Gregory King1, Julian Ayer2, David Celermajer3, Dominica Zentner4, Robert Justo5, Patrick Disney6, Diana Zannino7, Yves d'Udekem8. 1. Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Victoria, Australia; Department of Paediatrics, The University of Melbourne, Melbourne, Victoria, Australia; Heart Research Group, Murdoch Children's Research Institute, Melbourne, Victoria, Australia. 2. Heart Centre for Children, The Children's Hospital at Westmead, Sydney, New South Wales, Australia; Discipline of Paediatrics and Child Health, The University of Sydney, Sydney, New South Wales, Australia. 3. Department of Medicine, The University of Sydney, Sydney, New South Wales, Australia; Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia. 4. Department of Cardiology, The Royal Melbourne Hospital, Melbourne, Victoria, Australia; Royal Melbourne Hospital Clinical School, Faculty of Medicine, Dentistry and Health Sciences, The University of Melbourne, Melbourne, Victoria, Australia. 5. Paediatric Cardiology, Queensland Paediatric Cardiac Service, Lady Cilento Children's Hospital, Brisbane, Queensland, Australia. 6. Department of Cardiology, Royal Adelaide Hospital, Adelaide, South Australia, Australia. 7. Heart Research Group, Murdoch Children's Research Institute, Melbourne, Victoria, Australia. 8. Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Victoria, Australia; Department of Paediatrics, The University of Melbourne, Melbourne, Victoria, Australia; Heart Research Group, Murdoch Children's Research Institute, Melbourne, Victoria, Australia. Electronic address: yves.dudekem@rch.org.au.
Abstract
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.
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.
Authors: Neil D Patel; Patrick M Sullivan; Arash Sabati; Allison Hill; Chelsea Maedler-Kron; Shengmei Zhou; Nick Shillingford; Roberta Williams; Cheryl Takao; Sarah Badran Journal: Pediatr Cardiol Date: 2020-01-24 Impact factor: 1.655
Authors: Jiyong Moon; Li Shen; Donald S Likosky; Vikram Sood; Reilly D Hobbs; Peter Sassalos; Jennifer C Romano; Richard G Ohye; Edward L Bove; Ming-Sing Si Journal: J Am Coll Cardiol Date: 2020-07-28 Impact factor: 24.094
Authors: Stephanie Y Tseng; Saira Siddiqui; Michael V Di Maria; Garick D Hill; Adam M Lubert; Shelby Kutty; Alexander R Opotowsky; Mathias Possner; David L S Morales; James A Quintessenza; Tarek Alsaied Journal: J Am Heart Assoc Date: 2020-05-16 Impact factor: 5.501