title: Perioperative management of pediatric en-bloc combined heart-liver transplants: a case series review.
abstract: BACKGROUND
  Combined heart and liver transplantation (CHLT) in the pediatric population involves a complex group of patients, many of whom have palliated congenital heart disease (CHD) involving single ventricle physiology.
  
  
  OBJECTIVE
  The purpose of this study was to describe the perioperative management of pediatric patients undergoing CHLT at a single institution and to identify management strategies that may be used to optimize perioperative care.
  
  
  METHODS
  We did a retrospective database review of all patients receiving CHLT at a children's hospital between 2006 and 2014. Information collected included preoperative characteristics, intraoperative management, blood transfusions, and postoperative morbidity and mortality.
  
  
  RESULTS
  Five pediatric CHLTs were performed over an 8-year period. All patients had a history of complex CHD with multiple sternotomies, three of whom had failing Fontan physiology. Patient age ranged from 7 to 23 years and weight from 29.5 to 68.5 kg. All CHLTs were performed using an en-bloc technique where both the donor heart and liver were implanted together on cardiopulmonary bypass (CPB). The median operating room time was 14.25 h, median CPB time was 3.58 h, and median donor ischemia time was 4.13 h. Patients separated from CPB on dopamine, epinephrine, and milrinone infusions and two required inhaled nitric oxide. All patients received a massive intraoperative blood transfusion post CPB with amounts ranging from one to three times the patient's estimated blood volume. The patient who required the most transfusions was in decompensated heart and liver failure preoperatively. Four of the five patients received an antifibrinolytic agent as well as a procoagulant (prothrombin complex concentrate or recombinant activated Factor VII) to assist with hemostasis. There were no 30-day thromboembolic events detected. Postoperatively the median length of mechanical ventilation, ICU stay and stay to hospital discharge was 4, 8, and 37 days, respectively. All patients are alive and free from allograft rejection at this time.
  
  
  CONCLUSIONS
  Combined heart and liver transplantation in the pediatric population involves a complex group of patients with unique perioperative challenges. Successful management starts with thorough preoperative planning and communication and involves strategies to deal with massive intraoperative hemorrhage and coagulopathy in addition to protecting and supporting the transplanted heart and liver and meticulous surgical technique. An integrated multidisciplinary team approach is the cornerstone for successful outcomes.
authors.affiliation: Department of Anesthesiology, Perioperative and Pain Medicine, Stanford Children's Hospital, Stanford University Medical Center, Palo Alto, CA, USA.
authors.forename: Manchula
authors.initials: M
authors.lastname: Navaratnam
authors.affiliation: Department of Anesthesiology, Perioperative and Pain Medicine, Stanford Children's Hospital, Stanford University Medical Center, Palo Alto, CA, USA.
authors.forename: Ann
authors.initials: A
authors.lastname: Ng
authors.affiliation: Department of Anesthesiology, Perioperative and Pain Medicine, Stanford Children's Hospital, Stanford University Medical Center, Palo Alto, CA, USA.
authors.forename: Glyn D
authors.initials: GD
authors.lastname: Williams
authors.affiliation: Department of Cardiothoracic Surgery, Stanford Children's Hospital, Stanford University Medical Center, Palo Alto, CA, USA.
authors.forename: Katsuhide
authors.initials: K
authors.lastname: Maeda
authors.affiliation: Department of Anesthesiology, Perioperative and Pain Medicine, Stanford Children's Hospital, Stanford University Medical Center, Palo Alto, CA, USA.
authors.forename: Julianne M
authors.initials: JM
authors.lastname: Mendoza
authors.affiliation: Department of Transplant Surgery, Stanford Children's Hospital, Stanford University Medical Center, Palo Alto, CA, USA.
authors.forename: Waldo
authors.initials: W
authors.lastname: Concepcion
authors.affiliation: Division of Cardiology, Department of Pediatrics, Stanford Children's Hospital, Stanford University Medical Center, Palo Alto, CA, USA.
authors.forename: Seth A
authors.initials: SA
authors.lastname: Hollander
authors.affiliation: Department of Anesthesiology, Perioperative and Pain Medicine, Stanford Children's Hospital, Stanford University Medical Center, Palo Alto, CA, USA.
authors.forename: Chandra
authors.initials: C
authors.lastname: Ramamoorthy
mesh_terms: D000293:Adolescent; D000328:Adult; D001803:Blood Transfusion / Q000706:statistics & numerical data; D002648:Child; D005260:Female; D006330:Heart Defects, Congenital / Q000601:surgery*; D016027:Heart Transplantation / Q000379:methods*; D006801:Humans; D007902:Length of Stay / Q000706:statistics & numerical data; D016031:Liver Transplantation / Q000379:methods*; D008297:Male; D061646:Operative Time; D019990:Perioperative Care / Q000379:methods*; D011183:Postoperative Complications; D012189:Retrospective Studies; D013997:Time Factors; D055815:Young Adult
keywords: antifibrinolytic; blood transfusion; cardiac; coagulation; congenital heart disease; drugs; general anesthesia; hematology; massive transfusions; transplantation