Canadian Journal of Cardiology

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

  • Paul Khairy
    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.
    Electrophysiology Service and Adult Congenital Heart Disease Center, Montreal Heart Institute, Université de Montréal, Montreal, Québec, Canada
    Search for articles by this author


      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.


      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.
      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Canadian Journal of Cardiology
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Khairy P.
        • Van Hare G.F.
        • Balaji S.
        • et al.
        • PACES/HRS expert consensus statement on the recognition and management of arrhythmias in adult congenital heart disease: developed in partnership between the Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS)
        Endorsed by the governing bodies of PACES, HRS, the American College of Cardiology (ACC), the American Heart Association (AHA), the European Heart Rhythm Association (EHRA), the Canadian Heart Rhythm Society (CHRS), and the International Society for Adult Congenital Heart Disease (ISACHD).
        Can J Cardiol. 2014; 30: e1-e63
        • Sherwin E.D.
        • Triedman J.K.
        • Walsh E.P.
        Update on interventional electrophysiology in congenital heart disease: evolving solutions for complex hearts.
        Circ Arrhythm Electrophysiol. 2013; 6: 1032-1040
        • Kumar S.
        • Tedrow U.B.
        • Triedman J.K.
        Arrhythmias in adult congenital heart disease: diagnosis and management.
        Cardiol Clin. 2015; 33 (viii): 571-588
        • Escudero C.
        • Khairy P.
        • Sanatani S.
        Electrophysiologic considerations in congenital heart disease and their relationship to heart failure.
        Can J Cardiol. 2013; 29: 821-829
        • Attenhofer Jost C.H.
        • Connolly H.M.
        • Dearani J.A.
        • Edwards W.D.
        • Danielson G.K.
        Ebstein's anomaly.
        Circulation. 2007; 115: 277-285
        • Wei W.
        • Zhan X.
        • Xue Y.
        • et al.
        Features of accessory pathways in adult Ebstein's anomaly.
        Europace. 2014; 16: 1619-1625
        • Attenhofer Jost C.H.
        • Connolly H.M.
        • Edwards W.D.
        • et al.
        Ebstein's anomaly—review of a multifaceted congenital cardiac condition.
        Swiss Med Wkly. 2005; 135: 269-281
        • Pappone C.
        • Manguso F.
        • Santinelli R.
        • et al.
        Radiofrequency ablation in children with asymptomatic Wolff-Parkinson-White syndrome.
        N Engl J Med. 2004; 351: 1197-1205
        • Lupoglazoff J.M.
        • Magnier S.
        • Kabaker M.
        • et al.
        Role of right-to-left atrial shunt in exercise tolerance of patients with Ebstein anomaly.
        Arch Mal Coeur Vaiss. 1999; 92: 631-636
        • Dos L.
        • Teruel L.
        • Ferreira I.J.
        • et al.
        Late outcome of Senning and Mustard procedures for correction of transposition of the great arteries.
        Heart. 2005; 91: 652-656
        • Chaix M.A.
        • Chergui M.
        • Leduc C.
        • Khairy P.
        Sudden death in transposition of the great arteries with atrial switch surgery: autopsy evidence of acute myocardial ischemia despite normal coronary arteries.
        Int J Cardiol. 2019; 288: 65-67
        • Kammeraad J.A.
        • van Deurzen C.H.
        • Sreeram N.
        • et al.
        Predictors of sudden cardiac death after Mustard or Senning repair for transposition of the great arteries.
        J Am Coll Cardiol. 2004; 44: 1095-1102
        • 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.
        Circ Arrhythm Electrophysiol. 2008; 1: 250-257
        • Khairy P.
        Sudden cardiac death in transposition of the great arteries with a Mustard or Senning baffle: the myocardial ischemia hypothesis.
        Curr Opin Cardiol. 2017; 32: 101-107
        • Matthys D.
        • De Wolf D.
        • Verhaaren H.
        Lack of increase in stroke volume during exercise in asymptomatic adolescents in sinus rhythm after intra-atrial repair for simple transposition of the great arteries.
        Am J Cardiol. 1996; 78: 595-596
        • Derrick G.P.
        • Narang I.
        • White P.A.
        • et al.
        Failure of stroke volume augmentation during exercise and dobutamine stress is unrelated to load-independent indexes of right ventricular performance after the Mustard operation.
        Circulation. 2000; 102: III154-III159
        • Labbe L.
        • Douard H.
        • Barat J.L.
        • et al.
        Alteration of myocardial viability and systemic ventricular dysfunction after Senning procedure.
        Arch Mal Coeur Vaiss. 1997; 90: 631-637
        • Millane T.
        • Bernard E.J.
        • Jaeggi E.
        • et al.
        Role of ischemia and infarction in late right ventricular dysfunction after atrial repair of transposition of the great arteries.
        J Am Coll Cardiol. 2000; 35: 1661-1668
        • Lubiszewska B.
        • Gosiewska E.
        • Hoffman P.
        • et al.
        Myocardial perfusion and function of the systemic right ventricle in patients after atrial switch procedure for complete transposition: long-term follow-up.
        J Am Coll Cardiol. 2000; 36: 1365-1370
        • Khairy P.
        • Poirier N.
        • Mercier L.A.
        Univentricular heart.
        Circulation. 2007; 115: 800-812
        • de Leval M.R.
        The Fontan circulation: a challenge to William Harvey?.
        Nat Clin Pract Cardiovasc Med. 2005; 2: 202-208
        • Weipert J.
        • Noebauer C.
        • Schreiber C.
        • et al.
        Occurrence and management of atrial arrhythmia after long-term Fontan circulation.
        J Thorac Cardiovasc Surg. 2004; 127: 457-464
        • Ali W.B.
        • Bouhout I.
        • Khairy P.
        • Bouchard D.
        • Poirier N.
        Extracardiac versus lateral tunnel Fontan: a meta-analysis of long-term results.
        Ann Thorac Surg. 2018; 107: 837-843
        • Khairy P.
        • Fernandes S.M.
        • Mayer Jr., J.E.
        • et al.
        Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery.
        Circulation. 2008; 117: 85-92
        • Moore B.
        • Yu C.
        • Kotchetkova I.
        • Cordina R.
        • Celermajer D.S.
        Incidence and clinical characteristics of sudden cardiac death in adult congenital heart disease.
        Int J Cardiol. 2018; 254: 101-106
        • Diller G.P.
        • Dimopoulos K.
        • Broberg C.S.
        • et al.
        Presentation, survival prospects, and predictors of death in Eisenmenger syndrome: a combined retrospective and case-control study.
        Eur Heart J. 2006; 27: 1737-1742
        • Cantor W.J.
        • Harrison D.A.
        • Moussadji J.S.
        • et al.
        Determinants of survival and length of survival in adults with Eisenmenger syndrome.
        Am J Cardiol. 1999; 84: 677-681
        • Wolf P.A.
        • Abbott R.D.
        • Kannel W.B.
        Atrial fibrillation as an independent risk factor for stroke: the Framingham Study.
        Stroke. 1991; 22: 983-988
        • Wyse D.G.
        • Waldo A.L.
        • DiMarco J.P.
        • et al.
        A comparison of rate control and rhythm control in patients with atrial fibrillation.
        N Engl J Med. 2002; 347: 1825-1833
        • Hart R.G.
        • Pearce L.A.
        • Rothbart R.M.
        • et al.
        Stroke with intermittent atrial fibrillation: incidence and predictors during aspirin therapy. Stroke Prevention in Atrial Fibrillation Investigators.
        J Am Coll Cardiol. 2000; 35: 183-187
        • Gladstone D.J.
        • Bui E.
        • Fang J.
        • et al.
        Potentially preventable strokes in high-risk patients with atrial fibrillation who are not adequately anticoagulated.
        Stroke. 2009; 40: 235-240
        • Dulli D.
        • D'Alessio D.J.
        • Palta M.
        • Levine R.L.
        • Schutta H.S.
        Differentiation of acute cortical and subcortical ischemic stroke by risk factors and clinical examination findings.
        Neuroepidemiology. 1998; 17: 80-89
        • Hoffmann A.
        • Chockalingam P.
        • Balint O.H.
        • et al.
        Cerebrovascular accidents in adult patients with congenital heart disease.
        Heart. 2010; 96: 1223-1226
        • Bouchardy J.
        • Therrien J.
        • Pilote L.
        • et al.
        Atrial arrhythmias in adults with congenital heart disease.
        Circulation. 2009; 120: 1679-1686
        • Mongeon F.P.
        • Macle L.
        • Beauchesne L.M.
        • et al.
        Non-vitamin K antagonist oral anticoagulants (NOACs) in adult congenital heart disease.
        Can J Cardiol. 2019;
        • Khairy P.
        • Aboulhosn J.
        • Broberg C.S.
        • et al.
        Thromboprophylaxis for atrial arrhythmias in congenital heart disease: a multicenter study.
        Int J Cardiol. 2016; 223: 729-735
        • Warnes C.A.
        • Williams R.G.
        • Bashore T.M.
        • et al.
        ACC/AHA 2008 Guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease).
        J Am Coll Cardiol. 2008; 52: 1890-1947
        • Feltes T.F.
        • Friedman R.A.
        Transesophageal echocardiographic detection of atrial thrombi in patients with nonfibrillation atrial tachyarrhythmias and congenital heart disease.
        J Am Coll Cardiol. 1994; 24: 1365-1370
        • Ammash N.M.
        • Phillips S.D.
        • Hodge D.O.
        • et al.
        Outcome of direct current cardioversion for atrial arrhythmias in adults with congenital heart disease.
        Int J Cardiol. 2012; 154: 270-274
        • Andrade J.G.
        • Verma A.
        • Mitchell L.B.
        • et al.
        2018 Focused update of the Canadian Cardiovascular Society guidelines for the management of atrial fibrillation.
        Can J Cardiol. 2018; 34: 1371-1392
        • Gronberg T.
        • Hartikainen J.E.
        • Nuotio I.
        • et al.
        Anticoagulation, CHA2DS2VASc score, and thromboembolic risk of cardioversion of acute atrial fibrillation (from the FinCV study).
        Am J Cardiol. 2016; 117: 1294-1298
        • Nuotio I.
        • Hartikainen J.E.
        • Gronberg T.
        • Biancari F.
        • Airaksinen K.E.
        Time to cardioversion for acute atrial fibrillation and thromboembolic complications.
        JAMA. 2014; 312: 647-649
        • Airaksinen K.E.
        • Gronberg T.
        • Nuotio I.
        • et al.
        Thromboembolic complications after cardioversion of acute atrial fibrillation: the FinCV (Finnish CardioVersion) study.
        J Am Coll Cardiol. 2013; 62: 1187-1192
        • Idorn L.
        • Jensen A.S.
        • Juul K.
        • et al.
        Thromboembolic complications in Fontan patients: population-based prevalence and exploration of the etiology.
        Pediatr Cardiol. 2013; 34: 262-272
        • Jahangiri M.
        • Shore D.
        • Kakkar V.
        • Lincoln C.
        • Shinebourne E.
        Coagulation factor abnormalities after the Fontan procedure and its modifications.
        J Thorac Cardiovasc Surg. 1997; 113 (discussion: 992-3): 989-992
        • van Nieuwenhuizen R.C.
        • Peters M.
        • Lubbers L.J.
        • et al.
        Abnormalities in liver function and coagulation profile following the Fontan procedure.
        Heart. 1999; 82: 40-46
        • Tomita H.
        • Yamada O.
        • Ohuchi H.
        • et al.
        Coagulation profile, hepatic function, and hemodynamics following Fontan-type operations.
        Cardiol Young. 2001; 11: 62-66
        • Khairy P.
        • Poirier N.
        The extracardiac conduit is not the preferred Fontan approach for patients with univentricular hearts.
        Circulation. 2012; 126: 2516-2525
        • Valente A.M.
        • Bhatt A.B.
        • Cook S.
        • et al.
        The CALF (Congenital Heart Disease in Adults Lower Extremity Systemic Venous Health in Fontan Patients) study.
        J Am Coll Cardiol. 2010; 56: 144-150
        • Ravn H.B.
        • Hjortdal V.E.
        • Stenbog E.V.
        • et al.
        Increased platelet reactivity and significant changes in coagulation markers after cavopulmonary connection.
        Heart. 2001; 85: 61-65
        • Monagle P.
        • Cochrane A.
        • Roberts R.
        • et al.
        A multicenter, randomized trial comparing heparin/warfarin and acetylsalicylic acid as primary thromboprophylaxis for 2 years after the Fontan procedure in children.
        J Am Coll Cardiol. 2011; 58: 645-651
        • Potter B.J.
        • Leong-Sit P.
        • Fernandes S.M.
        • et al.
        Effect of aspirin and warfarin therapy on thromboembolic events in patients with univentricular hearts and Fontan palliation.
        Int J Cardiol. 2013; 168: 3940-3943
        • Fyfe D.A.
        • Kline C.H.
        • Sade R.M.
        • Gillette P.C.
        Transesophageal echocardiography detects thrombus formation not identified by transthoracic echocardiography after the Fontan operation.
        J Am Coll Cardiol. 1991; 18: 1733-1737
        • Engelings C.C.
        • Helm P.C.
        • Abdul-Khaliq H.
        • et al.
        Cause of death in adults with congenital heart disease—an analysis of the German National Register for Congenital Heart Defects.
        Int J Cardiol. 2016; 211: 31-36
        • Epstein A.E.
        • DiMarco J.P.
        • Ellenbogen K.A.
        • et al.
        2012 ACCF/AHA/HRS focused update incorporated into the ACCF/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines and the Heart Rhythm Society.
        J Am Coll Cardiol. 2013; 61: e6-e75
        • Vehmeijer J.T.
        • Brouwer T.F.
        • Limpens J.
        • et al.
        Implantable cardioverter-defibrillators in adults with congenital heart disease: a systematic review and meta-analysis.
        Eur Heart J. 2016; 37: 1439-1448
        • Gatzoulis M.A.
        • Balaji S.
        • Webber S.A.
        • et al.
        Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study.
        Lancet. 2000; 356: 975-981
        • Khairy P.
        • Landzberg M.J.
        • Gatzoulis M.A.
        • et al.
        Value of programmed ventricular stimulation after tetralogy of fallot repair: a multicenter study.
        Circulation. 2004; 109: 1994-2000
        • Khairy P.
        • Harris L.
        • Landzberg M.J.
        • et al.
        Implantable cardioverter-defibrillators in tetralogy of Fallot.
        Circulation. 2008; 117: 363-370
        • Valente A.M.
        • Gauvreau K.
        • Egidy Assenza G.
        • et al.
        Contemporary predictors of death and sustained ventricular tachycardia in patients with repaired tetralogy of Fallot enrolled in the INDICATOR cohort.
        Heart. 2014; 100: 247-253