title: Pacing in congenital heart disease - A four-decade experience in a single tertiary centre.
abstract: BACKGROUND
  The increased risk of brady- and tachy-arrhythmias in the congenital heart disease (CHD) population means that cardiac rhythm management devices are often required at an early age and expose patients to device-related complications. The present study drew upon four decades of experience at a tertiary adult congenital heart disease ACHD center and aimed to investigate the indication for cardiac implantable electronic devices (CIEDs) and predictors of late device-related complication requiring re-intervention.
  
  
  METHODS
  A retrospective review of pacing records of ACHD patients over forty years was carried out. The primary outcome measure was device related complication requiring re-intervention.
  
  
  RESULTS
  Between 1970 and 2009, 238 structural CHD patients who received CIEDs with follow-up data were identified (structural group). As a comparator group, 98 patients with congenital conduction disease or long QT syndrome with a structurally normal heart (electrical group) were included in the study. During a mean follow-up of 9.6±8.5years, 72 (21%) patients (44 structural group, 28 electrical group) required ≥1 re-intervention due to device related complications. Multivariate analysis showed that age at the time of device implant was an independent predictor of late device-related complications (HR 0.77, 95% CI 0.60-0.98, p=0.04). Sub-analysis of the structural group showed that ACHD complexity (Bethesda guideline) was the only predictor late device-related complication in the structural group (HR 2.96, 95% CI: 1.67-5.26, p<0.01).
  
  
  CONCLUSIONS
  Increasing age at device implant was inversely associated with late device-related complications. ACHD patients with complex anatomy are at increased risk of device-related complications at mid and long-term follow-up.
authors.affiliation: Heart Rhythm Centre, Royal Brompton and Harefield NHS Foundation Trust, NIHR Cardiovascular Biomedical Research Unit, Imperial College, London, United Kingdom.
authors.forename: Disha
authors.initials: D
authors.lastname: Midha
authors.affiliation: Heart Rhythm Centre, Royal Brompton and Harefield NHS Foundation Trust, NIHR Cardiovascular Biomedical Research Unit, Imperial College, London, United Kingdom.
authors.forename: Zhong
authors.initials: Z
authors.lastname: Chen
authors.affiliation: Heart Rhythm Centre, Royal Brompton and Harefield NHS Foundation Trust, NIHR Cardiovascular Biomedical Research Unit, Imperial College, London, United Kingdom.
authors.forename: David G
authors.initials: DG
authors.lastname: Jones
authors.affiliation: Heart Rhythm Centre, Royal Brompton and Harefield NHS Foundation Trust, NIHR Cardiovascular Biomedical Research Unit, Imperial College, London, United Kingdom.
authors.forename: Howell J
authors.initials: HJ
authors.lastname: Williams
authors.affiliation: Heart Rhythm Centre, Royal Brompton and Harefield NHS Foundation Trust, NIHR Cardiovascular Biomedical Research Unit, Imperial College, London, United Kingdom.
authors.forename: Karen
authors.initials: K
authors.lastname: Lascelles
authors.affiliation: Heart Rhythm Centre, Royal Brompton and Harefield NHS Foundation Trust, NIHR Cardiovascular Biomedical Research Unit, Imperial College, London, United Kingdom.
authors.forename: Julian
authors.initials: J
authors.lastname: Jarman
authors.affiliation: Heart Rhythm Centre, Royal Brompton and Harefield NHS Foundation Trust, NIHR Cardiovascular Biomedical Research Unit, Imperial College, London, United Kingdom.
authors.forename: Jonathan
authors.initials: J
authors.lastname: Clague
authors.affiliation: Heart Rhythm Centre, Royal Brompton and Harefield NHS Foundation Trust, NIHR Cardiovascular Biomedical Research Unit, Imperial College, London, United Kingdom.
authors.forename: Janice
authors.initials: J
authors.lastname: Till
authors.affiliation: Heart Rhythm Centre, Royal Brompton and Harefield NHS Foundation Trust, NIHR Cardiovascular Biomedical Research Unit, Imperial College, London, United Kingdom.
authors.forename: Konstatinos
authors.initials: K
authors.lastname: Dimopoulos
authors.affiliation: Heart Rhythm Centre, Royal Brompton and Harefield NHS Foundation Trust, NIHR Cardiovascular Biomedical Research Unit, Imperial College, London, United Kingdom.
authors.forename: Sonya V
authors.initials: SV
authors.lastname: Babu-Narayan
authors.affiliation: Heart Rhythm Centre, Royal Brompton and Harefield NHS Foundation Trust, NIHR Cardiovascular Biomedical Research Unit, Imperial College, London, United Kingdom.
authors.forename: Vias
authors.initials: V
authors.lastname: Markides
authors.affiliation: Heart Rhythm Centre, Royal Brompton and Harefield NHS Foundation Trust, NIHR Cardiovascular Biomedical Research Unit, Imperial College, London, United Kingdom.
authors.forename: Michael A
authors.initials: MA
authors.lastname: Gatzoulis
authors.affiliation: Heart Rhythm Centre, Royal Brompton and Harefield NHS Foundation Trust, NIHR Cardiovascular Biomedical Research Unit, Imperial College, London, United Kingdom. Electronic address: tom.wong@imperial.ac.uk.
authors.forename: Tom
authors.initials: T
authors.lastname: Wong
mesh_terms: D000293:Adolescent; D000328:Adult; D002304:Cardiac Pacing, Artificial / Q000009:adverse effects / Q000639:trends*; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000503:physiopathology* / Q000628:therapy*; D006801:Humans; D008297:Male; D010138:Pacemaker, Artificial / Q000009:adverse effects / Q000639:trends*; D012189:Retrospective Studies; D062606:Tertiary Care Centers / Q000639:trends*; D013997:Time Factors; D055815:Young Adult
keywords: Adult congenital heart disease; Complication; Pacing