Elevated right ventricular pressures are not a contraindication to liver transplantation in Alagille syndrome.
BACKGROUND
Elevated right ventricle pressure resulting from pulmonary artery stenoses may affect outcome and survival after liver transplantation in patients with Alagille syndrome.


RESULTS
Between 1984 and 1997, among 444 pediatric liver transplant recipients, 17 had liver transplantation for Alagille syndrome (mean age 3.5 years, range 1.2-13 years), mainly because of poor quality of life with intractable pruritus, and failure to thrive. All patients had pulmonary artery stenosis. In 10 patients considered to have elevated RV pressure on ECG and/or Doppler-echocardiography, a cardiac catheterization was performed before liver transplantation. Mean RV systolic pressure was 55 mmHg (median 49.5 mmHg, range 35-98 mm Hg), mean RV to left ventricular systolic pressure ratio 0.53 (median 0.53, range 0.29-0.78) with a ratio above 0.5 in 6 patients (median 0.66, range 0.5-0.8). All patients underwent successful liver transplantation. Five patients died 1 to 9 months after transplantation from noncardiac causes. In two of them, cardiac catheterization before transplantation showed a RV to left ventricular pressure ratio of 0.51 in one and 0.37 in the second. In the three others, echocardiography before transplantation estimated RV pressures below 0.5 systemic pressures. At follow-up (median 6 years, range 1.5-15 years), liver tests were normal in all, none complained of pruritus and body weight was normalized in 70%. None of the patients presented cardiac symptoms, arrhythmias, or worsening of their cardiac status.


CONCLUSIONS
Liver transplantation can be performed safely in children with Alagille syndrome, even in the presence of elevated right ventricular pressure.
Ovaert
C
C
Department of Pediatric Cardiology, Cliniques Universitaires Saint-Luc, Avenue Hippocrate 10, 1200 Brussels, Belgium.
Germeau
C
C
Barrea
C
C
Moniotte
S
S
Van Obbergh
L
L
Sokal
E
E
Reding
R
R
Otte
J B
JB
Sluysmans
T
T
D000293:Adolescent; D016738:Alagille Syndrome / Q000601:surgery*; D001157:Arterial Occlusive Diseases / Q000150:complications*; D001794:Blood Pressure; D006328:Cardiac Catheterization; D002648:Child; D002675:Child, Preschool; D000075202:Contraindications; D005500:Follow-Up Studies; D006439:Hemodynamics; D006801:Humans; D007223:Infant; D016031:Liver Transplantation / Q000379:methods / Q000502:physiology; D011651:Pulmonary Artery; D013997:Time Factors; D018497:Ventricular Dysfunction, Right / Q000503:physiopathology*; D016277:Ventricular Function, Left