Key result
Medical therapies including riociguat improve hemodynamics and exercise capacity in inoperable or persistent CTEPH.
Why the study?
Medical therapies for patients with inoperable CTEPH or persistent/recurrent pulmonary hypertension after PEA have recently advanced but require further evaluation and optimization.
Riociguat is an effective medical therapy for patients with inoperable CTEPH or persistent/recurrent pulmonary hypertension after pulmonary endarterectomy.
May support riociguat in inoperable or post-PEA CTEPH; leaves open need for confirmatory RCTs.
Chronic thromboembolic pulmonary hypertension (CTEPH) results from incomplete resolution of acute pulmonary emboli, organised into fibrotic material that obstructs large pulmonary arteries, and distal small-vessel arteriopathy. Pulmonary endarterectomy (PEA) is the treatment of choice for eligible patients with CTEPH; in expert centres, PEA has low in-hospital mortality rates and excellent long-term survival. Supportive medical therapy consists of lifelong anticoagulation plus diuretics and oxygen, as needed.An important recent advance in medical therapy for CTEPH is the arrival of medical therapies for patients with inoperable disease or persistent/recurrent pulmonary hypertension after PEA. The soluble guanylate cyclase stimulator riociguat is licensed for the treatment of CTEPH in patients with inoperable disease or with recurrent/persistent pulmonary hypertension after PEA. Clinical trials of this agent have shown improvements in patients' haemodynamics and exercise capacity. Phosphodiesterase-5 inhibitors, endothelin receptor antagonists and prostanoids have been used in the treatment of CTEPH, but evidence of benefit is limited. Challenges in the future development of medical therapy for CTEPH include better understanding of the underlying pathology, end-points to monitor the condition's progress, and the optimisation of pulmonary arterial hypertension therapies in relation to diverse patient characteristics and emerging options such as balloon pulmonary angioplasty.
No takes yet. Share an insight, caveat, or question.
Pepke‐Żaba et al. (2017) conducted a review in Chronic thromboembolic pulmonary hypertension (CTEPH). Medical management (e.g., riociguat) was evaluated. Medical therapies such as riociguat improve hemodynamics and exercise capacity in patients with inoperable chronic thromboembolic pulmonary hypertension or persistent disease after endarterectomy.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: