title: Experience with inhaled iloprost and bosentan in portopulmonary hypertension.
abstract: Novel treatments, such as prostanoids or endothelin receptor antagonists, have been introduced for various forms of pulmonary arterial hypertension, but the long-term effects of these treatments on portopulmonary hypertension (PPHT) are unknown. In a retrospective analysis, the present authors assessed the safety and efficacy of inhaled iloprost, a prostacyclin analogue, and bosentan, an endothelin receptor antagonist, in patients with PPHT. In total, 31 consecutive patients with Child class A or B cirrhosis and severe PPHT were treated for up to 3 yrs with either inhaled iloprost (n = 13) or bosentan (n = 18), and the effects on exercise capacity, haemodynamics and survival were evaluated. In the iloprost group, the survival rates at 1, 2 and 3 yrs were 77, 62 and 46%, respectively. In the bosentan group, the respective survival rates were 94, 89 and 89%. Event-free survival rates, i.e. survival without transplantation, right heart failure or clinical worsening requiring the introduction of a new treatment for pulmonary hypertension, was also significantly better in the bosentan group. Bosentan had significantly better effects than inhaled iloprost on exercise capacity, as determined by the 6-min walk test, as well as on haemodynamics. Both treatments proved to be safe, especially in regards of liver function. In the present series of patients with well-preserved liver function and severe portopulmonary hypertension, treatment with both inhaled iloprost and bosentan appeared to be safe. Patients treated with bosentan had higher survival rates, but prospective controlled studies are required to confirm these findings.
authors.affiliation: Dept of Respiratory Medicine, Hannover Medical School, 30623 Hannover, Germany. hoeper.marius@mh-hannover.de
authors.forename: M M
authors.initials: MM
authors.lastname: Hoeper
authors.forename: H J
authors.initials: HJ
authors.lastname: Seyfarth
authors.forename: G
authors.initials: G
authors.lastname: Hoeffken
authors.forename: H
authors.initials: H
authors.lastname: Wirtz
authors.forename: E
authors.initials: E
authors.lastname: Spiekerkoetter
authors.forename: M W
authors.initials: MW
authors.lastname: Pletz
authors.forename: T
authors.initials: T
authors.lastname: Welte
authors.forename: M
authors.initials: M
authors.lastname: Halank
mesh_terms: D000280:Administration, Inhalation; D000328:Adult; D000959:Antihypertensive Agents / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use; D000077300:Bosentan; D002404:Catheterization; D018572:Disease-Free Survival; D005260:Female; D006439:Hemodynamics; D006801:Humans; D006975:Hypertension, Portal / Q000188:drug therapy*; D006976:Hypertension, Pulmonary / Q000188:drug therapy*; D016285:Iloprost / Q000008:administration & dosage* / Q000009:adverse effects / Q000627:therapeutic use*; D008297:Male; D010975:Platelet Aggregation Inhibitors / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use; D016016:Proportional Hazards Models; D013449:Sulfonamides / Q000008:administration & dosage* / Q000009:adverse effects / Q000627:therapeutic use*; D016896:Treatment Outcome
chemical_list: D000959:Antihypertensive Agents; D010975:Platelet Aggregation Inhibitors; D013449:Sulfonamides; D016285:Iloprost; D000077300:Bosentan