title: Liver transplantation in children with congenital cardiac defects: a case report and a short literature review.
abstract: Advances in surgical techniques and follow-up of patients with complex congenital heart disease who were corrected in childhood increasingly survive to adolescence or adulthood. Increasingly anesthesiologists encounter these cases for major noncardiac surgery, including orthotopic liver transplantation (OLT) wherein there is an augmented risk of significant perioperative hemodynamic instability. We performed a successful OLT in a 12-year-old boy with end-stage cryptogenetic liver fibrosis and hepatopulmonary syndrome who was born with a double outflow right ventricle, pulmonary atresia, and pulmonary artery hypoplasia corrected at the age of 1 month. By the time he was considered for OLT his altered pulmonary valve apparatus resulted in severe pulmonary regurgitation, dilated right atrium and ventricle, and elevated right heart pressures. After a temporarily successful angioplasty he was at first placed on the waiting list, then removed, and finally relisted following implantation of a prosthetic pulmonary valve that resulted in significant reduction of right heart pressures.
authors.affiliation: Dipartimento di Medicina, UOC Anestesia e Terapia Intensiva, Università degli Studi di Padova, Padova, Italy. Electronic address: paolofeltracco@inwind.it.
authors.forename: P
authors.initials: P
authors.lastname: Feltracco
authors.forename: E
authors.initials: E
authors.lastname: Serra
authors.forename: M
authors.initials: M
authors.lastname: Milevoj
authors.forename: C
authors.initials: C
authors.lastname: Carollo
authors.forename: S
authors.initials: S
authors.lastname: Barbieri
authors.forename: A
authors.initials: A
authors.lastname: Vitale
authors.forename: E
authors.initials: E
authors.lastname: Gringeri
authors.forename: U
authors.initials: U
authors.lastname: Cillo
authors.forename: O
authors.initials: O
authors.lastname: Milanesi
authors.forename: C
authors.initials: C
authors.lastname: Ori
mesh_terms: D002648:Child; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D016031:Liver Transplantation; D008297:Male; D016896:Treatment Outcome; D014463:Ultrasonography