Background: Intrapulmonary shunting (IPS) is frequently observed and causes
hypoxaemia in liver cirrhosis. This study investigated the prevalence, pre
dictors of reversibility and the effect of living-related liver transplanta
tion (LRLT) on IPS using contrast echocardiography in patients with end-sta
ge biliary atresia.
Methods: Fifty consecutive patients with biliary atresia were examined for
IPS using contrast echocardiography before and after LRLT until IPS disappe
ared. The severity of IPS was classified into five grades according to the
extent of contrast in the left ventricle (0, none; 1, trivial; 2, apparent;
3, complete but less than in right ventricle; 4, as dense as in right vent
Results: Thirty-two patients (grade 1, n = 15; grade 2, n = 7; grade 3, n =
4; grade 4, n = 6) had IPS before LRLT. Forty-four patients have survived
for 9-26 months after LRLT. Among patients with grade 3 or 4 IPS (n = 8), t
here was a significant correlation between age at LRLT and the duration of
IPS persistence after LRLT (P = 0.044).
Conclusion: IPS takes longer to disappear in older children than in younger
ones, but always disappears eventually. LRLT is an effective treatment for
biliary atresia with IPS.