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


METHODS
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 microg/kg/min and 20 microg/kg/min by using a thermodilution catheter placed in the pulmonary artery.


RESULTS
There was a significant change in the baseline cardiac index (P<0.001) with an infusion of 20 microg/kg/min of dobutamine with an increase from 4.4(1.0) L/min/m2 to 6.4(1.5) L/min/m2. There was, however, a wide variation between individuals in the increase in cardiac index, ranging from 7.3-95%. There was no correlation between the baseline systolic right ventricular/aortic pressure ratios and the increase in cardiac index (r=0.1086).


CONCLUSIONS
The increase in cardiac index, in response to dobutamine in patients with Alagille's syndrome, is independent of the more conventional measurement of the right ventricular pressure. This method of producing a hemodynamic response is closer to the response that results after liver transplantation, and thus, it may be a better way of predicting the outcome of liver transplantation in these patients.
Razavi
R S
RS
Department of Paediatric Cardiology, Guy's Hospital, St. Thomas' Street, London SE1 9RT.
Baker
A
A
Qureshi
S A
SA
Rosenthal
E
E
Marsh
M J
MJ
Leech
S C
SC
Rela
M
M
Mieli-Vergani
G
G
D000293:Adolescent; D016738:Alagille Syndrome / Q000503:physiopathology* / Q000601:surgery; D001653:Bile Ducts, Intrahepatic / Q000002:abnormalities; D001794:Blood Pressure / Q000187:drug effects; D002302:Cardiac Output / Q000187:drug effects; D002316:Cardiotonic Agents / Q000008:administration & dosage*; D002648:Child; D002675:Child, Preschool; D004280:Dobutamine / Q000008:administration & dosage*; D006439:Hemodynamics / Q000187:drug effects*; D006801:Humans; D007223:Infant; D016031:Liver Transplantation; D011379:Prognosis; D011666:Pulmonary Valve Stenosis / Q000503:physiopathology
D002316:Cardiotonic Agents; D004280:Dobutamine