Abstract
Sinus node dysfunction (SND) with junctional rhythm (JR) is common after the Fontan operation. Atrial pacing (AP) restores atrioventricular (AV) synchrony, but the placement of a pacemaker carries significant morbidity. To study the impact of AP on echocardiographic parameters of function in Fontan patients with SND and JR. Nine Fontan patients with AP for SND and JR were prospectively studied with echocardiography in the following conditions—baseline paced rhythm, underlying JR and, if possible, slow-paced rhythm below their baseline paced rate (~ 10 bpm faster than their JR rate). Cardiac index was significantly lower in JR (3 ± 1.1 L/min/m2) vs AP (4.2 ± 1.4 L/min/m2; p = 0.002). Diastolic function also significantly worsened with increased ratio of early diastolic systemic AV valve inflow velocity to early diastolic systemic AV valve annulus velocity (E/e′ ratio) by tissue Doppler imaging (TDI) in JR (11.6 ± 4.6) vs AP (8.8 ± 2.2, p = 0.016). Pulmonary venous flow reversal was present in 7/9 patients in JR vs 0/9 in AP (p = 0.016). There were no significant differences in these echocardiographic measurements between the paced and slow-paced conditions. When compared to AP, JR was associated with a significant reduction in cardiac output and diastolic function, and an increased prevalence of pulmonary vein flow reversal. There were no differences between paced and slow-paced conditions, suggesting that AV synchrony rather than heart rate was primarily contributing to cardiac output. Further studies are needed to understand the chronic impact of JR on Fontan outcomes.
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Abbreviations
- AP:
-
Atrial pacing, atrial paced and atrial pacemaker
- AV:
-
Atrioventricular
- Bpm:
-
Beats per minute
- E/e′ ratio:
-
Ratio of early diastolic systemic atrioventricular valve inflow velocity to early diastolic systemic atrioventricular valve annulus velocity
- IRB:
-
Institutional Review Board
- JR:
-
Junctional rhythm
- SND:
-
Sinus node dysfunction
- TDI:
-
Tissue Doppler imaging
References
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
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
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
Cohen MI, Wernovsky G, Vetter VL et al (1998) Sinus node function after a systematically staged Fontan procedure. Circulation 98(19 Suppl):352–358
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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None of the authors have a relationship with industry-relevant to this manuscript. Seshadri Balaji has a research grant from Medtronic to study sudden cardiac death in hypertrophic cardiomyopathy. Seshadri Balaji is a consultant for Milestone Pharmaceuticals and Alta Thera Pharmaceuticals. The other authors have no disclosures.
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Yang, E.L., Evers, P.D., Long, M.J. et al. Impact of Atrial Pacing in Fontan Patients with Junctional Rhythm: A Prospective Echocardiographic Study. Pediatr Cardiol 45, 361–367 (2024). https://doi.org/10.1007/s00246-023-03345-0
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DOI: https://doi.org/10.1007/s00246-023-03345-0