title: Outcomes following implantation of mechanical circulatory support in adults with congenital heart disease: An analysis of the Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS).
abstract: Adults with congenital heart disease represent an expanding and unique population of patients with heart failure (HF) in whom the use of mechanical circulatory support (MCS) has not been characterized. We sought to describe overall use, patient characteristics, and outcomes of MCS in adult congenital heart disease (ACHD).
  
  
  
  All patients entered into the Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) between June 23, 2006, and December 31, 2015, were included. Patients with ACHD were identified using pre-operative data and stratified by ventricular morphology. Mortality was compared between ACHD and non-ACHD patients, and multivariate analysis was performed to identify predictors of death after device implantation.
  
  
  
  Of 16,182 patients, 126 with ACHD stratified as follows: systemic morphologic left ventricle (n = 63), systemic morphologic right ventricle (n = 45), and single ventricle (n = 17). ACHD patients were younger (42 years ± 14 vs 56 years ± 13; p < 0.0001) and were more likely to undergo device implantation as bridge to transplant (45% vs 29%; p < 0.0001). A higher proportion of ACHD patients had biventricular assist device (BiVAD)/total artificial heart (TAH) support compared with non-ACHD patients (21% vs 7%; p < 0.0001). More ACHD patients on BiVAD/TAH support were INTERMACS profile 1 compared with patients on systemic left ventricular assist device (LVAD) support (35% vs 15%; p = 0.002). ACHD and non-ACHD patients with LVADs had similar survival; survival was worse for patients on BIVAD/TAH support. BiVAD/TAH support was the only variable independently associated with mortality (early phase hazard ratio 4.4; 95% confidence interval, 1.8-11.1; p = 0.001). For ACHD patients receiving MCS, ventricular morphology was not associated with mortality.
  
  
  
  ACHD patients with LVADs have survival similar to non-ACHD patients. Mortality is higher for patients requiring BiVAD/TAH support, potentially owing to higher INTERMACS profile. These outcomes suggest a promising role for LVAD support in ACHD patients as part of the armamentarium of therapies for advanced HF.
authors.affiliation: Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts. Electronic address: Christina.vanderpluym@childrens.harvard.edu.
authors.forename: Christina J
authors.initials: CJ
authors.lastname: VanderPluym
authors.affiliation: Department of Cardiology, Baylor University Medical Center, Dallas, Texas.
authors.forename: Ari
authors.initials: A
authors.lastname: Cedars
authors.affiliation: Department of Cardiothoracic Surgery, Washington University School of Medicine, St. Louis, Missouri.
authors.forename: Pirooz
authors.initials: P
authors.lastname: Eghtesady
authors.affiliation: Department of Anesthesiology, Randall Children's Hospital, Portland, Oregon.
authors.forename: Bryan G
authors.initials: BG
authors.lastname: Maxwell
authors.affiliation: Knight Cardiovascular Institute, Oregon Health and Science University, Portland, Oregon.
authors.forename: Jill M
authors.initials: JM
authors.lastname: Gelow
authors.affiliation: Knight Cardiovascular Institute, Oregon Health and Science University, Portland, Oregon.
authors.forename: Luke J
authors.initials: LJ
authors.lastname: Burchill
authors.affiliation: Department of Cardiovascular Surgery, Mayo Clinic School of Medicine, Rochester, Minnesota.
authors.forename: Simon
authors.initials: S
authors.lastname: Maltais
authors.affiliation: Department of Cardiothoracic Surgery, University of Alabama at Birmingham, Birmingham, Alabama.
authors.forename: Devin A
authors.initials: DA
authors.lastname: Koehl
authors.affiliation: Department of Cardiothoracic Surgery, University of Alabama at Birmingham, Birmingham, Alabama; Department of Epidemiology, School of Public Health, University of Alabama at Birmingham, Birmingham, Alabama.
authors.forename: Ryan S
authors.initials: RS
authors.lastname: Cantor
authors.affiliation: Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts.
authors.forename: Elizabeth D
authors.initials: ED
authors.lastname: Blume
mesh_terms: D000328:Adult; D016208:Databases, Factual; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications*; D006333:Heart Failure / Q000150:complications* / Q000401:mortality / Q000601:surgery*; D006354:Heart, Artificial; D006353:Heart-Assist Devices; D006801:Humans; D008297:Male; D008875:Middle Aged; D012042:Registries; D015996:Survival Rate; D016896:Treatment Outcome; D014481:United States
keywords: LVAD; congenital heart disease; mechanical circulatory support; single ventricle; total artificial heart; ventricular assist device