), including combination therapy, parenteral therapy, and lung transplantation
Current treatment algorithms for pulmonary arterial hypertension emphasize combination and parenteral therapies for greater efficacy, with lung transplantation remaining an option for inadequate responders.
Pulmonary arterial hypertension leads to significant impairment in haemodynamics, right heart function, exercise capacity, quality of life and survival. Current therapies have mechanisms of action involving signalling via one of four pathways: endothelin-1, nitric oxide, prostacyclin and bone morphogenetic protein/activin signalling. Efficacy has generally been greater with therapeutic combinations and with parenteral therapy compared with monotherapy or nonparenteral therapies, and maximal medical therapy is now four-drug therapy. Lung transplantation remains an option for selected patients with an inadequate response to therapies.
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Kelly Chin
Seán Gaine
Christian Gerges
European Respiratory Journal
University of Michigan
Johns Hopkins University
The University of Texas Southwestern Medical Center
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Chin et al. (Thu,) studied this question.
www.synapsesocial.com/papers/69da2a57ba6014a02e83670f — DOI: https://doi.org/10.1183/13993003.01325-2024