title: Oral vasodilator therapy in patients with moderate to severe portopulmonary hypertension as a bridge to liver transplantation.
abstract: Portopulmonary hypertension (POPH) is a part of group 1 pulmonary hypertension (pulmonary hypertension associated with portal hypertension). Liver transplantation (LTx) may be curative, but is usually restricted to patients with mild-to-moderate POPH. The presence of severe POPH may be a contraindication to transplantation because of the elevated risk of peritransplantation and post-transplantation morbidity and mortality. This report describes a series of seven patients with onset of moderate (two patients) or severe (five patients) POPH before LTx, of whom six were treated with oral vasodilator therapy for POPH. Although previous studies recommend aggressive parenteral prostacyclin therapy (epoprostenol), we describe the opportunity to treat cases of severe POPH with an oral phosphodiesterase type 5 inhibitor (sildenafil) and/or an endothelin receptor antagonist (bosentan/ambrisentan) as a bridge to successful LTx in selected patients.
authors.affiliation: Department of Hepatology and Gastroenterology, Ghent University Hospital, 185 9000 Ghent, Belgium.
authors.forename: Sarah
authors.initials: S
authors.lastname: Raevens
authors.forename: Michel
authors.initials: M
authors.lastname: De Pauw
authors.forename: Koen
authors.initials: K
authors.lastname: Reyntjens
authors.forename: Anja
authors.initials: A
authors.lastname: Geerts
authors.forename: Xavier
authors.initials: X
authors.lastname: Verhelst
authors.forename: Frederik
authors.initials: F
authors.lastname: Berrevoet
authors.forename: Xavier
authors.initials: X
authors.lastname: Rogiers
authors.forename: Roberto I
authors.initials: RI
authors.lastname: Troisi
authors.forename: Hans
authors.initials: H
authors.lastname: Van Vlierberghe
authors.forename: Isabelle
authors.initials: I
authors.lastname: Colle
mesh_terms: D000284:Administration, Oral; D000328:Adult; D000959:Antihypertensive Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D000077300:Bosentan; D003131:Combined Modality Therapy; D005080:Exercise Test / Q000379:methods; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications / Q000188:drug therapy* / Q000601:surgery; D006976:Hypertension, Pulmonary / Q000150:complications / Q000188:drug therapy* / Q000601:surgery; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D010666:Phenylpropionates / Q000008:administration & dosage / Q000627:therapeutic use; D058986:Phosphodiesterase 5 Inhibitors / Q000008:administration & dosage / Q000627:therapeutic use; D011724:Pyridazines / Q000008:administration & dosage / Q000627:therapeutic use; D013449:Sulfonamides / Q000008:administration & dosage / Q000627:therapeutic use; D014665:Vasodilator Agents / Q000008:administration & dosage / Q000627:therapeutic use*
chemical_list: D000959:Antihypertensive Agents; D010666:Phenylpropionates; D058986:Phosphodiesterase 5 Inhibitors; D011724:Pyridazines; D013449:Sulfonamides; D014665:Vasodilator Agents; C467894:ambrisentan; D000077300:Bosentan