title: Surgical Intervention for Anomalous Origin of Left Coronary Artery From the Pulmonary Artery in Children: A Long-Term Follow-Up.
abstract: BACKGROUND
  Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect with limited data on long-term outcomes after surgical intervention.
  
  
  METHODS
  We conducted a retrospective review of all children (N = 42) who underwent surgical repair of ALCAPA between 1980 and 2014 at the Royal Children's Hospital, Melbourne.
  
  
  RESULTS
  Twenty-nine (69% [29 of 42]) patients underwent coronary reimplantation, 12 (29% [12 of 42]) had intrapulmonary baffle (Takeuchi) repair, and 1 (2% [1 of 42]) patient had ligation of the anomalous coronary artery. Nine (21%, 9 of 42) patients had concomitant mitral valve (MV) repair at the time of ALCAPA repair. A left ventricular assist device (LVAD) was used in 36% (15 of 42) of patients. Early mortality was 2.4% (1 of 42 patients). Median follow-up was 14 years (mean, 13 years; range, 4 months-31 years). There were no late deaths. Survival was 98% at 20 years. Freedom from reoperation was 81%, 81%, and 76% at 5, 10, and 20 years after operation, respectively. Eight patients underwent late MV repair or replacement at a median of 3 years (mean, 8 years; range, 2 months-25 years) after operation. Freedom from late MV repair or replacement was 86% at 5 and 10 years and 81% at 20 years after operation. Eleven (26% [11 of 42]) patients had severe mitral regurgitation (MR) preoperatively. Of those 11 patients, 5 (45% [5 of 11]) had concomitant MV repair at the time of ALCAPA repair, 3 (27% [3 of 11]) had late MV repair or replacement, and the remaining 3 (27% [3 of 11]) patients had mild MR at last follow-up. Thirty-six (90% [36 of 41]) patients had normal left ventricular function and 4 (10% [4 of 41]) patients had mildly reduced left ventricular (LV) function at last follow-up.
  
  
  CONCLUSIONS
  ALCAPA can be operated on with good outcomes. Persistent MR and a moderate rate of late MV repair warrants close follow-up.
authors.affiliation: Department of Cardiothoracic Surgery, Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia.
authors.forename: Phillip S
authors.initials: PS
authors.lastname: Naimo
authors.affiliation: Department of Cardiothoracic Surgery, Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia.
authors.forename: Tyson A
authors.initials: TA
authors.lastname: Fricke
authors.affiliation: Department of Cardiothoracic Surgery, Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia.
authors.forename: Yves
authors.initials: Y
authors.lastname: d'Udekem
authors.affiliation: Department of Cardiothoracic Surgery, Monash Medical Centre, Melbourne, Australia.
authors.forename: Andrew D
authors.initials: AD
authors.lastname: Cochrane
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, Women's and Children's Hospital, Adelaide, Australia.
authors.forename: Terry
authors.initials: T
authors.lastname: Robertson
authors.affiliation: Department of Cardiothoracic Surgery, Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia.
authors.forename: Christian P
authors.initials: CP
authors.lastname: Brizard
authors.affiliation: Department of Cardiothoracic Surgery, Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia. Electronic address: igor.konstantinov@rch.org.au.
authors.forename: Igor E
authors.initials: IE
authors.lastname: Konstantinov
mesh_terms: D002675:Child, Preschool; D003330:Coronary Vessel Anomalies / Q000150:complications / Q000601:surgery*; D003331:Coronary Vessels / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D019918:Heart Valve Prosthesis Implantation; D006353:Heart-Assist Devices; D017052:Hospital Mortality; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D008026:Ligation; D008297:Male; D008943:Mitral Valve / Q000601:surgery; D008944:Mitral Valve Insufficiency / Q000209:etiology / Q000601:surgery; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000601:surgery*; D012086:Reoperation / Q000706:statistics & numerical data; D012092:Replantation; D012189:Retrospective Studies; D013524:Surgical Flaps; D015996:Survival Rate; D016896:Treatment Outcome; D016277:Ventricular Function, Left