Riociguat Therapy for Pulmonary Hypertension

Indications and Contraindications

The American Thoracic Society recommends riociguat for treatment-naive PAH patients with WHO functional class II symptoms who are not candidates for, or have failed, calcium channel blocker (CCB) therapy, as it can improve 6-minute walking distance (6MWD) and delay time to clinical worsening 2, 3
The American College of Chest Physicians recommends riociguat for treatment-naive PAH patients with WHO functional class III symptoms who are not candidates for, or have failed, CCB therapy, as it can improve 6MWD, WHO functional class, and cardiopulmonary hemodynamics 4, 5
Riociguat is indicated for patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH) or persistent/recurrent CTEPH after pulmonary endarterectomy (PEA), and can significantly increase 6MWD and reduce pulmonary vascular resistance (PVR) 1

The American College of Chest Physicians recommends dose titration of riociguat based on systemic blood pressure response, with a starting dose that is typically lower and gradual titration to minimize risk of hypotension 4
Patients should be monitored for hypotension during dose adjustment period, and assessment of WHO functional class is recommended 4, 5

The European Respiratory Society suggests that all CTEPH patients should be evaluated by a multidisciplinary team including an experienced PEA surgeon to determine operability before considering riociguat 1
Male patients treated with riociguat should be cautioned not to use PDE5 inhibitors for erectile dysfunction due to risk of severe hypotension, as recommended by the American College of Chest Physicians 2, 4
Patients should be followed at specialized centers with at least one hemodynamic assessment 6-12 months after treatment initiation, as recommended by the American Thoracic Society 1