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Mild and moderate CHD in infants do not always need surgical correction but possibly augment the operative risk of patients with ESLD undergoing OLT. The aim of this study is to assess the intraoperative and post-operative complications and evaluate the outcome of these patients. The records of 196 patients were reviewed retrospectively in a cohort study, 50 CHD were found in 36 patients undergoing 41 OLT procedures. The prevalence of CHD during pediatric OLT was 18%. Our data identified no significant differences between the two groups of patients, regarding hypotension, desaturation, acidosis, and bleeding during the procedure. Post-operatively, no differences were observed in the hemodynamic, respiratory, and renal systems as no differences were detected for graft failure, surgical complications, infection, and rejection rates. Mortality is comparable in the two groups as are re-transplantation and recovery rates. The above mentioned results indicate that minor cardiac defects do not significantly influence the operative risk of these patients.

Liver transplantation is an accepted option of treatment in patients with inborn errors of metabolism limited to or mainly located into hepatocytes who have not responded well to medical treatment. Recurrent metabolic failure and neurological impairment might be an indication for early transplantation in patients with organic acidaemias. We discuss the anaesthetic management and metabolic implications of acidaemia in the first two cases of successfully treated propionic and methylmalonic acidaemia in Italy. A nine and 12 month follow up did not show any further metabolic failure after the procedure, indicating that early liver transplantation improves the quality of life of these patients.