Abstract
The expanding population of adults with congenital heart disease (CHD) combined with
the pervasiveness of arrhythmias has resulted in the rapid growth of a dedicated sector
of cardiology at the intersection between 2 subspecialties: electrophysiology and
adult CHD. Herein, practical considerations are offered regarding urgent referral
for catheter ablation of atrial arrhythmias, anticoagulation, and primary prevention
implantable cardioverter-defibrillators (ICDs). Patients with Ebstein anomaly and
ventricular pre-excitation should be referred promptly due to the high prevalence
of multiple accessory pathways and increased incidence of atrial tachyarrhythmias,
which may be poorly tolerated. In patients with transposition of the great arteries
and atrial switch surgery, atrial arrhythmias should be managed without delay because
they could provoke ventricular arrhythmias and sudden death. Other settings in which
atrial arrhythmias can be poorly tolerated include single ventricle physiology and
Eisenmenger syndrome. Long-term anticoagulation is generally indicated in patients
with sustained intra-atrial reentrant tachycardia or atrial fibrillation and a mechanical
valve, moderate or severe systemic atrioventricular valve stenosis, traditional risk
factors for stroke, and/or moderate or complex CHD. The only class I indication for
a primary prevention ICD is a systemic left ventricular ejection fraction ≤ 35%, with
biventricular physiology, and New York Heart Association class II or III symptoms.
ICD therapy is reasonable in selected adults with tetralogy of Fallot and multiple
risk factors for sudden death identified by observational studies. Indications for
ICDs in patients with systemic right ventricles and univentricular hearts are less
well established, underscoring the need for future research to inform risk stratification.
Résumé
L’augmentation de la population d’adultes présentant une cardiopathie congénitale
jumelée à l’omniprésence des troubles d’arythmie a entraîné la croissance rapide d’un
domaine spécialisé de la cardiologie, qui recoupe deux sous-spécialités : l’électrophysiologie
et la cardiopathie congénitale chez l’adulte. Les auteurs présentent des considérations
pratiques au sujet de l’aiguillage urgent des patients en vue d’une ablation par cathéter
afin d’éliminer les arythmies auriculaires, de l’instauration d’une anticoagulothérapie
et de la mise en place d’un défibrillateur cardioverteur implantable (DCI) en prévention
primaire. Les patients atteints de la maladie d’Ebstein ou présentant une préexcitation
ventriculaire doivent être aiguillés sans tarder vers un spécialiste, en raison de
la forte prévalence de multiples voies accessoires et de l’incidence accrue de tachyarythmies
auriculaires susceptibles d’être mal tolérées. Chez les patients présentant une transposition
des gros vaisseaux et ayant subi une détransposition auriculaire, les arythmies auriculaires
doivent être prises en charge sans délai, car elles peuvent provoquer des arythmies
ventriculaires et la mort subite. Les arythmies auriculaires sont également susceptibles
d’être mal tolérées en présence d’une physiologie univentriculaire ou d’un syndrome
d’Eisenmenger. Une anticoagulothérapie de longue durée est généralement indiquée chez
les patients présentant une tachycardie réentrante intra-auriculaire soutenue ou une
fibrillation auriculaire et une valve mécanique, une sténose de la valve auriculoventriculaire
systémique modérée ou grave, des facteurs de risque d’accident vasculaire cérébral
classiques et/ou une cardiopathie congénitale modérée ou complexe. La seule indication
de classe I pour la mise en place d’un DCI en prévention primaire est une fraction
d’éjection ventriculaire gauche ≤ 35 % avec physiologie biventriculaire et symptômes
de classe II ou III selon la New York Heart Association. La mise en place d’un DCI
est justifiable chez certains adultes présentant une tétralogie de Fallot et plusieurs
facteurs de risque de mort subite relevés dans le cadre d’études d’observation. Les
indications relatives à un DCI chez les patients ayant un ventricule droit systémique
ou un cœur univentriculaire sont moins bien établies, ce qui fait ressortir la nécessité
d’autres recherches pour éclairer la stratification du risque.
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Article info
Publication history
Published online: July 16, 2019
Accepted:
July 7,
2019
Received:
June 14,
2019
Footnotes
See page 1705 for disclosure information.
Identification
Copyright
© 2019 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.
