There is no consensus on imaging the head and neck for vascular anomalies in the absence of symptoms, as surgical intervention is unlikely under those circumstances. However, repair of a coarctation of the abdominal aorta or renal artery stenosis

should be considered prior to LT.[161] Comorbidities resulting from multiorgan selleck inhibitor involvement have a significant impact on the outcome of LT, with structural cardiac disease being the most important contributor to mortality.[152, 162] Increased intraoperative fluid requirements place a significant burden on cardiac function and pulmonary blood flow. Patients with AGS often have established right ventricular hypertrophy which, coupled with increased pulmonary vascular resistance associated with pulmonary selleck kinase inhibitor artery stenosis, may increase the risk of diminished cardiac output and poor graft perfusion. Echocardiogram alone may be insufficient to assess the descending aorta and peripheral pulmonary artery branches.[151] Utilization of a dynamic stress test with dobutamine during cardiac catheterization can identify those patients who can successfully increase their cardiac output by over 40%, the necessary cardiac response for successful LT.[163] Posttransplant survival rates vary between

82.9% and 87% at 1 year, 78.4% and 86% at 5 years,[164] and 80.9% at 10 years.[165] Long-term survival rates between AGS and all other pediatric liver transplant recipients were reported to be similar in a single-center experience.[165] However, a review of the UNOS database revealed 5-year graft and patient survival

was worse for AGS compared BA patients, 61.5% versus 70% (P = 0.02) and 78.4% versus 84% (P = 0.01), respectively.[164] Risk factors for poor outcome among AGS patients included neurological and cardiac complications. Renal disease associated with AGS will require a renal-sparing immunosuppressive this website regimen to minimize the risk of renal dysfunction following LT.[166] 36. Patients with AGS should be carefully assessed for evidence of extrahepatic manifestations of this multisystem disorder; decisions regarding liver transplant should be individualized to include potential nontransplant treatment options for nonlife-threatening complications such as intractable pruritus and deforming xanthoma with biliary diversion or ileal exclusion. (1-B). 37. Realistic expectations related to growth potential following LT should be made clear to the family. (1-B) 38. Careful assessment of cardiac and renal function should occur during LT evaluation in all liver transplant candidates. (2-B) 39. Pretransplant vascular imaging of the intra-abdominal vasculature should be performed (2-B); vascular imaging of the head and neck may be considered. (2-C) Wilson’s disease (WD) is a chronic liver condition with a myriad of presentations. WD may be clinically indistinguishable from autoimmune hepatitis, nonalcoholic fatty liver disease, or cryptogenic cirrhosis.