title: Successful cardiac transplantation outcomes in patients with adult congenital heart disease.
abstract: The purpose of our study is (1) to characterise patients with congenital heart disease undergoing heart transplantation by adult cardiac surgeons in a large academic medical centre and (2) to describe successful outcomes associated with our multidisciplinary approach to the evaluation and treatment of adults with congenital heart disease (ACHD) undergoing orthotopic heart transplantation (OHT).
  
  
  
  Heart failure is the leading cause of death in patients with ACHD leading to increasing referrals for OHT.
  
  
  
  The Penn Congenital Transplant Database comprises a cohort of patients with ACHD who underwent OHT between March 2010 and April 2016. We performed a retrospective cohort study of the 20 consecutive patients. Original cardiac diagnoses include single ventricle palliated with Fontan (n=8), dextro-transposition of the great arteries after atrial switch (n=4), tetralogy of Fallot (n=4), pulmonary atresia (n=1), Ebstein anomaly (n=1), unrepaired ventricular septal defect (n=1) and Noonan syndrome with coarctation of the aorta (n=1).
  
  
  
  Eight patients required pretransplant inotropes and two required pretransplant mechanical support. Nine patients underwent heart-liver transplant and three underwent heart-lung transplant. Three patients required postoperative mechanical circulatory support. Patients were followed for an average of 38 months as of April 2016, with 100% survival at 30 days and 1 year and 94% overall survival (19/20 patients).
  
  
  
  ACHD-OHT patients require highly specialised, complex and multidisciplinary healthcare. The success of our programme is attributed to using team-based, patient-centred care including our multidisciplinary staff and specialists across programmes and departments.
authors.affiliation: Division of Cardiology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, USA.
authors.forename: Jonathan N
authors.initials: JN
authors.lastname: Menachem
authors.affiliation: Division of Cardiology, University of Michigan, Ann Arbor, Michigan, USA.
authors.forename: Jessica R
authors.initials: JR
authors.lastname: Golbus
authors.affiliation: Department of Cardiovascular Surgery, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, USA.
authors.forename: Maria
authors.initials: M
authors.lastname: Molina
authors.affiliation: Division of Cardiology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, USA.
authors.forename: Jeremy A
authors.initials: JA
authors.lastname: Mazurek
authors.affiliation: Division of Cardiology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, USA.
authors.forename: Nicole
authors.initials: N
authors.lastname: Hornsby
authors.affiliation: Department of Cardiovascular Surgery, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, USA.
authors.forename: Pavan
authors.initials: P
authors.lastname: Atluri
authors.affiliation: Division of Cardiothoracic Surgery, Department of Surgery, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
authors.forename: Stephanie
authors.initials: S
authors.lastname: Fuller
authors.affiliation: Division of Cardiology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, USA.
authors.forename: Edo Y
authors.initials: EY
authors.lastname: Birati
authors.affiliation: Division of Cardiology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, USA.
authors.forename: Yuli Y
authors.initials: YY
authors.lastname: Kim
authors.affiliation: Division of Cardiology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, USA.
authors.forename: Lee R
authors.initials: LR
authors.lastname: Goldberg
authors.affiliation: Division of Cardiology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, USA.
authors.forename: Joyce W
authors.initials: JW
authors.lastname: Wald
mesh_terms: D000328:Adult; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000601:surgery*; D016027:Heart Transplantation; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends; D013997:Time Factors; D016896:Treatment Outcome; D014481:United States / Q000453:epidemiology; D055815:Young Adult
keywords: Congenital heart disease; Heart transplantation