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Canadian Journal of Cardiology

Arrhythmias in Adults With Congenital Heart Disease: What the Practicing Cardiologist Needs to Know

  • Paul Khairy
    Correspondence
    Corresponding author: Dr Paul Khairy, Montreal Heart Institute, 5000 Belanger St E., Montreal, Québec H1T 1C8, Canada. Tel.: +1-514-376-3330 ext. 3800; fax: +1-514-593-2581.
    Affiliations
    Electrophysiology Service and Adult Congenital Heart Disease Center, Montreal Heart Institute, Université de Montréal, Montreal, Québec, Canada
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      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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