Open Access

REVIEW

Frontiers in Fontan failure: Innovation and improving outcomes: A conference summary

Georges Ephrem¹, Camden Hebson², Anitha John³, Estella Moore⁴, Maan Jokhadar⁴, Ryan Ford⁵, Gruschen Veldtman⁶, Yoav Dori⁷, Michelle Gurvitz^8,9, Brian Kogon¹⁰, Adrienne Kovacs¹¹, Meghan Roswick, Michael McConnell¹², Wendy M. Book⁴, Fred Rodriguez III^4,12

1 Peter Munk Cardiac Centre, University Health Network, Toronto, Canada
2 Division of Cardiology, Department of Pediatrics, Children’s of Alabama, Birmingham, Alabama
3 Division of Pediatric Cardiology, Children’s National Health System, Washington, District of Columbia
4 Division of Cardiology, Department of Medicine, Emory University School of Medicine, Atlanta, Georgia
5 Division of Gastroenterology, Department of Medicine, Emory University School of Medicine, Atlanta, Georgia
6 Department of Cardiology, The Heart Institute, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio
7 Division of Cardiology, Department of Pediatrics, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania
8 Boston Children’s Hospital, Boston, Massachusetts
9 Brigham and Women’s Hospital, Boston, Massachusetts
10 Division of Pediatric Cardiothoracic Surgery, University of Mississippi Medical Center, Jackson, Mississippi
11 Knight Cardiovascular Institute, Oregon Health & Science University, Portland, Oregon
12 Sibley Heart Center Cardiology, Emory University School of Medicine, Atlanta, Georgia

* Corresponding Author: Georges Ephrem, MD, MSc, Division of Cardiology, Department of Medicine, University of Toronto, 519‐585 University Avenue, Toronto, ON M5G 2N2, Canada. Email:

Congenital Heart Disease 2019, 14(2), 128-137. https://doi.org/10.1111/chd.12685

Abstract

The initial “Frontiers in Fontan Failure” conference in 2015 in Atlanta, Georgia, provided an opportunity for experts in the field of pediatric cardiology and adult congenital heart disease to focus on the etiology, physiology, and potential interventions for patients with “Failing Fontan” physiology. Four types of “Fontan Failure” were described and then published by Dr Book et al. The acknowledgment that even Dr Fontan himself realized that the Fontan proce‐ dure “imposed a gradually declining functional capacity and premature late death after an initial period of often excellent palliation.” The purpose of the second “Frontiers in Fontan Failure” was to further the discussion regarding new data and technologies as well as novel interven‐ tions. The 2017 “Frontiers in Fontan Failure: Innovation and Improving Outcomes” was spon‐ sored by Children’s Healthcare of Atlanta, Sibley Heart Center Cardiology, and Emory University School of Medicine. Future directions in the management of Fontan failure include further investigations into the risk of sudden cardiac death and how to properly prevent it, achievable interventions in modifying the Fontan physiology to treat or prevent late complica‐ tions, and improved and refined algorithms in Fontan surveillance. Finally, further research into the interventional treatment of lymphatic‐related complications hold the promise of marked improvement in the quality of life of advanced Fontan failure patients and as such should be encouraged and contributed to.

---

Keywords

---