Clinical research priorities in adult congenital heart disease

Timothy Cotts, Paul Khairy, Alexander R. Opotowsky, Anitha S. John, Anne Marie Valente, Ali N. Zaidi, Stephen C. Cook, Jamil Aboulhosn, Jennifer Grando Ting, Michelle Gurvitz, Michael J. Landzberg, Amy Verstappen, Joseph Kay, Michael Earing, Wayne Franklin, Brian Kogon, Craig S. Broberg

Research output: Contribution to journalArticlepeer-review

31 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)351-360
Number of pages10
JournalInternational Journal of Cardiology
Issue number3
StatePublished - Feb 15 2014


  • Congenital heart disease
  • Fontan procedure
  • Survey
  • Tetralogy of Fallot
  • Transposition of the great arteries

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine


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