title: Secure Combination Therapy With Low-Dose Bosentan and Ambrisentan to Treat Portopulmonary Hypertension Minimizing Each Adverse Effect.
abstract: Although endothelin receptor antagonists (ERAs) including bosentan and ambrisentan are essential tools for the treatment of pulmonary arterial hypertension (PAH), each agent has a specific adverse effect with non-negligible frequency, ie, liver dysfunction for bosentan and peripheral edema for ambrisentan. These adverse effects often hinder the titration of the doses of ERAs up to the therapeutic levels. Portopulmonary hypertension, which is complicated with liver cirrhosis and successive portal hypertension, is one of the PAHs refractory to general anti-PAH agents because of the underlying progressed liver dysfunction and poor systemic condition. We here present a patient with portopulmonary hypertension, which was treated safely by combination therapy that included low-dose bosentan and ambrisentan, minimizing the adverse effects of each ERA. Combination therapy including different types of ERAs at each optimal dose may become a breakthrough to overcome portopulmonary hypertension in the future.
authors.affiliation: Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo.
authors.forename: Hironori
authors.initials: H
authors.lastname: Muraoka
authors.forename: Teruhiko
authors.initials: T
authors.lastname: Imamura
authors.forename: Masaru
authors.initials: M
authors.lastname: Hatano
authors.forename: Hisataka
authors.initials: H
authors.lastname: Maki
authors.forename: Atsushi
authors.initials: A
authors.lastname: Yao
authors.forename: Koichiro
authors.initials: K
authors.lastname: Kinugawa
authors.forename: Issei
authors.initials: I
authors.lastname: Komuro
mesh_terms: D000959:Antihypertensive Agents / Q000008:administration & dosage / Q000009:adverse effects; D000077300:Bosentan; D003131:Combined Modality Therapy / Q000379:methods; D003937:Diagnosis, Differential; D004305:Dose-Response Relationship, Drug; D004452:Echocardiography; D006439:Hemodynamics / Q000187:drug effects*; D006801:Humans; D006975:Hypertension, Portal / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D008103:Liver Cirrhosis / Q000150:complications*; D008297:Male; D008875:Middle Aged; D010666:Phenylpropionates / Q000008:administration & dosage / Q000009:adverse effects; D011724:Pyridazines / Q000008:administration & dosage / Q000009:adverse effects; D013449:Sulfonamides / Q000008:administration & dosage / Q000009:adverse effects; D016896:Treatment Outcome
chemical_list: D000959:Antihypertensive Agents; D010666:Phenylpropionates; D011724:Pyridazines; D013449:Sulfonamides; C467894:ambrisentan; D000077300:Bosentan