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Implantable cardioverter-defibrillators in congenital heart disease: 10 programming tips

Published:November 08, 2010DOI:https://doi.org/10.1016/j.hrthm.2010.10.046
      Advances in cardiac care of the young have given rise to a growing and aging population of patients with congenital heart disease. Despite remarkable improvements in overall survival, sudden cardiac death remains the most common cause of late mortality. As a result, implantable cardioverter-defibrillators (ICDs) are increasingly used in this heterogeneous patient population. Tetralogy of Fallot and transposition of the great arteries are the most prevalent subtypes of congenital heart disease in ICD recipients.
      • Khairy P.
      • Harris L.
      • Landzberg M.J.
      • et al.
      Implantable cardioverter-defibrillators in tetralogy of Fallot.
      • Berul C.I.
      • Van Hare G.F.
      • Kertesz N.J.
      • et al.
      Results of a multicenter retrospective implantable cardioverter-defibrillator registry of pediatric and congenital heart disease patients.
      • Khairy P.
      • Harris L.
      • Landzberg M.J.
      • et al.
      Sudden death and defibrillators in transposition of the great arteries with intra-atrial baffles: a multicenter study.
      Common to this young population is the high rate of lead-related complications and inappropriate shocks.
      • Khairy P.
      • Harris L.
      • Landzberg M.J.
      • et al.
      Implantable cardioverter-defibrillators in tetralogy of Fallot.
      • Berul C.I.
      • Van Hare G.F.
      • Kertesz N.J.
      • et al.
      Results of a multicenter retrospective implantable cardioverter-defibrillator registry of pediatric and congenital heart disease patients.
      • Khairy P.
      • Harris L.
      • Landzberg M.J.
      • et al.
      Sudden death and defibrillators in transposition of the great arteries with intra-atrial baffles: a multicenter study.
      In a multicenter study of patients with tetralogy of Fallot, 25% received inappropriate shocks, predominantly due to sinus or supraventricular tachycardia.
      • Khairy P.
      • Harris L.
      • Landzberg M.J.
      • et al.
      Implantable cardioverter-defibrillators in tetralogy of Fallot.
      Although a similar proportion of patients with transposition received inappropriate shocks (24%), 62% were due to oversensing or lead dysfunction.
      • Khairy P.
      • Harris L.
      • Landzberg M.J.
      • et al.
      Sudden death and defibrillators in transposition of the great arteries with intra-atrial baffles: a multicenter study.
      Herein, we offer 10 practical tips for optimizing ICD programming in patients with congenital heart disease (Table 1) in view of reducing inappropriate and potentially avoidable shocks.
      Table 1ICD programming tips for patients with congenital heart disease
      Programming tip Comment
      Program faster VT detection rate For example, 200 bpm for primary prevention; 30–60 ms slower than clinical or induced VT for secondary prevention
      Program monitoring zone Should not interact with active zones in modern ICDs
      Program longer detection times For example, 24 of 32 or 30 of 40 intervals
      Program fast VT zone Allows use of ATP
      Program discriminators For rates up to 200 bpm; “off” if complete AV block
      Deactivate rate smoothing Except for VT triggered by short–long–short sequences
      Deactivate “safety” timers “High rate time out” and “sustained rate duration”
      Program ATP in VF zone If programmable, two sequences are superior to one
      Optimize number of ATP sequences For example, at least 2 in faster VT zone, 4–6 in slower VT zone; burst not ramp
      Program maximum shock output Recommended pending further data
      ATP = antitachycardia pacing; ICD = implantable cardioverter-defibrillator; VF = ventricular fibrillation; VT = ventricular tachycardia.

      Keywords

      Abbreviations:

      ATP (antitachycardia pacing), ICD (implantable cardioverter-defibrillator), SVT (supraventricular tachycardia), VF (ventricular fibrillation), VT (ventricular tachycardia)
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