van der Linde D, Konings EEM, Slager MA, et al. Birth prevalence of congenital heart disease worldwide: a systematic review and meta-analysis. J Am Coll Cardiol. 2011;58(21):2241–7.

Article  PubMed  Google Scholar 

O’Leary P. Prevalence, clinical presentation and natural history of patients with single ventricle. Prog Pediatr Cardiol. 2002;16(1):31–8.

Article  Google Scholar 

Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax. 1971;26(3):240–8.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Petrossian E, Reddy VM, McElhinney DB, et al. Early results of the extracardiac conduit Fontan operation. J Thorac Cardiovasc Surg. 1999;117(4):688–96.

Article  CAS  PubMed  Google Scholar 

Rychik J, Atz AM, Celermajer DS, et al. Evaluation and management of the child and adult with Fontan circulation: a scientific statement from the American Heart Association. Circulation. 2019. https://doi.org/10.1161/CIR.0000000000000696.

Article  PubMed  Google Scholar 

Ghimire LV, Chou F, Pundi K, Moon-Grady AJ. In-hospital outcomes in Fontan completion surgery according to age. Am J Cardiol. 2022;166:81–7.

Article  PubMed  Google Scholar 

Pundi KN, Johnson JN, Dearani JA, et al. 40-year follow-up after the Fontan operation: long-term outcomes of 1,052 patients. J Am Coll Cardiol. 2015;66(15):1700–10.

Article  PubMed  Google Scholar 

Dennis M, Zannino D, Plessis K, et al. Clinical outcomes in adolescents and adults after the Fontan procedure. J Am Coll Cardiol. 2018;71(9):1009–17.

Article  PubMed  Google Scholar 

Akintoye E, Miranda W, Veldtman GR, Connolly HM. National trends in Fontan operation and in-hospital outcomes in the USA. Heart. 2019;105(9):708–14.

Article  PubMed  Google Scholar 

Khairy P, Fernandes SM, Mayer JE, et al. Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery. Circulation. 2008;117(1):85–92.

Article  PubMed  Google Scholar 

Gewillig M. The Fontan circulation. Heart. 2005;91(6):839–46.

Article  PubMed  PubMed Central  Google Scholar 

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(2):457–64.

Article  PubMed  Google Scholar 

Khairy P, Van Hare GF, 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(10):e1–63.

Article  PubMed  Google Scholar 

de Leval MR, Deanfield JE. Four decades of Fontan palliation. Nat Rev Cardiol. 2010;7(9):520–7.

Article  PubMed  Google Scholar 

Lee JR, Kwak J, Kim KC, et al. Comparison of lateral tunnel and extracardiac conduit Fontan procedure. Interact Cardiovasc Thorac Surg. 2007;6(3):328–30.

Article  PubMed  Google Scholar 

Giannakoulas G, Dimopoulos K, Yuksel S, et al. Atrial tachyarrhythmias late after Fontan operation are related to increase in mortality and hospitalization. Int J Cardiol. 2012;157(2):221–6.

Article  PubMed  Google Scholar 

Carins TA, Shi Wy, Iyengar AJ, et al. Long-term outcomes after first-onset arrhythmia in Fontan physiology. J Thorac Cardiovasc Surg. 2016;152(5):1355–63 e1.

Article  PubMed  Google Scholar 

Durongpisitkul K, Porter C, Cetta F, et al. Predictors of early- and late-onset supraventricular tachyarrhythmias after Fontan operation. Circulation. 1998;98(11):1099–107.

Article  CAS  PubMed  Google Scholar 

Balaji S, Gewillig M, Bull C, de Leval MR, Deanfield JE. Arrhythmias after the Fontan procedure. Comparison of total cavopulmonary connection and atriopulmonary connection. Circulation. 1991;84(5 Suppl):III162–7.

CAS  PubMed  Google Scholar 

van den Bosch AE, Roos-Hesselink JW, Domburg R, Bogers AJ, Simoons M, Meijboom F. Long-term outcome and quality of life in adult patients after the Fontan operation. Am J Cardiol. 2004;93(9):1141–5.

Article  PubMed  Google Scholar 

Kim SJ, Kim W, Lim H, Lee J. Outcome of 200 patients after an extracardiac Fontan procedure. J Thorac Cardiovasc Surg. 2008;136(1):108–16.

Article  PubMed  Google Scholar 

Lasa JJ, Glatz AC, Daga A, Shah M. Prevalence of arrhythmias late after the Fontan operation. Am J Cardiol. 2014;113(7):1184–8.

Article  PubMed  Google Scholar 

Del Duca D, Tadevosyan A, Karbassi F, et al. Hypoxia in early life is associated with lasting changes in left ventricular structure and function at maturity in the rat. Int J Cardiol. 2010;156(2):165–73.

Article  PubMed  Google Scholar 

Dahlqvist JA, Karlsson M, Wiklund U, et al. Heart rate variability in children with Fontan circulation: lateral tunnel and extracardiac conduit. Pediatr Cardiol. 2012;33(2):307–15.

Article  PubMed  Google Scholar 

van Stee ES. Autonomic innervation of the heart. Environ Health Perspect. 1978;26:151–8.

Article  PubMed  PubMed Central  Google Scholar 

Zhao QY, Huang H, Tang YH, et al. Relationship between autonomic innervation in crista terminalis and atrial arrhythmia. J Cardiovasc Electrophysiol. 2009;20(5):551–7.

Article  PubMed  Google Scholar 

Jonas RA. The intra/extracardiac conduit fenestrated fontan. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2011;14(1):11–8.

Article  PubMed  Google Scholar 

Rodefeld MD, Bromberg B, Schuessler R, Boineau J, Cox J, Huddleston C. Atrial flutter after lateral tunnel construction in the modified Fontan operation: a canine model. J Thorac Cardiovasc Surg. 1996;111(3):514–26.

Article  CAS  PubMed  Google Scholar 

Khairy P, Poirier N. Is the extracardiac conduit the preferred Fontan approach for patients with univentricular hearts? The extracardiac conduit is not the preferred Fontan approach for patients with univentricular hearts. Circulation. 2012;126(21):2516–25 (Discussion 2525).

Article  PubMed  Google Scholar 

Gewillig M, Wyse R, de Leval M, Deanfield J. Early and late arrhythmias after the Fontan operation: predisposing factors and clinical consequences. Br Heart J. 1992;67(1):72–9.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Kverneland LS, Kramer P, Ovroutski S. Five decades of the Fontan operation: a systematic review of international reports on outcomes after univentricular palliation. Congenit Heart Dis. 2018;13(2):181–93.

Article  PubMed  Google Scholar 

Deshaies C, Hamilton R, Shohoudi A, et al. Thromboembolic risk after atriopulmonary, lateral tunnel, and extracardiac conduit Fontan surgery. J Am Coll Cardiol. 2019;74(8):1071–81.

Article  PubMed  Google Scholar 

Deal BJ, Jacobs ML. Management of the failing Fontan circulation. Heart. 2012;98(14):1098–104.

Article  PubMed  Google Scholar 

Wan D, Grewal J, Barlow A, et al. Atrial arrhythmias and thromboembolic complications in adults post Fontan surgery. Open Heart. 2020. https://doi.org/10.1136/openhrt-2019-001224.

Article  PubMed  PubMed Central  Google Scholar 

Pundi KN, Pundi K, Johnson J, et al. Sudden cardiac death and late arrhythmias after the Fontan operation. Congenit Heart Dis. 2017;12(1):17–23.

Article  PubMed  Google Scholar 

Song MK, Bae EJ, Kwon BS, et al. Intra-atrial reentrant tachycardia in adult patients after Fontan operation. Int J Cardiol. 2015;187:157–63.

Article  PubMed  Google Scholar 

Moore BM, Anderson R, Bisbet AM, et al. Ablation of atrial arrhythmias after the atriopulmonary Fontan procedure: mechanisms of arrhythmia and outcomes. JACC Clin Electrophysiol. 2018;4(10):1338–46.

Article  PubMed  Google Scholar 

Mandapati R, Walsh EP, Triedman JK. Pericaval and periannular intra-atrial reentrant tachycardias in patients with congenital heart disease. J Cardiovasc Electrophysiol. 2003;14(2):119–25.

Article  PubMed  Google Scholar 

Yap SC, Harris L, Downar E, Nanthakumar K, Silversides C, Chauhan V. Evolving electroanatomic substrate and intra-atrial reentrant tachycardia late after Fontan surgery. J Cardiovasc Electrophysiol. 2012;23(4):339–45.

Article  PubMed  Google Scholar 

Moore JP, Shannon KM, Fish FA, et al. Catheter ablation of supraventricular tachyarrhythmia after extracardiac Fontan surgery. Heart Rhythm. 2016;13(9):1891–7.

Article  PubMed  Google Scholar 

d’Udekem Y, Iygengar AJ, Cochrane AD, et al. The Fontan procedure: contemporary techniques have improved long-term outcomes. Circulation. 2007;116(11 Suppl):I157–64.

PubMed  Google Scholar 

Stamm C, Friehs I, Mayer JE, et al. Long-term results of the lateral tunnel Fontan operation. J Thorac Cardiovasc Surg. 2001;121(1):28–41.

Article