BACKGROUND: Elevated right ventricle pressure resulting from pulmonary artery stenoses may affect outcome and survival after liver transplantation in @entity1 with @entity8404 . METHODS AND RESULTS: Between 1984 and 1997, among 444 pediatric liver transplant recipients, 17 had liver transplantation for @entity8404 (mean age 3.5 years, range 1.2-13 years), mainly because of poor quality of life with intractable @entity111 , and failure to thrive. All @entity1 had @entity849 . In 10 @entity1 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 @entity1 (median 0.66, range 0.5-0.8). All @entity1 underwent successful liver transplantation. Five @entity1 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 @entity111 and body weight was normalized in 70%. None of the @entity1 presented @entity299 , @entity943 , or worsening of their cardiac status. CONCLUSIONS: Liver transplantation can be performed safely in @entity1 with @entity8404 , even in the presence of elevated right ventricular pressure.

Elevated right ventricular pressures are not a contraindication to liver transplantation in XXXX .

@entity1 :: ('9606', 'Species') :: ['children', 'patients'] @entity8404 :: ('MESH:D016738', 'Disease') :: ['Alagille syndrome'] @entity111 :: ('MESH:D011537', 'Disease') :: ['pruritus'] @entity849 :: ('MESH:D011666', 'Disease') :: ['pulmonary artery stenosis'] @entity299 :: ('MESH:D006331', 'Disease') :: ['cardiac symptoms'] @entity943 :: ('MESH:D001145', 'Disease') :: ['arrhythmias']
@entity8404 :: (MESH:D016738,Disease) :: ['Alagille syndrome']
