Elevated right ventricular pressures are not a contraindication to liver transplantation in alagille syndrome

Background. Elevated right ventricle pressure resulting from pulmonary arte ry stenoses may affect outcome and survival after liver transplantation in patients with Alagille syndrome. Methods and Results. Between 1984 and 1997, among 444 pediatric liver trans plant 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 pulmonar y artery stenosis. In 10 patients considered to have elevated RV pressure o n ECG and/or Doppler-echocardiography, a cardiac catheterization was perfor med before liver transplantation. Mean RV systolic pressure was 55 mm-Hg (m edian 49.5 mmHg, range 35-98 mm Hg), mean RV to left ventricular systolic p ressure 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 transplantat ion from noncardiac causes. In two of them, cardiac catheterization before transplantation showed a RV to left ventricular pressure ratio of 0.51 in o ne and 0.37 in the second. In the three others, echocardiography before tra nsplantation 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, n one complained of pruritus and body weight was normalized in 70%. None of t he patients presented cardiac symptoms, arrythmias, or worsening of their c ardiac status. Conclusions. Liver transplantation can be performed safely in children with Alagille syndrome, even in the presence of elevated right ventricular pres sure.