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.
Original language | English |
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Pages (from-to) | 193-198 |
Number of pages | 6 |
Journal | Chang Gung Medical Journal |
Volume | 26 |
Issue number | 3 |
Publication status | Published - Mar 1 2003 |
Externally published | Yes |
Keywords
- Congenital hepatic fibrosis
- Plexogenic pulmonary arteriopathy
- Portal hypertension
- Pulmonary hypertension
ASJC Scopus subject areas
- Medicine(all)