Roles of cyclooxygenase 2 and hepatic venous flow in patients with HHT or hepatopulmonary syndrome

Abstract

Background: Hereditary hemorrhagic telangiectasia (HHT) and hepatopulmonary syndrome are disorders characterized by the development of multiple pulmonary arteriovenous malformations (PAVM).

Presentation of the hypothesis: COX2 may be at the origin of a cascade of pro inflammatory events to favour angiogenesis and PAVM development.

Testing the hypothesis: HHT and hepatopulmonary syndrome mouse models may be used to show its effects on PAVM formation. Anti COX-2 therapy could also be tested in human individuals, particularly in patients presenting a hepatopulmonary syndrome or HHT with small PAVM.

Implication of the hypothesis: PAVMs are one of the main causes of morbidity in patients presenting with HHT disease, owing to the risks of rupture as well as paradoxical embolism exposing to stroke and/or cerebral abscess. Percutaneous embolization has become the treatment of choice of PAVM. Anti COX2 may prevent from PAVM development and subsequent related complications and avoid either surgery and/or percutaneous embolization and thus subsequent related complication.