Hepatopulmonory syndrome--an uncommon and severe complication of certain liver diseases.

ABSTRACT

Hepatopulmonary syndrome (HPS) is charaterised by arterial hypoxaemia found mainly in association with chronic liver disease (most commonly cirrhosis of liver) but also rarely with acute liver diseases like fulminant hepatic failure or Budd-Chiari syndrome. The purpose of this article is to present an uncommon but grave complication mostly associated with cirrhosis of liver and to differentiate this entry from cyanotic heart diseases or certain lung conditions. Investigations include history and physical examination, certain biochemical tests, upper gastro-intestinal (GI) endoscopy, abdominal ultrasonography, liver biopsy and venacavography in appropriate cases. Finally, the suspected cases underwent arterial blood gas (ABG) analysis and contrast enhanced echocardiography (CEE) for confirmation of the diagnosis of HPS. Of the 123 cases of cirrhosis of liver, three cases of HPS (2.4%) were found-all of them being males. Also one male patient with inferior vena cava (IVC) obstruction amongst other causes presenting with HPS was encountered. As of now, no medical treatment has been proved to be useful and liver transplant remains the only hope for this disorder.