Choussat A, Fontan F, Besse P, Vallot F, Chauve A, Bricaud H (1977) Selection for Fontan’s procedure. In: Anderson R, Shinebourne E (eds) Pediatric cardiology. Churchhill Livingstone, London, pp 559–566

Google Scholar 

Rychik J, Atz AM, Celermajer DS et al (2019) Evaluation and management of the child and adult with Fontan circulation: a scientific statement from the American Heart Association. Circulation 140(6):e234–e284. https://doi.org/10.1161/CIR.0000000000000696

Article  PubMed  Google Scholar 

Balaji S, Daga A, Bradley DJ et al (2014) An international multicenter study comparing arrhythmia prevalence between the intracardiac lateral tunnel and the extracardiac conduit type of Fontan operations. J Thorac Cardiovasc Surg 148(2):576–581. https://doi.org/10.1016/j.jtcvs.2013.08.070

Article  PubMed  Google Scholar 

Cohen MI, Wernovsky G, Vetter VL et al (1998) Sinus node function after a systematically staged Fontan procedure. Circulation 98(19 Suppl):352–358

Google Scholar 

Dilawar M, Bradley SM, Saul JP, Stroud MR, Balaji S (2003) Sinus node dysfunction after intraatrial lateral tunnel and extracardiac conduit Fontan procedures. Pediatr Cardiol 24(3):284–288. https://doi.org/10.1007/s00246-002-0238-9

Article  CAS  PubMed  Google Scholar 

Pundi KN, Pundi KN, Johnson JN et al (2017) Sudden cardiac death and late arrhythmias after the Fontan operation. Congenit Heart Dis 12(1):17–23. https://doi.org/10.1111/chd.12401

Article  PubMed  Google Scholar 

Gewillig M, Wyse RK, de Leval MR, Deanfield JE (1992) Early and late arrhythmias after the Fontan operation: predisposing factors and clinical consequences. Br Heart J 67(1):72–79. https://doi.org/10.1136/hrt.67.1.72

Article  CAS  PubMed  PubMed Central  Google Scholar 

Carins TA, Shi WY, Iyengar AJ et al (2016) Long-term outcomes after first-onset arrhythmia in Fontan physiology. J Thorac Cardiovasc Surg 152(5):1355-1363.e1. https://doi.org/10.1016/j.jtcvs.2016.07.073

Article  PubMed  Google Scholar 

Fishberger SB, Wernovsky G, Gentles TL et al (1996) Long-term outcome in patients with pacemakers following the Fontan operation. Am J Cardiol 77(10):887–889. https://doi.org/10.1016/s0002-9149(97)89191-6

Article  CAS  PubMed  Google Scholar 

Balaji S, Evers PD, Batra AS, Moore J (2023) Management of junctional rhythm in patients after the Fontan operation: a multicenter congenital cardiology survey. Pediatr Cardiol. https://doi.org/10.1007/s00246-023-03296-6

Article  PubMed  Google Scholar 

Dodge-Khatami A, Rahn M, Prêtre R, Bauersfeld U (2005) Dual chamber epicardial pacing for the failing atriopulmonary Fontan patient. Ann Thorac Surg 80(4):1440–1444. https://doi.org/10.1016/j.athoracsur.2005.03.128

Article  PubMed  Google Scholar 

Poh CL, Celermajer DS, Grigg LE et al (2019) Pacemakers are associated with a higher risk of late death and transplantation in the Fontan population. Int J Cardiol 282:33–37. https://doi.org/10.1016/j.ijcard.2019.01.088

Article  PubMed  Google Scholar 

Bulic A, Zimmerman FJ, Ceresnak SR et al (2017) Ventricular pacing in single ventricles—a bad combination. Heart Rhythm 14(6):853–857. https://doi.org/10.1016/j.hrthm.2017.03.035

Article  PubMed  Google Scholar 

Kochav JD, Rosenbaum M, Kochav SM, Slater E, Wassercug-Zemer N, Lewis MJ (2020) Effect of ventricular pacing on morbidity in adults after Fontan repair. Am J Cardiol 125(8):1263–1269. https://doi.org/10.1016/j.amjcard.2020.01.026

Article  PubMed  Google Scholar 

Williams RV, Travison T, Kaltman JR et al (2013) Comparison of Fontan survivors with and without pacemakers: a report from the pediatric heart network Fontan cross-sectional study. Congenit Heart Dis 8(1):32–39. https://doi.org/10.1111/j.1747-0803.2012.00692.x

Article  PubMed  Google Scholar 

Egbe AC, Huntley GD, Connolly HM et al (2017) Outcomes of cardiac pacing in adult patients after a Fontan operation. Am Heart J 194:92–98. https://doi.org/10.1016/j.ahj.2017.08.019

Article  PubMed  Google Scholar 

Barber BJ, Batra AS, Burch GH et al (2005) Acute hemodynamic effects of pacing in patients with Fontan physiology: a prospective study. J Am Coll Cardiol 46(10):1937–1942. https://doi.org/10.1016/j.jacc.2005.07.045

Article  PubMed  Google Scholar 

Hasselman T, Schneider D, Madan N, Jacobs M (2005) Reversal of fenestration flow during ventricular systole in Fontan patients in junctional or ventricular paced rhythm. Pediatr Cardiol 26(5):638–641. https://doi.org/10.1007/s00246-005-0879-6

Article  CAS  PubMed  Google Scholar 

Alnoor M, Burch G, Armsby L, Batra A, Balaji S (2022) Hemodynamic impact of atrial pacing in patients with Fontan physiology and junctional rhythm: a cardiac catheterization study. Pediatr Cardiol 43(3):508–514. https://doi.org/10.1007/s00246-021-02747-2

Article  PubMed  Google Scholar 

Gewillig M, Brown SC (2016) The Fontan circulation after 45 years: update in physiology. Heart 102(14):1081–1086. https://doi.org/10.1136/heartjnl-2015-307467

Article  PubMed  Google Scholar 

Gewillig M, Brown SC, van de Bruaene A, Rychik J (2020) Providing a framework of principles for conceptualising the Fontan circulation. Acta Paediatr 109(4):651–658. https://doi.org/10.1111/apa.15098

Article  PubMed  PubMed Central  Google Scholar 

Van De Bruaene A, Claessen G, Salaets T, Gewillig M (2022) Late Fontan circulatory failure. What drives systemic venous congestion and low cardiac output in adult Fontan patients? Frontiers Cardiovasc Med 9:825. https://doi.org/10.3389/fcvm.2022.825472

Article  Google Scholar 

Rychik J, Fogel MA, Donofrio MT et al (1997) Comparison of patterns of pulmonary venous blood flow in the functional single ventricle heart after operative aortopulmonary shunt versus superior cavopulmonary shunt. Am J Cardiol 80(7):922–926. https://doi.org/10.1016/s0002-9149(97)00546-8

Article  CAS  PubMed  Google Scholar 

Ta HT, Alsaied T, Steele JM et al (2020) Atrial function and its role in the non-invasive evaluation of diastolic function in congenital heart disease. Pediatr Cardiol 41(4):654–668. https://doi.org/10.1007/s00246-020-02351-w

Article  PubMed  Google Scholar 

Ferrari I, Shehu N, Stern H, Meierhofer C (2023) Junctional rhythm produces retrograde flow in the whole Fontan system: cardiac magnetic resonance-derived flow documentation—a case report. Eur Heart J Case Rep 7(3):126. https://doi.org/10.1093/ehjcr/ytad126

Article  Google Scholar 

Barber G, Di Sessa T, Child JS, Perloff JK, Laks H, George BL, Williams RG (1988) Hemodynamic responses to isolated increments in heart rate by atrial pacing after a Fontan procedure. Am Heart J 115(4):837–841. https://doi.org/10.1016/0002-8703(88)90887-3

Article  CAS  PubMed  Google Scholar 

Paridon SM, Karpawich PP, Pinsky WW (1993) The effects of rate responsive pacing on exercise performance in the postoperative univentricular heart. Pacing Clin Electrophysiol 16(6):1256–1262. https://doi.org/10.1111/j.1540-8159.1993.tb01712.x

Article  CAS  PubMed  Google Scholar