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: