title: Management of a cadaveric orthotopic liver transplantation in a pediatric patient with complex congenital heart disease.
abstract: Pediatric orthotopic liver transplantations (OLT) are commonly performed nowadays. Two primary reasons for OLT in children are complications from either extrahepatic biliary atresia (EHBA) or inborn errors of metabolism. However, congenital liver disease may be associated with significant other congenital abnormalities. We present a case of a successful OLT in a pediatric patient with a history of EHBA, situs inversus, and complex congenital heart disease. The cardiac anomalies include dextrocardia, absence of the atrial septum (single atrium), single atrioventricular valve (a-v canal), and an incomplete ventricular septum. Prior surgery include a Kasai procedure for EHBA, banding of the proximal main pulmonary artery, and Broviac catheter placement. We present the anesthesia concerns and management for this complicated case.
authors.affiliation: The Mount Sinai Medical Center, New York, NY, USA. dfeierman@nyc.rr.com
authors.forename: Dennis E
authors.initials: DE
authors.lastname: Feierman
authors.forename: Francine S
authors.initials: FS
authors.lastname: Yudkowitz
authors.forename: Joanne
authors.initials: J
authors.lastname: Hojsak
authors.forename: Sukru
authors.initials: S
authors.lastname: Emre
mesh_terms: D000015:Abnormalities, Multiple; D001656:Biliary Atresia / Q000150:complications / Q000601:surgery*; D002102:Cadaver; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications*; D006801:Humans; D007223:Infant; D007430:Intraoperative Care; D016031:Liver Transplantation / Q000009:adverse effects / Q000379:methods*; D011182:Postoperative Care; D012857:Situs Inversus / Q000150:complications; D016896:Treatment Outcome