title: Combination therapy for portopulmonary hypertension with intravenous iloprost and oral bosentan.
abstract: Severe portopulmonary hypertension (PPHTN) is a rare complication of liver cirrhosis and carries a poor prognosis. In the last years, intravenous (IV) epoprostenol has been suggested to be the optimal medical treatment for PPHTN, and recently oral bosentan has been shown to be efficacious and safe in selected patients with PPHTN. We report a case of PPHTN suffering from recurrent right heart failure while on treatment with IV iloprost, which was successfully managed by combination therapy with IV iloprost plus oral bosentan, providing sustained cardiopulmonary stabilization for at least two years. This report documents the first case of a patient with PPHTN successfully treated with the combination of IV iloprost and oral bosentan over an extended period. Thus, combination therapy with IV iloprost and oral bosentan might be a promising new option for selected patients suffering from PPHTN and recurrent right heart failure.
authors.affiliation: Internal Medicine I, Carl Gustav Carus University Hospital, TU Dresden, Germany. Michael.Halank@uniklinikum-dresden.de
authors.forename: Michael
authors.initials: M
authors.lastname: Halank
authors.forename: Martin
authors.initials: M
authors.lastname: Kolditz
authors.forename: Stephan
authors.initials: S
authors.lastname: Miehlke
authors.forename: Steffen
authors.initials: S
authors.lastname: Schiemanck
authors.forename: Alexander
authors.initials: A
authors.lastname: Schmeisser
authors.forename: Gert
authors.initials: G
authors.lastname: Hoeffken
mesh_terms: D000284:Administration, Oral; D000328:Adult; D000959:Antihypertensive Agents / Q000008:administration & dosage*; D000077300:Bosentan; D004359:Drug Therapy, Combination; D005500:Follow-Up Studies; D006333:Heart Failure / Q000209:etiology; D006439:Hemodynamics; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D006976:Hypertension, Pulmonary / Q000150:complications / Q000188:drug therapy* / Q000503:physiopathology; D016285:Iloprost / Q000008:administration & dosage*; D007275:Injections, Intravenous; D008104:Liver Cirrhosis, Alcoholic / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D008297:Male; D012008:Recurrence; D013449:Sulfonamides / Q000008:administration & dosage*; D013997:Time Factors
chemical_list: D000959:Antihypertensive Agents; D013449:Sulfonamides; D016285:Iloprost; D000077300:Bosentan