Arrhythmia and Devices

  • Ilaria Cazzoli
  • Sabine ErnstEmail author
Part of the Congenital Heart Disease in Adolescents and Adults book series (CHDAA)


Arrhythmias are a frequent complication in adult patients with congenital heart disease (ACHD). Exercise intolerance and ventricular dysfunction can ensue. Acute arrhythmias have the potential to be life-threatening with severe haemodynamic instability. In this chapter pathophysiology, type of arrhythmias and the range of interventions and devices used in ACHD are discussed in detail.


Arrhythmias Ablation Pacemaker Cardiac resynchronization Implantable defibrillator 


  1. 1.
    Walsh EP, Cecchin F. Arrhythmias in adult patients with congenital heart disease. Circulation. 2007;115(4):534–45.CrossRefPubMedGoogle Scholar
  2. 2.
    Zomer AC, Vaartjes I, Uiterwaal CS, van der Velde ET, van den Merkhof LF, Baur LH, et al. Circumstances of death in adult congenital heart disease. Int J Cardiol. 2012;154(2):168–72.CrossRefPubMedGoogle Scholar
  3. 3.
    Oechslin EN, Harrison DA, Connelly MS, Webb GD, Siu SC. Mode of death in adults with congenital heart disease. Am J Cardiol. 2000;86(10):1111–6.CrossRefPubMedGoogle Scholar
  4. 4.
    Ho SY, Anderson RH. Conduction tissue in congenital heart surgery. World J Surg. 1985;9(4):550–67.CrossRefPubMedGoogle Scholar
  5. 5.
    Zomer AC, Vaartjes I, van der Velde ET, de Jong HM, Konings TC, Wagenaar LJ, et al. Heart failure admissions in adults with congenital heart disease: risk factors and prognosis. Int J Cardiol. 2013;168(3):2487–93.CrossRefPubMedGoogle Scholar
  6. 6.
    Motonaga KS, Khairy P, Dubin AM. Electrophysiologic therapeutics in heart failure in adult congenital heart disease. Heart Fail Clin. 2014;10(1):69–89.CrossRefPubMedGoogle Scholar
  7. 7.
    Ernst S, Saenen J, Rydman R, Gomez F, Roy K, Mantziari L, et al. Utility of noninvasive arrhythmia mapping in patients with adult congenital heart disease. Cardiac Electrophysiol Clin. 2015;7(1):117–23.CrossRefGoogle Scholar
  8. 8.
    Chubb H, Williams SE, Wright M, Rosenthal E, O’Neill M. Tachyarrhythmias and catheter ablation in adult congenital heart disease. Expert Rev Cardiovasc Ther. 2014;12(6):751–70.CrossRefPubMedGoogle Scholar
  9. 9.
    Garrey WE. The nature of fibrillary contraction of the heart: Its relation to tissue mass and form. Am J Phys. 1914;33:397–414.Google Scholar
  10. 10.
    Ueda A, Adachi I, McCarthy KP, Li W, Ho SY, Uemura H. Substrates of atrial arrhythmias: histological insights from patients with congenital heart disease. Int J Cardiol. 2013;168(3):2481–6.CrossRefPubMedGoogle Scholar
  11. 11.
    Escudero C, Khairy P, Sanatani S. Electrophysiologic considerations in congenital heart disease and their relationship to heart failure. Can J Cardiol. 2013;29(7):821–9.CrossRefPubMedGoogle Scholar
  12. 12.
    Walsh EP. Interventional electrophysiology in patients with congenital heart disease. Circulation. 2007;115(25):3224–34.CrossRefPubMedGoogle Scholar
  13. 13.
    Abadir S, Khairy P. Electrophysiology and adult congenital heart disease: advances and options. Prog Cardiovasc Dis. 2011;53(4):281–92.CrossRefPubMedGoogle Scholar
  14. 14.
    Hebe J. Ebstein’s anomaly in adults. Arrhythmias: diagnosis and therapeutic approach. Thorac Cardiovasc Surg. 2000;48(4):214–9.CrossRefPubMedGoogle Scholar
  15. 15.
    Bar-Cohen Y, Khairy P, Morwood J, Alexander ME, Cecchin F, Berul CI. Inaccuracy of Wolff-Parkinson-white accessory pathway localization algorithms in children and patients with congenital heart defects. J Cardiovasc Electrophysiol. 2006;17(7):712–6.CrossRefPubMedGoogle Scholar
  16. 16.
    Epstein MR, Saul JP, Weindling SN, Triedman JK, Walsh EP. Atrioventricular reciprocating tachycardia involving twin atrioventricular nodes in patients with complex congenital heart disease. J Cardiovasc Electrophysiol. 2001;12(6):671–9.CrossRefPubMedGoogle Scholar
  17. 17.
    Suman-Horduna I, Babu-Narayan SV, Ueda A, Mantziari L, Gujic M, Marchese P, et al. Magnetic navigation in adults with atrial isomerism (heterotaxy syndrome) and supraventricular arrhythmias. Europace. 2013;15(6):877–85.CrossRefPubMedGoogle Scholar
  18. 18.
    van der Velde ET, Vriend JW, Mannens MM, Uiterwaal CS, Brand R, Mulder BJ. CONCOR, an initiative towards a national registry and DNA-bank of patients with congenital heart disease in the Netherlands: rationale, design, and first results. Eur J Epidemiol. 2005;20(6):549–57.CrossRefPubMedGoogle Scholar
  19. 19.
    Bouchardy J, Therrien J, Pilote L, Ionescu-Ittu R, Martucci G, Bottega N, et al. Atrial arrhythmias in adults with congenital heart disease. Circulation. 2009;120(17):1679–86.CrossRefPubMedGoogle Scholar
  20. 20.
    Kaemmerer H, Bauer U, Pensl U, Oechslin E, Gravenhorst V, Franke A, et al. Management of emergencies in adults with congenital cardiac disease. Am J Cardiol. 2008;101(4):521–5.CrossRefPubMedGoogle Scholar
  21. 21.
    Verheugt CL, Uiterwaal CS, van der Velde ET, Meijboom FJ, Pieper PG, Sieswerda GT, et al. The emerging burden of hospital admissions of adults with congenital heart disease. Heart. 2010;96(11):872–8.CrossRefPubMedGoogle Scholar
  22. 22.
    Triedman JK, Alexander ME, Berul CI, Bevilacqua LM, Walsh EP. Electroanatomic mapping of entrained and exit zones in patients with repaired congenital heart disease and intra-atrial reentrant tachycardia. Circulation. 2001;103(16):2060–5.CrossRefPubMedGoogle Scholar
  23. 23.
    Collins KK, Love BA, Walsh EP, Saul JP, Epstein MR, Triedman JK. Location of acutely successful radiofrequency catheter ablation of intraatrial reentrant tachycardia in patients with congenital heart disease. Am J Cardiol. 2000;86(9):969–74.CrossRefPubMedGoogle Scholar
  24. 24.
    Khairy P, Harris L, Landzberg MJ, Fernandes SM, Barlow A, Mercier LA, et al. Sudden death and defibrillators in transposition of the great arteries with intra-atrial baffles: a multicenter study. Circ Arrhythm Electrophysiol. 2008;1(4):250–7.CrossRefPubMedGoogle Scholar
  25. 25.
    Flinn CJ, Wolff GS, Dick M 2nd, Campbell RM, Borkat G, Casta A, et al. Cardiac rhythm after the Mustard operation for complete transposition of the great arteries. N Engl J Med. 1984;310(25):1635–8.CrossRefPubMedGoogle Scholar
  26. 26.
    Khairy P, Landzberg MJ, Lambert J, O’Donnell CP. Long-term outcomes after the atrial switch for surgical correction of transposition: a meta-analysis comparing the Mustard and Senning procedures. Cardiol Young. 2004;14(3):284–92.CrossRefPubMedGoogle Scholar
  27. 27.
    Li W, Somerville J. Atrial flutter in grown-up congenital heart (GUCH) patients. Clinical characteristics of affected population. Int J Cardiol. 2000;75(2-3):129–37. discussion 38–9CrossRefPubMedGoogle Scholar
  28. 28.
    Roos-Hesselink J, Perlroth MG, McGhie J, Spitaels S. Atrial arrhythmias in adults after repair of tetralogy of Fallot. Correlations with clinical, exercise, and echocardiographic findings. Circulation. 1995;91(8):2214–9.CrossRefPubMedGoogle Scholar
  29. 29.
    Bartz PJ, Driscoll DJ, Dearani JA, Puga FJ, Danielson GK, O'Leary PW, et al. Early and late results of the modified fontan operation for heterotaxy syndrome 30 years of experience in 142 patients. J Am Coll Cardiol. 2006;48(11):2301–5.CrossRefPubMedGoogle Scholar
  30. 30.
    Nurnberg JH, Ovroutski S, Alexi-Meskishvili V, Ewert P, Hetzer R, Lange PE. New onset arrhythmias after the extracardiac conduit Fontan operation compared with the intraatrial lateral tunnel procedure: early and midterm results. Ann Thorac Surg. 2004;78(6):1979–88. discussion 88CrossRefPubMedGoogle Scholar
  31. 31.
    Wong T, Davlouros PA, Li W, Millington-Sanders C, Francis DP, Gatzoulis MA. Mechano-electrical interaction late after Fontan operation: relation between P-wave duration and dispersion, right atrial size, and atrial arrhythmias. Circulation. 2004;109(19):2319–25.CrossRefPubMedGoogle Scholar
  32. 32.
    Kirsh JA, Walsh EP, Triedman JK. Prevalence of and risk factors for atrial fibrillation and intra-atrial reentrant tachycardia among patients with congenital heart disease. Am J Cardiol. 2002;90(3):338–40.CrossRefPubMedGoogle Scholar
  33. 33.
    Khairy P, Aboulhosn J, Gurvitz MZ, Opotowsky AR, Mongeon FP, Kay J, et al. Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: a multi-institutional study. Circulation. 2010;122(9):868–75.CrossRefPubMedGoogle Scholar
  34. 34.
    Fuster V, Ryden LE, Cannom DS, Crijns HJ, Curtis AB, Ellenbogen KA, et al. 2011 ACCF/AHA/HRS focused updates incorporated into the ACC/AHA/ESC 2006 guidelines for the management of patients with atrial fibrillation: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines developed in partnership with the European Society of Cardiology and in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society. J Am Coll Cardiol. 2011;57(11):e101–98.CrossRefPubMedGoogle Scholar
  35. 35.
    Egbe AC, Asirvatham SJ, Connolly HM, Kapa S, Desimone CV, Vaidya VR, et al. Outcomes of direct current cardioversion in adults with congenital heart disease. Am J Cardiol. 2017;119(9):1468–72.CrossRefPubMedPubMedCentralGoogle Scholar
  36. 36.
    Ernst S, Chun JK, Koektuerk B, Kuck KH. Magnetic navigation and catheter ablation of right atrial ectopic tachycardia in the presence of a hemi-azygos continuation: a magnetic navigation case using 3D electroanatomical mapping. J Cardiovasc Electrophysiol. 2009;20(1):99–102.CrossRefPubMedGoogle Scholar
  37. 37.
    Ernst S, Babu-Narayan SV, Keegan J, Horduna I, Lyne J, Till J, et al. Remote-controlled magnetic navigation and ablation with 3D image integration as an alternative approach in patients with intra-atrial baffle anatomy. Circ Arrhythm Electrophysiol. 2012;5(1):131–9.CrossRefPubMedGoogle Scholar
  38. 38.
    Broberg C, Meadows AK. Advances in imaging: the impact on the care of the adult with congenital heart disease. Prog Cardiovasc Dis. 2011;53(4):293–304.CrossRefPubMedGoogle Scholar
  39. 39.
    Harrison JL, Jensen HK, Peel SA, Chiribiri A, Grondal AK, Bloch LO, et al. Cardiac magnetic resonance and electroanatomical mapping of acute and chronic atrial ablation injury: a histological validation study. Eur Heart J. 2014;35(22):1486–95.CrossRefPubMedPubMedCentralGoogle Scholar
  40. 40.
    Peichl P, Kautzner J, Gebauer R. Ablation of atrial tachycardias after correction of complex congenital heart diseases: utility of intracardiac echocardiography. Europace. 2009;11(1):48–53.CrossRefPubMedGoogle Scholar
  41. 41.
    El-Said HG, Ing FF, Grifka RG, Nihill MR, Morris C, Getty-Houswright D, et al. 18-year experience with transseptal procedures through baffles, conduits, and other intra-atrial patches. Catheter Cardiovasc Interv. 2000;50(4):434–9. discussion 40CrossRefPubMedGoogle Scholar
  42. 42.
    de Groot NM, Atary JZ, Blom NA, Schalij MJ. Long-term outcome after ablative therapy of postoperative atrial tachyarrhythmia in patients with congenital heart disease and characteristics of atrial tachyarrhythmia recurrences. Circ Arrhythm Electrophysiol. 2010;3(2):148–54.CrossRefPubMedGoogle Scholar
  43. 43.
    Ueda A, Suman-Horduna I, Mantziari L, Gujic M, Marchese P, Ho SY, et al. Contemporary outcomes of supraventricular tachycardia ablation in congenital heart disease: a single-center experience in 116 patients. Circ Arrhythm Electrophysiol. 2013;6(3):606–13.CrossRefPubMedGoogle Scholar
  44. 44.
    Triedman JK, Alexander ME, Love BA, Collins KK, Berul CI, Bevilacqua LM, et al. Influence of patient factors and ablative technologies on outcomes of radiofrequency ablation of intra-atrial re-entrant tachycardia in patients with congenital heart disease. J Am Coll Cardiol. 2002;39(11):1827–35.CrossRefPubMedGoogle Scholar
  45. 45.
    de Groot NM, Zeppenfeld K, Wijffels MC, Chan WK, Blom NA, Van der Wall EE, et al. Ablation of focal atrial arrhythmia in patients with congenital heart defects after surgery: role of circumscribed areas with heterogeneous conduction. Heart Rhythm. 2006;3(5):526–35.CrossRefPubMedGoogle Scholar
  46. 46.
    Ector J, De Buck S, Adams J, Dymarkowski S, Bogaert J, Maes F, et al. Cardiac three-dimensional magnetic resonance imaging and fluoroscopy merging: a new approach for electroanatomic mapping to assist catheter ablation. Circulation. 2005;112(24):3769–76.CrossRefPubMedGoogle Scholar
  47. 47.
    Everett TH, Lee KW, Wilson EE, Guerra JM, Varosy PD, Olgin JE. Safety profiles and lesion size of different radiofrequency ablation technologies: a comparison of large tip, open and closed irrigation catheters. J Cardiovasc Electrophysiol. 2009;20(3):325–35.CrossRefPubMedGoogle Scholar
  48. 48.
    Ernst S. Robotic approach to catheter ablation. Curr Opin Cardiol. 2008;23(1):28–31.CrossRefPubMedGoogle Scholar
  49. 49.
    Ueda A, Horduna I, Rubens M, Ernst S. Reaching the ventricular aspect of the inferior isthmus in a Fontan patient using magnetic navigation. Heart Rhythm. 2013;10(7):1094–5.CrossRefPubMedGoogle Scholar
  50. 50.
    Khairy P, Van Hare GF, Balaji S, Berul CI, Cecchin F, Cohen MI, 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). Heart Rhythm. 2014;11(10):e102–65.CrossRefPubMedGoogle Scholar
  51. 51.
    Triedman JK, DeLucca JM, Alexander ME, Berul CI, Cecchin F, Walsh EP. Prospective trial of electroanatomically guided, irrigated catheter ablation of atrial tachycardia in patients with congenital heart disease. Heart Rhythm. 2005;2(7):700–5.CrossRefPubMedGoogle Scholar
  52. 52.
    Correa R, Sherwin ED, Kovach J, Mah DY, Alexander ME, Cecchin F, et al. Mechanism and ablation of arrhythmia following total cavopulmonary connection. Circ Arrhythm Electrophysiol. 2015;8(2):318–25.CrossRefPubMedGoogle Scholar
  53. 53.
    Correa R, Walsh EP, Alexander ME, Mah DY, Cecchin F, Abrams DJ, et al. Transbaffle mapping and ablation for atrial tachycardias after mustard, senning, or Fontan operations. J Am Heart Assoc. 2013;2(5):e000325.CrossRefPubMedPubMedCentralGoogle Scholar
  54. 54.
    Yap SC, Harris L, Silversides CK, Downar E, Chauhan VS. Outcome of intra-atrial re-entrant tachycardia catheter ablation in adults with congenital heart disease: negative impact of age and complex atrial surgery. J Am Coll Cardiol. 2010;56(19):1589–96.CrossRefPubMedGoogle Scholar
  55. 55.
    Ernst S, Pedersen M, Uebing A, Suman-Horduna I, Mantziari L, Li W, et al. Successful ablation of two right atrial tachycardias on either side of the lateral tunnel patch in a patient with double inlet left ventricle and total cavopulmonary connection: two sites and two mechanisms. Global Cardiol Sci Pract. 2013;2013(2):198–202.Google Scholar
  56. 56.
    Agnoletti G, Borghi A, Vignati G, Crupi GC. Fontan conversion to total cavopulmonary connection and arrhythmia ablation: clinical and functional results. Heart. 2003;89(2):193–8.CrossRefPubMedPubMedCentralGoogle Scholar
  57. 57.
    Gatzoulis MA, Walters J, McLaughlin PR, Merchant N, Webb GD, Liu P. Late arrhythmia in adults with the mustard procedure for transposition of great arteries: a surrogate marker for right ventricular dysfunction? Heart. 2000;84(4):409–15.CrossRefPubMedPubMedCentralGoogle Scholar
  58. 58.
    Dobson R, Danton M, Nicola W, Hamish W. The natural and unnatural history of the systemic right ventricle in adult survivors. J Thorac Cardiovasc Surg. 2013;145(6):1493–501. discussion 501–3CrossRefPubMedGoogle Scholar
  59. 59.
    Wu J, Deisenhofer I, Ammar S, Fichtner S, Reents T, Zhu P, et al. Acute and long-term outcome after catheter ablation of supraventricular tachycardia in patients after the Mustard or Senning operation for D-transposition of the great arteries. Europace. 2013;15(6):886–91.CrossRefPubMedGoogle Scholar
  60. 60.
    Silka MJ, Hardy BG, Menashe VD, Morris CD. A population-based prospective evaluation of risk of sudden cardiac death after operation for common congenital heart defects. J Am Coll Cardiol. 1998;32(1):245–51.CrossRefPubMedGoogle Scholar
  61. 61.
    Koyak Z, Harris L, de Groot JR, Silversides CK, Oechslin EN, Bouma BJ, et al. Sudden cardiac death in adult congenital heart disease. Circulation. 2012;126(16):1944–54.CrossRefPubMedGoogle Scholar
  62. 62.
    Harrison DA, Harris L, Siu SC, MacLoghlin CJ, Connelly MS, Webb GD, et al. Sustained ventricular tachycardia in adult patients late after repair of tetralogy of Fallot. J Am Coll Cardiol. 1997;30(5):1368–73.CrossRefPubMedGoogle Scholar
  63. 63.
    Shiga T, Kasanuki H. Drug therapy for ventricular tachyarrhythmia in heart failure. Circ J. 2007;71(Suppl A):A90–6.CrossRefPubMedGoogle Scholar
  64. 64.
    Gatzoulis MA, Balaji S, Webber SA, Siu SC, Hokanson JS, Poile C, et al. Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study. Lancet (Lond, England). 2000;356(9234):975–81.CrossRefGoogle Scholar
  65. 65.
    Murphy JG, Gersh BJ, Mair DD, Fuster V, McGoon MD, Ilstrup DM, et al. Long-term outcome in patients undergoing surgical repair of tetralogy of Fallot. N Engl J Med. 1993;329(9):593–9.CrossRefPubMedGoogle Scholar
  66. 66.
    Bonello B, Kempny A, Uebing A, Li W, Kilner PJ, Diller GP, et al. Right atrial area and right ventricular outflow tract akinetic length predict sustained tachyarrhythmia in repaired tetralogy of Fallot. Int J Cardiol. 2013;168(4):3280–6.CrossRefPubMedPubMedCentralGoogle Scholar
  67. 67.
    Khairy P, Landzberg MJ, Gatzoulis MA, Lucron H, Lambert J, Marcon F, et al. Value of programmed ventricular stimulation after tetralogy of fallot repair: a multicenter study. Circulation. 2004;109(16):1994–2000.CrossRefPubMedGoogle Scholar
  68. 68.
    Anderson KR, Lie JT. Pathologic anatomy of Ebstein’s anomaly of the heart revisited. Am J Cardiol. 1978;41(4):739–45.CrossRefPubMedGoogle Scholar
  69. 69.
    Zeppenfeld K, Schalij MJ, Bartelings MM, Tedrow UB, Koplan BA, Soejima K, et al. Catheter ablation of ventricular tachycardia after repair of congenital heart disease: electroanatomic identification of the critical right ventricular isthmus. Circulation. 2007;116(20):2241–52.CrossRefPubMedGoogle Scholar
  70. 70.
    Russo AM, Stainback RF, Bailey SR, Epstein AE, Heidenreich PA, Jessup M, et al. ACCF/HRS/AHA/ASE/HFSA/SCAI/SCCT/SCMR 2013 appropriate use criteria for implantable cardioverter-defibrillators and cardiac resynchronization therapy: a report of the American College of Cardiology Foundation appropriate use criteria task force, Heart Rhythm Society, American Heart Association, American Society of Echocardiography, Heart Failure Society of America, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, and Society for Cardiovascular Magnetic Resonance. Heart Rhythm. 2013;10(4):e11–58.CrossRefPubMedGoogle Scholar
  71. 71.
    Furushima H, Chinushi M, Sugiura H, Komura S, Tanabe Y, Watanabe H, et al. Ventricular tachycardia late after repair of congenital heart disease: efficacy of combination therapy with radiofrequency catheter ablation and class III antiarrhythmic agents and long-term outcome. J Electrocardiol. 2006;39(2):219–24.CrossRefPubMedGoogle Scholar
  72. 72.
    Morwood JG, Triedman JK, Berul CI, Khairy P, Alexander ME, Cecchin F, et al. Radiofrequency catheter ablation of ventricular tachycardia in children and young adults with congenital heart disease. Heart Rhythm. 2004;1(3):301–8.CrossRefPubMedGoogle Scholar
  73. 73.
    Gross GJ, Chiu CC, Hamilton RM, Kirsh JA, Stephenson EA. Natural history of postoperative heart block in congenital heart disease: implications for pacing intervention. Heart Rhythm. 2006;3(5):601–4.CrossRefPubMedGoogle Scholar
  74. 74.
    Hansky B, Blanz U, Peuster M, Gueldner H, Sandica E, Crespo-Martinez E, et al. Endocardial pacing after Fontan-type procedures. Pacing Clin Electrophysiol. 2005;28(2):140–8.CrossRefPubMedGoogle Scholar
  75. 75.
    Paech C, Kostelka M, Dahnert I, Flosdorff P, Riede FT, Gebauer RA. Performance of steroid eluting bipolar epicardial leads in pediatric and congenital heart disease patients: 15 years of single center experience. J Cardiothorac Surg. 2014;9:84.CrossRefPubMedPubMedCentralGoogle Scholar
  76. 76.
    Silvetti MS, Drago F, Di Carlo D, Placidi S, Brancaccio G, Carotti A. Cardiac pacing in paediatric patients with congenital heart defects: transvenous or epicardial? Europace. 2013;15(9):1280–6.CrossRefPubMedGoogle Scholar
  77. 77.
    Janousek J, van Geldorp IE, Krupickova S, Rosenthal E, Nugent K, Tomaske M, et al. Permanent cardiac pacing in children: choosing the optimal pacing site: a multicenter study. Circulation. 2013;127(5):613–23.CrossRefPubMedGoogle Scholar
  78. 78.
    Khairy P, Fernandes SM, Mayer JE Jr, Triedman JK, Walsh EP, Lock JE, et al. Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery. Circulation. 2008;117(1):85–92.CrossRefPubMedGoogle Scholar
  79. 79.
    Khairy P, Harris L, Landzberg MJ, Viswanathan S, Barlow A, Gatzoulis MA, et al. Implantable cardioverter-defibrillators in tetralogy of Fallot. Circulation. 2008;117(3):363–70.CrossRefPubMedGoogle Scholar
  80. 80.
    Koyak Z, de Groot JR, Bouma BJ, Van Gelder IC, Budts W, Zwinderman AH, et al. Symptomatic but not asymptomatic non-sustained ventricular tachycardia is associated with appropriate implantable cardioverter therapy in tetralogy of Fallot. Int J Cardiol. 2013;167(4):1532–5.CrossRefPubMedGoogle Scholar
  81. 81.
    Kammeraad JA, van Deurzen CH, Sreeram N, Bink-Boelkens MT, Ottenkamp J, Helbing WA, et al. Predictors of sudden cardiac death after Mustard or Senning repair for transposition of the great arteries. J Am Coll Cardiol. 2004;44(5):1095–102.CrossRefPubMedGoogle Scholar
  82. 82.
    Berul CI, Van Hare GF, Kertesz NJ, Dubin AM, Cecchin F, Collins KK, et al. Results of a multicenter retrospective implantable cardioverter-defibrillator registry of pediatric and congenital heart disease patients. J Am Coll Cardiol. 2008;51(17):1685–91.CrossRefPubMedGoogle Scholar
  83. 83.
    DeMaso DR, Lauretti A, Spieth L, van der Feen JR, Jay KS, Gauvreau K, et al. Psychosocial factors and quality of life in children and adolescents with implantable cardioverter-defibrillators. Am J Cardiol. 2004;93(5):582–7.CrossRefPubMedGoogle Scholar
  84. 84.
    Hayward RM, Dewland TA, Moyers B, Vittinghoff E, Tanel RE, Marcus GM, et al. Device complications in adult congenital heart disease. Heart Rhythm. 2015;12(2):338–44.CrossRefPubMedGoogle Scholar
  85. 85.
    Vehmeijer JT, Koyak Z, Bokma JP, Budts W, Harris L, Mulder BJ, et al. Sudden cardiac death in adults with congenital heart disease: does QRS-complex fragmentation discriminate in structurally abnormal hearts? Europace. 2017.
  86. 86.
    Epstein AE, DiMarco JP, Ellenbogen KA, Estes NA 3rd, Freedman RA, Gettes LS, et al. ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices) developed in collaboration with the American Association for Thoracic Surgery and Society of Thoracic Surgeons. J Am Coll Cardiol. 2008;51(21):e1–62.CrossRefPubMedGoogle Scholar
  87. 87.
    Abraham WT, Fisher WG, Smith AL, Delurgio DB, Leon AR, Loh E, et al. Cardiac resynchronization in chronic heart failure. N Engl J Med. 2002;346(24):1845–53.CrossRefPubMedPubMedCentralGoogle Scholar
  88. 88.
    Cazeau S, Leclercq C, Lavergne T, Walker S, Varma C, Linde C, et al. Effects of multisite biventricular pacing in patients with heart failure and intraventricular conduction delay. N Engl J Med. 2001;344(12):873–80.CrossRefPubMedGoogle Scholar
  89. 89.
    Cleland JG, Daubert JC, Erdmann E, Freemantle N, Gras D, Kappenberger L, et al. The effect of cardiac resynchronization on morbidity and mortality in heart failure. N Engl J Med. 2005;352(15):1539–49.CrossRefPubMedPubMedCentralGoogle Scholar
  90. 90.
    Khairy P, Fournier A, Thibault B, Dubuc M, Therien J, Vobecky SJ. Cardiac resynchronization therapy in congenital heart disease. Int J Cardiol. 2006;109(2):160–8.CrossRefPubMedGoogle Scholar
  91. 91.
    Diller GP, Dimopoulos K, Okonko D, Li W, Babu-Narayan SV, Broberg CS, et al. Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication. Circulation. 2005;112(6):828–35.CrossRefPubMedGoogle Scholar
  92. 92.
    Dubin AM, Janousek J, Rhee E, Strieper MJ, Cecchin F, Law IH, et al. Resynchronization therapy in pediatric and congenital heart disease patients: an international multicenter study. J Am Coll Cardiol. 2005;46(12):2277–83.CrossRefPubMedGoogle Scholar
  93. 93.
    Cecchin F, Frangini PA, Brown DW, Fynn-Thompson F, Alexander ME, Triedman JK, et al. Cardiac resynchronization therapy (and multisite pacing) in pediatrics and congenital heart disease: five years experience in a single institution. J Cardiovasc Electrophysiol. 2009;20(1):58–65.CrossRefPubMedGoogle Scholar
  94. 94.
    Janousek J, Gebauer RA. Cardiac resynchronization therapy in pediatric and congenital heart disease. Pacing Clin Electrophysiol. 2008;31(Suppl 1):S21–3.CrossRefPubMedGoogle Scholar
  95. 95.
    Zimmerman FJ, Starr JP, Koenig PR, Smith P, Hijazi ZM, Bacha EA. Acute hemodynamic benefit of multisite ventricular pacing after congenital heart surgery. Ann Thorac Surg. 2003;75(6):1775–80.CrossRefPubMedGoogle Scholar
  96. 96.
    Budts W, Roos-Hesselink J, Radle-Hurst T, Eicken A, McDonagh TA, Lambrinou E, et al. Treatment of heart failure in adult congenital heart disease: a position paper of the Working Group of Grown-Up Congenital Heart Disease and the Heart Failure Association of the European Society of Cardiology. Eur Heart J. 2016;37(18):1419–27.CrossRefPubMedPubMedCentralGoogle Scholar
  97. 97.
    Thambo JB, De Guillebon M, Xhaet O, Dos Santos P, Roubertie F, Labrousse L, et al. Biventricular pacing in patients with Tetralogy of Fallot: non-invasive epicardial mapping and clinical impact. Int J Cardiol. 2013;163(2):170–4.CrossRefPubMedGoogle Scholar
  98. 98.
    Stewart GC, Mayer JE Jr. Heart transplantation in adults with congenital heart disease. Heart Fail Clin. 2014;10(1):207–18.CrossRefPubMedGoogle Scholar
  99. 99.
    Mondesert B, Marcotte F, Mongeon FP, Dore A, Mercier LA, Ibrahim R, et al. Fontan circulation: success or failure? Can J Cardiol. 2013;29(7):811–20.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of CardiologyRoyal Brompton and Harefield HospitalLondonUK
  2. 2.National Heart and Lung InstituteImperial College LondonLondonUK

Personalised recommendations