Pressure gradients, laboratory changes, and outcomes with transjugular intrahepatic portosystemic shunts in pediatric portal hypertension.

Abstract

Indications for TIPS are well described in adults and involve complications of PHTN. Complications from PHTN are associated with PSG of>12mmHg in adults. It is unclear if these parameters apply to children with PHTN. To assess whether adult criteria for TIPS placement can be utilized in children, describe laboratory changes over time, and report outcomes. We performed a retrospective review of 34 pediatric patients who underwent TIPS, examining indications, radiology, PSG reductions, laboratory changes, and outcomes. Most patients had PHTN due to parenchymal liver disease including congenital hepatic fibrosis (n=5), biliary atresia (n=5), cystic fibrosis-related liver disease (n=3) and cavernous transformation of the portal vein (n=6). Indications for TIPS included variceal bleeding, recurrent ascites, and maintenance of portal vein flow following thrombolysis. Variceal bleeding was observed in six children with PSG<12mmHg. Minor complications occurred in eight subjects. Continued bleeding occurred in one patient. Six patients were successfully bridged to transplantation, and three patients died secondary to end-stage disease. Standard laboratory tests stabilized after TIPS placement and hematocrit increased. TIPS placement in pediatric patients was performed for complications of PHTN. Unlike adult series, a substantial proportion of our cases treated extrahepatic PHTN from cavernous transformation of the portal vein. Children presented with sequelae of PHTN with PSG below 12mmHg, below the adult standard. We found TIPS in pediatrics to be safe and effective with laboratory stabilization and improvement in hematocrit.