title: Severe portopulmonary hypertension in congenital hepatic fibrosis.
abstract: Portopulmonary hypertension is a rare complication of portal hypertension. Although epoprostenol infusion, nitric oxide inhalation, isosorbide-5-mononitrate, nitroglycerin, and calcium channel blockers may reduce pulmonary artery pressure in patients with portopulmonary hypertension, the prognosis remains poor. We present a case of congenital hepatic fibrosis associated with pulmonary hypertension. A 42-year-old man with congenital hepatic fibrosis visited our hospital with syncope. The man had suffered from breathlessness on exertion for 2 weeks before the episode of syncope. He also had a history of portal hypertension with documented gastric cardiac varices at the age of 28 years. Despite undergoing intensive care, the patient died 1 week after admission owing to severe right-sided heart failure. Autopsy revealed dilatation of the right atrium and right ventricle grossly and plexogenic pulmonary arteriopathy microscopically. Accurate diagnosis of portopulmonary hypertension requires awareness of the disease and a high index of suspicion when examining patients with portal hypertension and dyspnea.
authors.affiliation: Division of Gastroenterology, Department of Internal Medicine, Chang Gung Memorial Hospital, Taipei, Taiwan, ROC.
authors.forename: Chen-Ming
authors.initials: CM
authors.lastname: Hsu
authors.forename: Cheng-Tang
authors.initials: CT
authors.lastname: Chiu
authors.forename: Jau-Min
authors.initials: JM
authors.lastname: Lien
authors.forename: Kwai-Fong
authors.initials: KF
authors.lastname: Ng
mesh_terms: D000328:Adult; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology*; D006976:Hypertension, Pulmonary / Q000209:etiology*; D008103:Liver Cirrhosis / Q000151:congenital*; D008297:Male; D011379:Prognosis