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Chronic post-embolic pulmonary hypertension: a new target for medical therapies?

Marion Delcroix
European Respiratory Review 2013 22: 258-264; DOI: 10.1183/09059180.00003513
Marion Delcroix
Respiratory Division, University Hospitals and Dept of Clinical and Experimental Medicine, University of Leuven, Leuven, Belgium.
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Abstract

The rationale for the use of pulmonary arterial hypertension-targeted drugs in chronic thromboembolic pulmonary hypertension is based on four bundles of evidence, as follows: 1) the pathobiology of the disease, with a distal component of pre-capillary arteriopathy that is very similar to pulmonary arterial hypertension; 2) the inoperability of some patients, and the persistence or recurrence of pulmonary hypertension after pulmonary endarterectomy in others; 3) the short-term efficacy and safety of pulmonary arterial hypertension-targeted drugs in these patients; and 4) their potential effect on survival. Chronic thromboembolic pulmonary hypertension is essentially a surgical disease, curable by pulmonary endarterectomy, with acceptable procedural mortality in experienced centres. Patient selection for surgery is extremely complex and results in 30–50% of patients considered inoperable. A large clinical experience has been built up with endothelin receptor antagonists and phosphodiesterase-5 inhibitors, while evidence from controlled trials is running far behind schedule. More recently, a randomised controlled trial with the guanylate cyclase stimulator, riociguat, achieved its target and showed haemodynamic, as well as functional, improvements within 4 months of therapy. The place of this therapy in the therapeutic arsenal needs to be further defined, but should be strictly limited to inoperable patients.

Footnotes

  • Provenance: Publication of this peer-reviewed article was supported by the World Scleroderma Foundation, Switzerland (principal sponsor, European Respiratory Review issue 129).

  • Conflict of interest: Disclosures can be found alongside the online version of this article at err.ersjournals.com

  • Received May 16, 2013.
  • Accepted June 17, 2013.
  • ©ERS 2013
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Chronic post-embolic pulmonary hypertension: a new target for medical therapies?
Marion Delcroix
European Respiratory Review Sep 2013, 22 (129) 258-264; DOI: 10.1183/09059180.00003513

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Chronic post-embolic pulmonary hypertension: a new target for medical therapies?
Marion Delcroix
European Respiratory Review Sep 2013, 22 (129) 258-264; DOI: 10.1183/09059180.00003513
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