Hemodynamic response to continuous infusion of dobutamine in Alagille's syndrome.

R S Razavi, A Baker, S A Qureshi, E Rosenthal, M J Marsh, S C Leech, M Rela, G Mieli-Vergani

Research output: Contribution to journalArticlepeer-review

32 Citations (Scopus)


Background. Alagille's syndrome is a rare condition that is characterized by paucity of interlobular bile ducts and peripheral pulmonary artery stenosis. Liver transplantation in the setting of peripheral pulmonary stenosis and right ventricular hypertension seems to be associated with a higher mortality, which raises the concern that these patients are unable to increase their cardiac output in the immediate posttransplantation period to meet the demands of reperfusion and early graft dysfunction and cope with further increases in pulmonary vascular resistance. Method. Cardiac catheterization was performed in 15 children with Alagille's syndrome and peripheral pulmonary artery stenosis to measure the cardiac output response to dobutamine infusion. The cardiac output was measured before and during each increment of infusion of dobutamine at 10 mug/kg/min and 20 mug/kg/min by using a thermodilution catheter placed in the pulmonary artery. Results. There was a significant change in the baseline cardiac index (P
Original languageEnglish
Pages (from-to)823 - 828
Number of pages6
Issue number5
Publication statusPublished - 2001


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