Hemodynamic changes in patients with Alagille's syndrome during orthotopicliver transplantation

Children with Alagille's syndrome are at increased perioperative risk durin g 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 transpl antations. 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 mea n pulmonary artery pressure and central venous pressure increased by 3 +/- 4 mm Hg and 1 +/- 4 mm Hg, respectively, at portal vein unclamping. There w as no correlation between severity of pulmonary artery stenosis and hemodyn amic changes. Veno-venous bypass used in four cases resulted in smaller hem odynamic changes. Time to extubation and duration of intensive care unit st ay were unrelated to severity of pulmonary artery stenosis. Implications: S ome children with Alagille's syn drome require liver transplantation. In ou r study, associated pulmonary artery stenosis did not dramatically increase perioperative risk. Veno-venous bypass decreased intraoperative hemodynami c changes in these patients.