title: Common atrioventricular valve failure during single ventricle palliation†.
abstract: OBJECTIVE
  To determine the risk of atrioventricular valve failure (valve intervention or moderate or greater regurgitation) during the lifetime of patients with single ventricle physiology and common atrioventricular valve.
  
  
  METHODS
  Patients' data were extracted from an existing bi-national, population based registry. A retrospective review of their medical records was undertaken to determine the incidence of atrioventricular valve repair/replacement or moderate or greater regurgitation.
  
  
  RESULTS
  From a registry of 1468 Fontan survivors, 136 patients with common atrioventricular valve were identified. Complete echocardiographic follow-up was available for 114 patients. Median length of follow-up was 10.2 years (interquartile range 5-15 years). Twenty-five year survival and freedom from Fontan failure were 94% [95% confidence interval (CI), 88-100%] and 74% (95% CI, 64-87%), respectively. Twenty-eight patients underwent 24 initial repairs and 4 replacements. The 24 patients undergoing repair subsequently needed 6 re-repairs, 2 replacements and 8 had moderate or greater regurgitation at last follow-up. Four-year freedom from atrioventricular valve repair failure was 50% (95% CI, 34-75%). An additional 30 patients developed moderate or greater atrioventricular valve regurgitation (6 New York Heart Association ≥3, 10 Fontan failures, 0 deaths). Cumulative incidence of the composite endpoint of atrioventricular valve failure at 28 years was 62% (95% CI, 49-74%).
  
  
  CONCLUSIONS
  Patients with single ventricle physiology and common atrioventricular valve experience a continuous decline in valve function. The majority of patients experience valve failure in the first 30 years of life.
authors.affiliation: Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia.
authors.forename: Gregory
authors.initials: G
authors.lastname: King
authors.affiliation: Green Lane Paediatric and Congenital Cardiac Service, Starship Children's Hospital, Auckland, New Zealand.
authors.forename: Thomas L
authors.initials: TL
authors.lastname: Gentles
authors.affiliation: Heart Centre for Children, The Children's Hospital at Westmead, Sydney, Australia.
authors.forename: David S
authors.initials: DS
authors.lastname: Winlaw
authors.affiliation: Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia.
authors.forename: Rachel
authors.initials: R
authors.lastname: Cordina
authors.affiliation: Department of Cardiology, Princess Margaret Hospital for Children, Perth, Australia.
authors.forename: Andrew
authors.initials: A
authors.lastname: Bullock
authors.affiliation: Department of Cardiology, Royal Melbourne Hospital, Melbourne, Australia.
authors.forename: Leeanne E
authors.initials: LE
authors.lastname: Grigg
authors.affiliation: Queensland Paediatric Cardiac Services, Lady Cilento Children's Hospital, Brisbane, Australia.
authors.forename: Nelson
authors.initials: N
authors.lastname: Alphonso
authors.affiliation: Adult Congenital Heart Unit, The Prince Charles Hospital, Brisbane, Australia.
authors.forename: Dorothy J
authors.initials: DJ
authors.lastname: Radford
authors.affiliation: Heart Research, Murdoch Childrens Research Institute, Melbourne, Australia.
authors.forename: Diana
authors.initials: D
authors.lastname: Zannino
authors.affiliation: Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia.|Heart Research, Murdoch Childrens Research Institute, Melbourne, Australia.|Department of Paediatrics, Faculty of Medicine, The University of Melbourne, Melbourne, Australia.
authors.forename: Edward
authors.initials: E
authors.lastname: Buratto
authors.affiliation: Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia.|Heart Research, Murdoch Childrens Research Institute, Melbourne, Australia.|Department of Paediatrics, Faculty of Medicine, The University of Melbourne, Melbourne, Australia.
authors.forename: Yves
authors.initials: Y
authors.lastname: d'Udekem
mesh_terms: D002648:Child; D002675:Child, Preschool; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects / Q000401:mortality / Q000706:statistics & numerical data; D006343:Heart Septal Defects / Q000453:epidemiology / Q000401:mortality / Q000601:surgery; D006349:Heart Valve Diseases / Q000401:mortality; D006352:Heart Ventricles / Q000503:physiopathology / Q000601:surgery; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D010166:Palliative Care / Q000706:statistics & numerical data; D012189:Retrospective Studies
keywords: Common atrioventricular valve; Fontan; Regurgitation; Single ventricle; Unbalanced atrioventricular septal defect; Valve repair