title: Clinical research priorities in adult congenital heart disease.
abstract: BACKGROUND
  Adult congenital heart disease (ACHD) clinicians are hampered by the paucity of data to inform clinical decision-making. The objective of this study was to identify priorities for clinical research in ACHD.
  
  
  METHODS
  A list of 45 research questions was developed by the Alliance for Adult Research in Congenital Cardiology (AARCC), compiled into a survey, and administered to ACHD providers. Patient input was sought via the Adult Congenital Heart Association at community meetings and online forums. The 25 top questions were sent to ACHD providers worldwide via an online survey. Each question was ranked based on perceived priority and weighted based on time spent in ACHD care. The top 10 topics identified are presented and discussed.
  
  
  RESULTS
  The final online survey yielded 139 responses. Top priority questions related to tetralogy of Fallot (timing of pulmonary valve replacement and criteria for primary prevention ICDs), patients with systemic right ventricles (determining the optimal echocardiographic techniques for measuring right ventricular function, and indications for tricuspid valve replacement and primary prevention ICDs), and single ventricle/Fontan patients (role of pulmonary vasodilators, optimal anticoagulation, medical therapy for preservation of ventricular function, treatment for protein losing enteropathy). In addition, establishing criteria to refer ACHD patients for cardiac transplantation was deemed a priority.
  
  
  CONCLUSIONS
  The ACHD field is in need of prospective research to address fundamental clinical questions. It is hoped that this methodical consultation process will inform researchers and funding organizations about clinical research topics deemed to be of high priority.
authors.affiliation: Department of Internal Medicine, University of Michigan Medical School, Ann Arbor, MI, United States; Department of Pediatrics, University of Michigan Medical School, Ann Arbor, MI, United States. Electronic address: cottstim@umich.edu.
authors.forename: Timothy
authors.initials: T
authors.lastname: Cotts
authors.affiliation: Montreal Heart Institute, Université de Montréal, Montreal, Canada.
authors.forename: Paul
authors.initials: P
authors.lastname: Khairy
authors.affiliation: Boston Children's Hospital, Department of Cardiology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, United States; Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, United States.
authors.forename: Alexander R
authors.initials: AR
authors.lastname: Opotowsky
authors.affiliation: Division of Cardiology, Children's National Medical Center, Washington, D.C., United States.
authors.forename: Anitha S
authors.initials: AS
authors.lastname: John
authors.affiliation: Boston Children's Hospital, Department of Cardiology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, United States; Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, United States.
authors.forename: Anne Marie
authors.initials: AM
authors.lastname: Valente
authors.affiliation: Columbus Ohio Adult Congenital Heart Disease Program, The Heart Center, Nationwide Children's Hospital, The Ohio State University, Columbus, OH, United States; Department of Pediatrics, The Ohio State University, Columbus, OH, United States; Department of Internal Medicine, The Ohio State University, Columbus, OH, United States.
authors.forename: Ali N
authors.initials: AN
authors.lastname: Zaidi
authors.affiliation: Department of Pediatrics, Heart Institute, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA, United States.
authors.forename: Stephen C
authors.initials: SC
authors.lastname: Cook
authors.affiliation: Department of Internal Medicine, University of California, Los Angeles, CA, United States.
authors.forename: Jamil
authors.initials: J
authors.lastname: Aboulhosn
authors.affiliation: Heart & Vascular Institute, Hershey Medical Center, Pennsylvania State University, Hershey, PA, United States.
authors.forename: Jennifer Grando
authors.initials: JG
authors.lastname: Ting
authors.affiliation: Boston Children's Hospital, Department of Cardiology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, United States; Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, United States.
authors.forename: Michelle
authors.initials: M
authors.lastname: Gurvitz
authors.affiliation: Boston Children's Hospital, Department of Cardiology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, United States; Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, United States.
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Landzberg
authors.affiliation: Adult Congenital Heart Association, Philadelphia, PA, United States.
authors.forename: Amy
authors.initials: A
authors.lastname: Verstappen
authors.affiliation: Department of Internal Medicine, University of Colorado, Denver, United States; Department of Pediatrics, University of Colorado, Denver, United States.
authors.forename: Joseph
authors.initials: J
authors.lastname: Kay
authors.affiliation: Department of Internal Medicine, Medical College of Wisconsin, Milwaukee, WI, United States; Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI, United States.
authors.forename: Michael
authors.initials: M
authors.lastname: Earing
authors.affiliation: Department of Medicine, Baylor College of Medicine, Texas Children's Hospital, Houston, TX, United States; Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston, TX, United States.
authors.forename: Wayne
authors.initials: W
authors.lastname: Franklin
authors.affiliation: Division of Cardiothoracic Surgery, Emory University, Atlanta, GA, United States.
authors.forename: Brian
authors.initials: B
authors.lastname: Kogon
authors.affiliation: Knight Cardiovascular Institute, Oregon Health & Science University, Portland, OR, United States.
authors.forename: Craig S
authors.initials: CS
authors.lastname: Broberg
mesh_terms: D000328:Adult; D035843:Biomedical Research / Q000379:methods / Q000639:trends*; D003226:Congresses as Topic / Q000639:trends; D003625:Data Collection / Q000379:methods*; D005260:Female; D006330:Heart Defects, Congenital / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy*; D006801:Humans; D008297:Male; D010820:Physicians / Q000639:trends*; D011446:Prospective Studies
keywords: Congenital heart disease; Fontan procedure; Survey; Tetralogy of Fallot; Transposition of the great arteries