title: Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study.
abstract: BACKGROUND
  Ventricular arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot are devastating complications in adult survivors of early surgery, but their prediction remains difficult.
  
  
  METHODS
  We examined surgical, electrocardiographic, and late haemodynamic data, and their relation to clinical arrhythmia and sudden death occurring over 10 years, in a multicentre cohort of patients with repaired tetralogy, who were alive in 1985.
  
  
  RESULTS
  Of 793 patients (mean age at repair 8.2 years [SD 8], mean time from repair 21.1 years [8.7]) who entered the study, 33 patients developed sustained monomorphic ventricular tachycardia, 16 died suddenly, and 29 had new-onset sustained atrial flutter or fibrillation. Electrocardiographic markers (QRS duration, QRS rate of change between 1985 and 1995) were significantly greater in the ventricular tachycardia and sudden-death groups. Older age at repair was associated with a higher risk of sudden death and atrial tachyarrhythmia. Pulmonary regurgitation was the main underlying haemodynamic lesion for patients with ventricular tachycardia and sudden death, whereas tricuspid regurgitation was for those with atrial flutter/fibrillation. Despite adverse haemodynamics, no patient who died suddenly had undergone late reoperation.
  
  
  CONCLUSIONS
  Arrhythmia and sudden death are important late sequelae for patients after repair of tetralogy of Fallot. The electrophysiological and haemodynamic substrate of sudden death resembled that of sustained ventricular tachycardia, with pulmonary regurgitation being the predominant haemodynamic lesion. Preservation or restoration of pulmonary valve function may thus reduce the risk of sudden death. Furthermore, electrocardiographic markers can help to identify patients at risk.
authors.affiliation: Department of Paediatrics, Royal Brompton Hospital and the National Heart and Lung Institute, London, UK. m.gatzoulis@rbh.nthames.nhs.uk
authors.forename: M A
authors.initials: MA
authors.lastname: Gatzoulis
authors.forename: S
authors.initials: S
authors.lastname: Balaji
authors.forename: S A
authors.initials: SA
authors.lastname: Webber
authors.forename: S C
authors.initials: SC
authors.lastname: Siu
authors.forename: J S
authors.initials: JS
authors.lastname: Hokanson
authors.forename: C
authors.initials: C
authors.lastname: Poile
authors.forename: M
authors.initials: M
authors.lastname: Rosenthal
authors.forename: M
authors.initials: M
authors.lastname: Nakazawa
authors.forename: J H
authors.initials: JH
authors.lastname: Moller
authors.forename: P C
authors.initials: PC
authors.lastname: Gillette
authors.forename: G D
authors.initials: GD
authors.lastname: Webb
authors.forename: A N
authors.initials: AN
authors.lastname: Redington
mesh_terms: D000328:Adult; D001282:Atrial Flutter / Q000209:etiology* / Q000401:mortality; D002648:Child; D015331:Cohort Studies; D016757:Death, Sudden, Cardiac / Q000209:etiology*; D004562:Electrocardiography; D015716:Electrocardiography, Ambulatory; D005260:Female; D006439:Hemodynamics; D006753:Hospital Records; D006801:Humans; D008297:Male; D015999:Multivariate Analysis; D011183:Postoperative Complications; D012307:Risk Factors; D015996:Survival Rate; D017180:Tachycardia, Ventricular / Q000209:etiology* / Q000401:mortality; D013771:Tetralogy of Fallot / Q000601:surgery*; D013997:Time Factors