title: Sudden cardiac death and late arrhythmias after the Fontan operation.
abstract: OBJECTIVE
  We sought to examine the incidence and predictors of arrhythmias and sudden cardiac death (SCD) after Fontan operation.
  
  
  BACKGROUND
  Arrhythmias and SCD have been reported following operations for congenital heart disease, but the incidence and risk factors have not been well defined in patients after a Fontan operation.
  
  
  METHODS
  We reviewed records of all patients who had a Fontan operation from 1973 to 2012 (n = 1052) at our institution. A questionnaire was mailed to patients who were not known to be deceased at the initiation of the study. Late arrhythmias were classified as bradyarrhythmias or tachyarrhythmias requiring treatment >30 days after operation.
  
  
  RESULTS
  We included 996/1052 (95%) patients with no arrhythmia diagnosis prior to Fontan. Overall 10-, 20-, and 30-year freedom from arrhythmias was 71%, 42%, and 24%, respectively. Of 864 patients who survived >30 days after Fontan, 304 (35%) had atrial flutter, 161 (19%) had atrial fibrillation, 108 (13%) had atrial tachycardia, 37 (4%) had reentrant supraventricular tachycardia, 40 (5%) had ventricular tachycardia, and 113 (13%) had sinus node dysfunction. Predictors of late arrhythmias included an atriopulmonary Fontan, age at operation (>16 years) or atrial arrhythmias postoperatively. During follow-up, 52/1052 (5%) patients had SCD, with 51 having documentation available; 8 patients died suddenly within 30 days and the remaining 43 had an average time to SCD of 6.9 ± 6.7 years (median was 3.8 years). Arrhythmias were documented in 28/43 (65%) patients prior to SCD. Predictors of SCD included atrioventricular valve replacement and post-bypass Fontan pressures >20 mm Hg; preoperative sinus rhythm was protective.
  
  
  CONCLUSIONS
  Arrhythmias and SCD are significant concerns among Fontan patients and specific risk factors may warrant closer follow-up and earlier consideration for therapy.
authors.affiliation: Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota.
authors.forename: Kavitha N
authors.initials: KN
authors.lastname: Pundi
authors.affiliation: Mayo Clinic College of Medicine, Mayo Clinic, Rochester, Minnesota.
authors.forename: Krishna N
authors.initials: KN
authors.lastname: Pundi
authors.affiliation: Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota.|Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota.
authors.forename: Jonathan N
authors.initials: JN
authors.lastname: Johnson
authors.affiliation: Division of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota.
authors.forename: Joseph A
authors.initials: JA
authors.lastname: Dearani
authors.affiliation: Division of Biomedical Statistics and Informatics, Mayo Clinic, Rochester, Minnesota.
authors.forename: Zhuo
authors.initials: Z
authors.lastname: Li
authors.affiliation: Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota.
authors.forename: David J
authors.initials: DJ
authors.lastname: Driscoll
authors.affiliation: Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota.
authors.forename: Philip L
authors.initials: PL
authors.lastname: Wackel
authors.affiliation: Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota.
authors.forename: Christopher J
authors.initials: CJ
authors.lastname: McLeod
authors.affiliation: Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota.|Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota.
authors.forename: Frank
authors.initials: F
authors.lastname: Cetta
authors.affiliation: Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota.|Division of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota.
authors.forename: Bryan C
authors.initials: BC
authors.lastname: Cannon
mesh_terms: D000293:Adolescent; D000328:Adult; D001919:Bradycardia / Q000453:epidemiology* / Q000401:mortality / Q000503:physiopathology / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D016757:Death, Sudden, Cardiac / Q000453:epidemiology*; D018572:Disease-Free Survival; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects* / Q000401:mortality; D006330:Heart Defects, Congenital / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D015994:Incidence; D007223:Infant; D053208:Kaplan-Meier Estimate; D008297:Male; D008499:Medical Records; D008910:Minnesota / Q000453:epidemiology; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D012307:Risk Factors; D011795:Surveys and Questionnaires; D017741:Survivors; D013610:Tachycardia / Q000453:epidemiology* / Q000401:mortality / Q000503:physiopathology / Q000628:therapy; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
keywords: Fontan; ICD; arrhythmias; pacemaker; sudden cardiac death