Severe portopulmonary hypertension in congenital hepatic fibrosis

Chen Ming Hsu, Cheng Tang Chiu, Jau Min Lien, Kwai Fong Ng

Research output: Contribution to journalArticlepeer-review

2 Citations (Scopus)


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 languageEnglish
Pages (from-to)193-198
Number of pages6
JournalChang Gung Medical Journal
Issue number3
Publication statusPublished - Mar 1 2003
Externally publishedYes


  • Congenital hepatic fibrosis
  • Plexogenic pulmonary arteriopathy
  • Portal hypertension
  • Pulmonary hypertension

ASJC Scopus subject areas

  • General Medicine


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