Hemodynamic changes in patients with Alagille's syndrome during orthotopic liver transplantation.

Children with Alagille's syndrome are at increased perioperative risk during orthotopic liver transplantation due to the cardiopulmonary abnormalities and the hemodynamic changes associated with this procedure. We studied 16 children with Alagille's syndrome who underwent 21 orthotopic liver transplantations. Peripheral pulmonary stenosis was present in all subjects. Right ventricular pressures were increased in 15 cases. Caval clamping resulted in a mean decrease of 15 +/-9 mm Hg in systolic blood pressure, 5 +/- 3 mm Hg in mean pulmonary artery pressure, and 4 +/- 3 mm Hg in central venous pressure. Systolic blood pressure decreased by 16 +/- 13 mm Hg, whereas mean pulmonary artery pressure and central venous pressure increased by 3 +/- 4 mm Hg and 1 +/- 4 mm Hg, respectively, at portal vein unclamping. There was no correlation between severity of pulmonary artery stenosis and hemodynamic changes. Veno-venous bypass used in four cases resulted in smaller hemodynamic changes. Time to extubation and duration of intensive care unit stay were unrelated to severity of pulmonary artery stenosis. IMPLICATIONS: Some children with Alagille's syndrome require liver transplantation. In our study, associated pulmonary artery stenosis did not dramatically increase perioperative risk. Veno-venous bypass decreased intraoperative hemodynamic changes in these patients.