Hepatopulmonary syndrome.

ABSTRACT

Hepatopulmonary syndrome (HPS) is defined as clinical triad of advanced liver disease, arterial deoxygenation and intra pulmonary vascular dilatation. It is a rare complication of liver disease of varied etiology and indicates a poor prognosis. Many theories have been put forward to throw light over its pathogenesis. The major clinical manifestations are arterial hypoxemia, clubbed fingers and spider navei. Orthodeoxia and platypnea are usual clinical features. A simple non invasive method to screen HPS is desirable. Contrast enhanced 2D ECHO cardiography is the preferred screening test. No effective medical treatment has been found. Although liver transplant seems feasible to reverse (at least partially) this situation, however it is associated with increased post operative morbidity and mortality.