title: Trends and outcomes of patients with adult congenital heart disease and pulmonary hypertension listed for orthotopic heart transplantation in the United States.
abstract: Heart transplantation is increasing in patients with adult congenital heart disease (ACHD). In this population, the association of pulmonary hypertension (PH) with post-transplant outcomes is not well-defined.
  
  
  
  Using data from the United Network for Organ Sharing database (1987 to 2014), we identified ACHD patients listed for heart transplantation, and examined survival between those with and without PH (pre-transplant PH defined as transpulmonary pressure gradient >12 mm Hg).
  
  
  
  Among 983 ACHD patients, 216 (22%) had PH. At time of listing, PH patients had a transpulmonary pressure gradient of 17.0 mm Hg vs 6.0 mm Hg (p < 0.01) in the no-PH group. Although left ventricular assist device (LVAD) use was infrequent, 3.1% of PH patients were treated with an LVAD versus 6.8% of the no-PH patients. Days from listing to transplant, days from listing to death on the waitlist and length of post-transplant hospitalization were not significantly different between the PH and no-PH groups. However, PH was associated with higher waitlist mortality (HR 1.73, CI 1.25 to 2.41). Pre-transplant PH was not associated with post-transplant mortality at 30 days (HR 0.51, CI 0.23 to 1.13), 1 year (HR 0.68, 95% CI 0.40 to 1.18) or 5 years (HR 0.84, 95% CI 0.55 to 1.29).
  
  
  
  PH is common among ACHD patients listed for transplant and is associated with increased waitlist mortality. Conversely, PH was not associated with worse survival after transplant. Bridge-to-transplant LVAD therapy was uncommon in this ACHD population.
authors: Duke University School of Medicine, Durham, North Carolina; Department of Medicine, Duke University Medical Center, Durham, North Carolina.
authors: Yamini
authors: Y
authors: Krishnamurthy
authors: Department of Medicine, Duke University Medical Center, Durham, North Carolina; Duke Clinical Research Institute and the Department of Medicine, Duke University Medical Center, Durham, North Carolina.
authors: Lauren B
authors: LB
authors: Cooper
authors: Duke Clinical Research Institute and the Department of Medicine, Duke University Medical Center, Durham, North Carolina.
authors: Di
authors: D
authors: Lu
authors: Department of Surgery, Duke University Medical Center, Durham, North Carolina.
authors: Jacob N
authors: JN
authors: Schroder
authors: Department of Surgery, Duke University Medical Center, Durham, North Carolina.
authors: Mani A
authors: MA
authors: Daneshmand
authors: Department of Medicine, Duke University Medical Center, Durham, North Carolina; Duke Clinical Research Institute and the Department of Medicine, Duke University Medical Center, Durham, North Carolina.
authors: Joseph G
authors: JG
authors: Rogers
authors: Duke Clinical Research Institute and the Department of Medicine, Duke University Medical Center, Durham, North Carolina; Department of Surgery, Duke University Medical Center, Durham, North Carolina.
authors: Carmelo A
authors: CA
authors: Milano
authors: Department of Medicine, Duke University Medical Center, Durham, North Carolina; Duke Clinical Research Institute and the Department of Medicine, Duke University Medical Center, Durham, North Carolina.
authors: Adrian F
authors: AF
authors: Hernandez
authors: Department of Medicine, Duke University Medical Center, Durham, North Carolina; Duke Clinical Research Institute and the Department of Medicine, Duke University Medical Center, Durham, North Carolina. Electronic address: chetan.patel@duke.edu.
authors: Chetan B
authors: CB
authors: Patel
mesh_terms: D000328:Adult; D006330:Heart Defects, Congenital; D006333:Heart Failure; D016027:Heart Transplantation; D006353:Heart-Assist Devices; D006801:Humans; D006976:Hypertension, Pulmonary; D012189:Retrospective Studies; D016896:Treatment Outcome; D014481:United States; D014850:Waiting Lists
keywords: adult congenital heart disease; mechanical circulatory support; orthotopic heart transplantation; outcomes; pulmonary hypertension