title: Anesthetic management of a combined heart and liver transplantation in an amyloidotic patient: a case report.
abstract: Few cases of combined heart and liver transplantation (CHLT) have been reported for familial amyloidosis. Our first CHLT was performed on a female patient with familial amyloidosis due to a genetic defect in transthyretin, characterized by deposition of amyloid in various organs and tissues. This disease produced autonomic heart dysfunction that preceded the development of clinical manifestations and may be an important factor in determining the optimal timing for liver transplantation. CHLT can be performed successfully, even in patients with advanced disease. However, the most compromised patients are more exposed to intraoperative risks, postoperative complications, and worsening of extracardiac and extrahepatic symptoms. Our patient presented severe cardiac dysfunction requiring CHLT. The operative technique is far from being consolidated, despite this, both organs were transplanted in the same day with 2 hours in the intensive care unit (ICU) between surgeries. The outcome of both organs has been favorable. The amyloidotic liver was transplanted to another patient, a sequential (domino) transplantation.
authors: Department of Anesthesiology, Hospital Doce de Octubre, Madrid, Spain. mariabarrioperez@yahoo.es
authors: I M
authors: IM
authors: Barrio
authors: M A
authors: MA
authors: Mtnez de Guereñu
authors: M I
authors: MI
authors: Real
authors: I
authors: I
authors: Del Campo
authors: F
authors: F
authors: Pérez-Cerdá
authors: E
authors: E
authors: Moreno
mesh_terms: D019943:Amino Acid Substitution; D028226:Amyloidosis, Familial / Q000235:genetics / Q000601:surgery*; D000758:Anesthesia / Q000379:methods*; D005260:Female; D016027:Heart Transplantation; D006498:Hepatectomy; D006801:Humans; D008111:Liver Function Tests; D016031:Liver Transplantation; D019520:Living Donors; D008875:Middle Aged; D011228:Prealbumin / Q000235:genetics; D016896:Treatment Outcome
chemical_list: D011228:Prealbumin