Author + information
- Received July 5, 2018
- Revision received November 29, 2018
- Accepted December 10, 2018
- Published online February 18, 2019.
- Gregory King, MDa,b,c,
- Julian Ayer, MBBS, PhDd,e,
- David Celermajer, MBBS, PhD, DScf,g,
- Dominica Zentner, MBBS, PhDh,i,
- Robert Justo, MBBSj,
- Patrick Disney, MBBSk,
- Diana Zannino, MScc and
- Yves d’Udekem, MD, PhDa,b,c,∗ (, )@dudekem_yves
- aDepartment of Cardiac Surgery, Royal Children’s Hospital, Melbourne, Victoria, Australia
- bDepartment of Paediatrics, The University of Melbourne, Melbourne, Victoria, Australia
- cHeart Research Group, Murdoch Children’s Research Institute, Melbourne, Victoria, Australia
- dHeart Centre for Children, The Children's Hospital at Westmead, Sydney, New South Wales, Australia
- eDiscipline of Paediatrics and Child Health, The University of Sydney, Sydney, New South Wales, Australia
- fDepartment of Medicine, The University of Sydney, Sydney, New South Wales, Australia
- gDepartment of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia
- hDepartment of Cardiology, The Royal Melbourne Hospital, Melbourne, Victoria, Australia
- iRoyal Melbourne Hospital Clinical School, Faculty of Medicine, Dentistry and Health Sciences, The University of Melbourne, Melbourne, Victoria, Australia
- jPaediatric Cardiology, Queensland Paediatric Cardiac Service, Lady Cilento Children’s Hospital, Brisbane, Queensland, Australia
- kDepartment of Cardiology, Royal Adelaide Hospital, Adelaide, South Australia, Australia
- ↵∗Address for correspondence:
Dr. Yves d’Udekem, Cardiac Surgery Unit, Royal Children’s Hospital, Flemington Road, Parkville, VIC 3052, Australia.
Background Atrioventricular valve failure (moderate or greater regurgitation, or valve operation) is a risk factor for adverse outcomes in patients undergoing Fontan palliation.
Objectives This study determined the incidence of atrioventricular valve failure and its clinical impact on patients undergoing Fontan palliation.
Methods A retrospective cohort longitudinal study was conducted using patient data extracted from an existing bi-national, population-based registry.
Results A total of 1,468 patients who underwent Fontan palliation were identified; complete follow-up data were available for 1,199 patients. Six hundred eighty-six patients had 2 atrioventricular valves, 286 had a single mitral valve, 130 had a common atrioventricular valve, and 97 had a single tricuspid valve. A total of 132 repairs were performed in 110 patients, and 15 replacements were performed in 13 patients. The cumulative incidence of atrioventricular valve failure at 25 years of age for patients with a common atrioventricular, single tricuspid, single mitral, and 2 atrioventricular valves was 56% (95% confidence interval [CI]: 46% to 67%), 46% (95% CI: 31% to 61%), 8% (95% CI: 4% to 12%), and 26% (95% CI: 21% to 30%), respectively. In patients without valve failure, freedom from Fontan failure at 10 and 20 years post-Fontan palliation was 91% (95% CI: 89% to 93%) and 77% (95% CI: 73% to 81%), respectively, compared with 77% (95% CI: 69% to 85%) and 54% (95% CI: 42% to 68%), respectively, in patients with valve failure (hazard ratio: 2.43; 95% CI: 1.74 to 3.39; p < 0.001).
Conclusions Atrioventricular valve failure occurs frequently in patients undergoing Fontan palliation. Patients with valve failure are twice as likely to have their Fontan circulation fail than those without valve failure.
This work was supported by a National Health and Medical Research Council (NHMRC) Partnership grant (1076849). Dr. King is supported by an Avant Doctor in Training Research Scholarship. Dr. d’Udekem has been a consultant for Merck Sharp and Dohme and Actelion. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.
Listen to this manuscript's audio summary by Editor-in-Chief Dr. Valentin Fuster on JACC.org.
- Received July 5, 2018.
- Revision received November 29, 2018.
- Accepted December 10, 2018.
- 2019 American College of Cardiology Foundation
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