Eur Respir J. 2013 Feb 8. [Epub ahead of print]

Chronic Thromboembolic Pulmonary Hypertension: Role of medical therapy.

Pepke-Zaba J, Jansa P, Kim NH, Naejie R, Simonneau G.
Papworth Hospital, Cambridge, UK.

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive disease with poor prognosis if not treated. The treatment of choice is surgery with pulmonary endarterectomy. However, a significant percentage of patients are deemed non-operable due to distal distribution of the disease and arteriopathy in the non-occluded areas that is indistinguishable from pulmonary arterial hypertension (PAH). The overlap in clinical presentation, pathological features and pathogenesis between PAH and CTEPH provides a compelling rationale for exploring the efficacy of PAH-targeted therapies in CTEPH. These therapies are often considered for non-operable patients and are also used in operable patients as a bridge to surgery or as post-PEA therapy for persistent pulmonary hypertension, despite the fact they are not licensed for CTEPH.Two randomised clinical trials (RCTs) have been performed in non-operable CTEPH patients. The BENEFiT study, with the endothelin receptor antagonist bosentan, did not show improvement in walking distance. Recently, the CHEST-1 trial, with the soluble guanylate cyclase stimulator riociguat, met study endpoint and demonstrated significant improvement in walking distance in patients with non-operable CTEPH.There is an urgent need for more RCTs designed to clarify whether administration of PAH-targeted therapies improves clinically meaningful endpoints in various CTEPH populations.

PMID:23397304