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The hepatopulmonary syndrome.

Abstract
The hepatopulmonary syndrome is a triad of liver disease, increased alveolar-arterial oxygen gradient and intrapulmonary vascular dilatations. Manifestations include orthodeoxia, platypnoea and hyperdynamic circulation. Intrapulmonary vascular abnormalities, perhaps mediated by nitric oxide, cause hypoxaemia by shunting, a perfusion-diffusion defect, and ventilation-perfusion mismatching. Contrast-enhanced echocardiography is the method of choice for demonstrating pulmonary vascular abnormalities, although perfusion lung scanning is a more specific and sensitive test. Angiography is best reserved for patients with poor response to 100% oxygen and defines whether vascular dilatations are of the diffuse 'spongy' type or, less commonly, discrete arteriovenous communications amenable to embolization. About 80% of patients with the hepatopulmonary syndrome eventually have improved oxygenation after liver transplantation, thereby making worsening hypoxaemia the primary indication for transplantation in many instances. Nevertheless, severe hypoxaemia carries a peri-operative mortality of 30% and reliable predictors of successful outcome after transplantation remain to be determined.
AuthorsL S Aboussouan, J K Stoller
JournalBailliere's best practice & research. Clinical gastroenterology (Baillieres Best Pract Res Clin Gastroenterol) Vol. 14 Issue 6 Pg. 1033-48 (Dec 2000) England
PMID11139353 (Publication Type: Journal Article, Review)
CopyrightCopyright 2000 Harcourt Publishers Ltd.
Topics
  • Combined Modality Therapy
  • Female
  • Hepatopulmonary Syndrome (diagnosis, mortality, therapy)
  • Humans
  • Male
  • Prognosis
  • Severity of Illness Index
  • Survival Rate

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