title: Single-center outcomes of combined heart and liver transplantation in the failing Fontan.
abstract: Long-term outcomes of the Fontan operation include Fontan failure and liver disease. Combined heart-liver transplantation (CHLT) is an option for select patients although limited data exist on this strategy. A retrospective review of Fontan patients 18 years or older referred for cardiac transplant evaluation between 2000 and 2013 at the Hospital of the University of Pennsylvania was performed. All patients were considered for potential CHLT. Clinical variables such as demographics, perioperative factors, and short-term outcomes were reviewed. Of 17 referrals for cardiac transplantation, seven Fontan patients underwent CHLT. All patients who underwent CHLT had either advanced fibrosis or cirrhosis on liver biopsy. There were no perioperative deaths. The most common postoperative morbidity was acute kidney injury. Short-term complications include one episode of acute liver rejection but no cardiac rejection greater than 1R. CHLT is an acceptable therapeutic option for patients with failing Fontan physiology who exhibit concomitant advanced liver fibrosis. However, optimal patient selection is currently undefined, and long-term outcomes are not known.
authors.affiliation: Division of Cardiology, Department of Medicine, Hospital of the University of Pennsylvania, Philadelphia, PA, USA.
authors.forename: Benjamin A
authors.initials: BA
authors.lastname: D'Souza
authors.affiliation: Division of Cardiac Surgery, Department of Surgery, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
authors.forename: Stephanie
authors.initials: S
authors.lastname: Fuller
authors.affiliation: Division of Cardiac Surgery, Department of Surgery, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
authors.forename: Lacey P
authors.initials: LP
authors.lastname: Gleason
authors.affiliation: Division of Cardiology, Department of Medicine, Hospital of the University of Pennsylvania, Philadelphia, PA, USA.
authors.forename: Nicole
authors.initials: N
authors.lastname: Hornsby
authors.affiliation: Division of Cardiology, Department of Medicine, Hospital of the University of Pennsylvania, Philadelphia, PA, USA.
authors.forename: Joyce
authors.initials: J
authors.lastname: Wald
authors.affiliation: Division of Gastroenterology, Department of Medicine, Penn State Hershey Medical Center, Hershey, PA, USA.
authors.forename: Karen
authors.initials: K
authors.lastname: Krok
authors.affiliation: Division of Transplant Surgery, Department of Surgery, Hospital of the University of Pennsylvania, Philadelphia, PA, USA.
authors.forename: Abraham
authors.initials: A
authors.lastname: Shaked
authors.affiliation: Division of Cardiology, Department of Medicine, Hospital of the University of Pennsylvania, Philadelphia, PA, USA.
authors.forename: Lee R
authors.initials: LR
authors.lastname: Goldberg
authors.affiliation: Division of Cardiac Surgery, Department of Surgery, Mayo Clinic, Rochester, MN, USA.
authors.forename: Alberto
authors.initials: A
authors.lastname: Pochettino
authors.affiliation: Division of Transplant Surgery, Department of Surgery, Hospital of the University of Pennsylvania, Philadelphia, PA, USA.
authors.forename: Kim M
authors.initials: KM
authors.lastname: Olthoff
authors.affiliation: Division of Cardiology, Department of Medicine, Hospital of the University of Pennsylvania, Philadelphia, PA, USA.
authors.forename: Yuli Y
authors.initials: YY
authors.lastname: Kim
mesh_terms: D000328:Adult; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000379:methods*; D006084:Graft Rejection / Q000209:etiology*; D006085:Graft Survival; D006331:Heart Diseases / Q000601:surgery*; D016027:Heart Transplantation / Q000009:adverse effects*; D006801:Humans; D008107:Liver Diseases / Q000601:surgery*; D016031:Liver Transplantation / Q000009:adverse effects*; D008297:Male; D011183:Postoperative Complications; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D055815:Young Adult
keywords: Fontan; combined heart and liver transplantation; congenital heart disease; liver cirrhosis; single ventricle