Intrapulmonary vascular abnormalities consisting of arteriovenous malformations and capillary dilatations have been described in patients with severe liver disease. These intrapulmonary vascular abnormalities can result in intrapulmonary right-to-left shunting and hypoxemia. Twenty-five of 53 patients (47%) with end-stage hepatic disease were found to have contrast echocardiographic evidence of intrapulmonary right-to-left shunting. There was no difference in mean age, gender distribution, or severity of hepatic disease in those with and without evidence of such shunting. Although there was no difference in mean partial arterial oxygen pressure (PaO2) values in the 2 groups (82 +/- 11 vs 76 +/- 11 mm Hg), the mean PaO2 value of those with at least 2+ left ventricular opacification (2 to 4+) was significantly lower (66 +/- 3 mm Hg, n = 8; p less than 0.01). Unexpectedly, patients with evidence of intrapulmonary shunting had a lower mortality rate before transplantation (3 of 25, 12%) than those without evidence of shunting (10 of 28, 36%) resulting in a significant difference in actuarial survival (p less than 0.05) by the end of the follow-up period. It is concluded that intrapulmonary right-to-left shunting occurs frequently in patients with end-stage liver disease and may be a marker of a positive biologic process that, in some way, leads to improved short-term survival.