[374, 377-381] 85. The role of LT in NCPH should be considered in those patients with cardiopulmonary complications of portal hypertension. (2-B) LT has been successfully

performed in a small number of children and adults with advanced liver disease in the setting of sickle cell anemia, but morbidity from vascular thrombosis including graft thrombosis, stroke or pulmonary embolus, and infections is common.[382-384] Vaso-occlusive crises continue after LT.[384] Careful patient selection as well as management of the sickle cell disease, including exchange transfusion, is required in order to successfully perform LT in patients with this systemic MEK inhibitor disorder. Hepatopulmonary syndrome (HPS) is a condition in which intrapulmonary vascular dilatations (IPVD) develop in the setting of portal systemic shunting.[385] The presence of HPS is associated with increased morbidity and mortality,[386] but is generally reversible after transplantation and is not a contraindication for transplantation.[387] ACP-196 molecular weight HPS is present in 4 to 29% chronic liver disease patients of all ages.[43, 388, 389] Among patients with biliary

atresia, HPS may occur more commonly in children with splenic malformation syndrome.[125, 381] It is important to recognize that HPS can occur in patients without evidence of liver dysfunction (e.g., congenital hepatic fibrosis, portal vein thrombosis, cavernous Oxymatrine transformation of the portal vein). The diagnosis of HPS in children is confirmed by the presence of hypoxia and one of the following

demonstrating the presence of IPVD: 1) contrast-enhanced transthoracic echocardiography; 2) technetium-labeled macro-aggregated albumin lung perfusion scan demonstrating a shunt fraction of >6%; or 3) cardiac catheterization demonstrating IPVD.[44] Severe shunting of >20%, as determined by macro-aggregated albumin scan, is associated with increased posttransplantation morbidity and mortality in adults.[44, 386] The median survival in the absence of LT in adults with severe HPS (paO2 <50 mmHg) is less than 12 months, but is unknown in children.[390-392] Patients with HPS may benefit from supplemental oxygen, particularly during periods of increased physical activity.[12] LT is appropriate for the treatment of HPS in children with cirrhotic liver disease and may be appropriate in some noncirrhosis patients with HPS. Noncirrhotic liver disease or congenital/acquired portosystemic venous communications (e.g., Abernathy syndrome) resulting in HPS may present opportunities for alternative nontransplant approaches to management.[387, 393-396] These approaches include ligation of the shunt or endovascular treatment using an occlusion device placed by an interventional radiologist. 86.