Abstract
Background
The implantable cardioverter-defibrillator (ICD) has been proven to improve survival
in adults with congenital heart disease (ACHD), but it is associated with a high rate
of complications. We aimed to quantify the incidence of early (≤ 3 months; ECs) and
late (> 3 months; LCs) complications in ACHD patients implanted with an ICD and to
identify their clinical predictors.
Methods
We retrospectively reviewed 207 patients who had ICD follow-up at Toronto General
Hospital from 1996 to 2019.
Results
The most common diagnoses were tetralogy of Fallot (32.4%), dextro-transposition of
the great arteries (17.9%), and congenital corrected transposition of the great arteries
(13%). No intraprocedural complications were observed. Median follow-up was 3.4 years
(IQR 0.1-23). 24 patients (12%) developed EC (4 hematomas, 20 lead dislodgements).
A total of 56 LCs occurred (46% lead failure, 21% infection, 11% prophylactic lead
extraction, 9% neurologic pain, 9% erosion, 4% other) with an incidence rate of LCs
of 18% per 5 person-years. Anatomic complexity (odds ratio 2.9; P = 0.02) and cardiac resynchronization therapy defibrillator implant (odds ratio 2.5;
P = 0.04) were associated with ECs. Survival rates free from LCs were 92%, 86%, and
65%, respectively, after 1, 5, and 10 years. Presence of legacy leads (hazard ratio
2.9; P = 0.006) and subpulmonary ejection fraction (5% increase, hazard ratio 1.35; P = 0.031) were associated with LCs.
Conclusions
ACHD patients at risk of sudden cardiac death continue to benefit from newer device
technology. However, these patients, particularly those with greater anatomic and
device complexity, remain at increased risk of developing complications over their
lifetime. Given the life expectancy of this population, careful consideration needs
to be given when a device for primary prevention is being contemplated.
Résumé
Contexte
La mise en place d’un défibrillateur cardioverteur implantable (DCI) s’est révélée
efficace pour améliorer la survie des adultes atteints d’une cardiopathie congénitale
(CC), mais ce dispositif est aussi associé à un taux élevé de complications. Nous
avons tenté de quantifier l’incidence des complications précoces (≤ 3 mois; CP) et
tardives (> 3 mois; CT) chez les adultes atteints d’une CC et porteurs d’un DCI, en
plus de déterminer les facteurs cliniques prévisionnels.
Méthodologie
Nous avons examiné rétrospectivement les cas de 207 patients porteurs d’un DCI qui
ont fait l’objet d’un suivi au Toronto General Hospital entre 1996 et 2019.
Résultats
Les diagnostics les plus fréquents étaient la tétralogie de Fallot (32,4 %), la dextrotransposition
des gros vaisseaux (17,9 %) et la transposition congénitalement corrigée des gros
vaisseaux (13 %). Aucune complication survenue durant l’intervention n’a été observée.
La durée médiane du suivi était de 3,4 ans (intervalle interquartile : 0,1 à 23).
Vingt-quatre patients (12 %) ont subi des CP (4 hématomes, 20 déplacements de la sonde).
Au total, 56 CT sont survenues (défaillance de la sonde [46 %], infection [21 %],
extraction prophylactique de la sonde [11 %], douleur neurologique [9 %], érosion
[9 %], autre [4 %]). Leur taux d’incidence des CT s’établissait à 18 % pour 5 années-personnes.
La complexité anatomique (rapport de cotes : 2,9; p = 0,02) et l’implantation d’un défibrillateur de resynchronisation cardiaque (rapport
de cotes : 2,5; p = 0,04) ont été associées à des CP. Les taux de survie sans CT s’établissaient à
92 %, 86 % et 65 % après 1 an, 5 ans et 10 ans, respectivement. La présence d’une
sonde d’une génération antérieure (rapport des risques instantanés : 2,9; p = 0,006) et d’une fraction d’éjection infrapulmonaire (augmentation de 5 %; rapport
des risques instantanés : 1,35; p = 0,031) a été associée à des CT.
Conclusions
Les adultes atteints d’une CC qui sont exposés à un risque de mort subite d’origine
cardiaque continuent de tirer un bienfait des dispositifs technologiques plus récents.
Ces patients, surtout ceux dont les caractéristiques anatomiques et le dispositif
sont plus complexes, courent toujours un risque accru de complications tout au long
de la vie. Étant donné l’espérance de vie de ces patients, il importe d’évaluer soigneusement
la situation lorsqu’on envisage l’implantation d’un dispositif en prévention primaire.
Graphical abstract

Graphical Abstract
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Article info
Publication history
Published online: December 21, 2022
Accepted:
December 13,
2022
Received:
April 27,
2022
Publication stage
In Press Journal Pre-ProofFootnotes
See editorial by Khairy, pages XXX-XX of this issue.
See page 9 for disclosure information.
Identification
Copyright
© 2023 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.
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- Implantable Cardioverter-Defibrillators in Adults With Congenital Heart Disease: Toward the More Global Assessment of HealthspanCanadian Journal of Cardiology
- PreviewSudden cardiac death is thought to be the second most common cause of mortality in the growing population of adults with congenital heart disease (CHD), surpassed only by heart failure–related deaths.1 Sudden cardiac deaths account for up to 25% of all causes of mortality in adults with CHD, with rates that vary widely across the broad spectrum of congenital heart malformations. Risk stratification for sudden cardiac death in view of selecting appropriate candidates for primary-prevention implantable cardioverter-defibrillators (ICDs) is an evolving and imperfect science.
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