title: Intermediate-Term Outcome of 140 Consecutive Fontan Conversions With Arrhythmia Operations.
abstract: BACKGROUND
  Atrial arrhythmias and progressive circulatory failure frequently develop in patients with a Fontan circulation. Improvement of flow dynamics and revision of the arrhythmia substrate may improve outcomes in selected patients. We sought to determine intermediate-term outcomes after Fontan conversion with arrhythmia operations and identify characteristics associated with decreased transplant-free survival.
  
  
  METHODS
  The first 140 Fontan conversions with arrhythmia operations at a single institution were analyzed for predictors of cardiac death or transplant and incidence of arrhythmia recurrence.
  
  
  RESULTS
  The median age at the Fontan conversion operation was 23.2 years (range, 2.6 to 47.3 years). Preoperative arrhythmias were present in 136 patients: right atrial tachycardia in 48 patients, left atrial tachycardia in 21, and atrial fibrillation in 67. Freedom from cardiac death or transplant was 90% at 5 years, 84% at 10 years, and 66% at 15 years. The median age at the last follow-up among survivors was 32 years (range, 15 to 61 years). By multivariable analysis, risk factors for cardiac death or heart transplantation were a right or indeterminate ventricular morphology, cardiopulmonary bypass time exceeding 240 minutes, ascites, protein-losing enteropathy, or a biatrial arrhythmia operation at the time of conversion. Freedom from recurrence of atrial tachycardia was 77% at 10 years. Among 67 patients with atrial fibrillation undergoing biatrial arrhythmia operations, none had recurrent atrial fibrillation.
  
  
  CONCLUSIONS
  Freedom from cardiac death or transplant for patients undergoing Fontan conversion with an arrhythmia operation is 84% at 10 years. The effects of atrial arrhythmia operations are durable in most patients. These outcomes may serve as useful benchmarks for alternative management strategies.
authors.affiliation: Divisions of Cardiology and Cardiovascular-Thoracic Surgery, Departments of Pediatrics and Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois. Electronic address: bdeal@luriechildrens.org.
authors.forename: Barbara J
authors.initials: BJ
authors.lastname: Deal
authors.affiliation: Divisions of Cardiology and Cardiovascular-Thoracic Surgery, Departments of Pediatrics and Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
authors.forename: John M
authors.initials: JM
authors.lastname: Costello
authors.affiliation: Divisions of Cardiology and Cardiovascular-Thoracic Surgery, Departments of Pediatrics and Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
authors.forename: Gregory
authors.initials: G
authors.lastname: Webster
authors.affiliation: Divisions of Cardiology and Cardiovascular-Thoracic Surgery, Departments of Pediatrics and Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
authors.forename: Sabrina
authors.initials: S
authors.lastname: Tsao
authors.affiliation: Divisions of Cardiology and Cardiovascular-Thoracic Surgery, Departments of Pediatrics and Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
authors.forename: Carl L
authors.initials: CL
authors.lastname: Backer
authors.affiliation: Congenital Heart Institute, Florida Hospital for Children, Orlando, Florida.
authors.forename: Constantine
authors.initials: C
authors.lastname: Mavroudis
mesh_terms: D000293:Adolescent; D000328:Adult; D001281:Atrial Fibrillation / Q000401:mortality / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D005260:Female; D018729:Fontan Procedure; D006801:Humans; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D012189:Retrospective Studies; D015996:Survival Rate; D013610:Tachycardia / Q000401:mortality / Q000601:surgery*; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult