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Pulmonary Therapy

, Volume 3, Issue 1, pp 31–43

First Online: 20 February 2017Received: 07 November 2016DOI: 10.1007-s41030-017-0029-3

Cite this article as: Germani, J., Kingman, M., Kitterman, N. et al. Pulm Ther 2017 3: 31. doi:10.1007-s41030-017-0029-3


Pulmonary arterial hypertension PAH and chronic thromboembolic pulmonary hypertension CTEPH are subtypes of pulmonary hypertension—a rare, life-threatening condition defined by chronically elevated pulmonary artery pressure. Treatment goals include improving patient functioning, symptom management, and delaying disease progression. Traditional therapies have focused on affected physiologic pathways, with endothelin-receptor antagonists and-or phosphodiesterase-5 inhibitors as first-line agents and prostacyclins for more severe disease. Riociguat, a therapeutic agent that stimulates soluble guanylate cyclase via nitric-oxide pathways, was approved in 2013 for treatment of PAH and CTEPH. Riociguat significantly improved exercise capacity, hemodynamic parameters, and other functional endpoints in phase 3 trials. Safety and efficacy were maintained in long-term extension studies. Adverse events AEs were generally mild to moderate and self-limiting and did not require treatment discontinuation. To optimize adherence, frequent patient monitoring and management of AEs are recommended. Management strategies include patient education, treatment-prevention of adverse events, and individualized dose adjustment.

Funding: Bayer HealthCare Pharmaceuticals, Whippany, NJ, USA.

KeywordsChronic thromboembolic pulmonary hypertension CTEPH Patient management Pulmonary arterial hypertension PAH Riociguat Enhanced contentTo view enhanced content for this article go to

Autor: Judy Germani - Martha Kingman - Natalie Kitterman - Traci Stewart - Melisa Wilson - Debra Zupancic


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