Early Observations on the Use of Riociguat in a Large, Metropolitan Pulmonary Arterial Hypertension/Chronic Thromboembolic Pulmonary Hypertension Treatment Center.

ABSTRACT:

Introduction

Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are rare, life-threatening diseases in which chronically elevated pressure in the pulmonary arteries results in vascular remodeling and right heart failure. Treatment goals are to improve patient functioning, exercise capacity, and symptoms; delay disease progression; normalize the right ventricular function; and, ultimately, improve survival. Therapeutic management centers on the affected physiologic pathways and includes endothelin receptor antagonists, phosphodiesterase-5 inhibitors, and prostacyclins. Recently, riociguat, a novel therapeutic agent that stimulates soluble guanylate cyclase via the nitric oxide pathway, was approved for the treatment of both PAH and CTEPH.

SUBMITTER: Sulica R 

PROVIDER: S-EPMC4675747 | biostudies-literature | 2015 Dec

REPOSITORIES: biostudies-literature