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Modelling right and left ventricular adaptation over time after tetralogy of Fallot repair and the need for pulmonary valve replacement in adolescence
Published online by Cambridge University Press: 28 July 2025
Abstract
Background:
Progressive ventricular remodelling in children with repaired tetralogy of Fallot may or may not result in the need for pulmonary valve replacement. We aimed to model and compare the rates of right and left ventricular adaptation over time, as assessed by cardiac MRI after surgical repair of tetralogy of Fallot, in children who did or did not require pulmonary valve replacement later in adolescence.
Methods:
Single-centre, retrospective cohort study from 2000 to 2020 including patients with tetralogy of Fallot who had complete surgical repair before 24 months.
Results:
From 214 patients included in this analysis, 142 (66.3%) had pulmonary valve replacement at a median age of 12 years (interquartile range 9–15.5) during follow-up. Assessing 323 cardiac MRI studies from 201 patients commencing from a median age of 9.4 years (interquartile range 5.9–12.3), the group that required pulmonary valve replacement later during the follow-up had a steeper time-related right ventricular dilation trajectory than non-pulmonary valve replacement patients: the increment in right ventricular end-diastolic volume index was 19.4 versus 2.8 ml/m2/log2year, P < 0.001; also, right ventricular end-systolic volume index incremented more quickly, at 11.9 versus 0.8 ml/m2/log2year, P < 0.001. Left ventricular end-diastolic volume index increased more quickly in patients who eventually had pulmonary valve replacement, at 7.2 versus 1.5 ml/m2/log2year, P = 0.005; the same occurred for indexed left ventricular end-systolic volume at 3.2 versus –0.4 ml/m2/log2year, P = 0.001.
Conclusion:
Early right and left ventricular dilation over time are identifiable by cardiac MRI in patients destined to require pulmonary valve replacement following tetralogy of Fallot repair.
Keywords
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- Original Article
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- © The Author(s), 2025. Published by Cambridge University Press
References
Geva, T. Indications for pulmonary valve replacement in repaired tetralogy of Fallot. Circulation 2013; 128 (
17
): 1855–1857.10.1161/CIRCULATIONAHA.113.005878CrossRefGoogle ScholarPubMed
Jp, B, T., G, La, S, et al. A propensity score-adjusted analysis of clinical outcomes after pulmonary valve replacement in tetralogy of Fallot. Heart Br Card Soc 2018; 104 (
9
): 738–744.Google Scholar
Jg, M, Bj, G, Dd, M, et al. Long-term outcome in patients undergoing surgical repair of tetralogy of Fallot. N Engl J Med 1993; 329 (
9
): 593–599.Google Scholar
Stout, KK, Daniels, CJ, Aboulhosn, JA, et al. 2018 AHA/ACC guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association task force on clinical practice guidelines. Circulation 2019; 139 (
14
): e698–e800.Google Scholar
Ma, G, S., B, Sa, W, et al. Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study. Lancet Lond Engl 2000; 356 (
9234
): 975–981.Google Scholar
Sasson, L, Houri, S, Raucher Sternfeld, A, et al. Right ventricular outflow tract strategies for repair of tetralogy of Fallot: effect of monocusp valve reconstruction. Eur J Cardio-Thorac Surg Off J Eur Assoc Cardio-Thorac Surg 2013; 43 (
4
): 743–751.10.1093/ejcts/ezs479CrossRefGoogle ScholarPubMed
Andrade, AC, Jerosch-Herold, M, Wegner, P, et al. Determinants of left ventricular dysfunction and remodeling in patients with corrected tetralogy of Fallot. J Am Heart Assoc Cardiovasc Cerebrovasc Dis 2019; 8 (
17
): e009618.10.1161/JAHA.118.009618CrossRefGoogle ScholarPubMed
Li, SN, Yu, W, Lai, CTM, Wong, SJ, Cheung, YF. Left ventricular mechanics in repaired tetralogy of Fallot with and without pulmonary valve replacement: analysis by three-dimensional speckle tracking echocardiography. PloS One 2013; 8 (
11
): e78826.10.1371/journal.pone.0078826CrossRefGoogle ScholarPubMed
Geva, T. MRI is the preferred method for evaluating right ventricular size and function in patients with congenital heart disease. Circ Cardiovasc Imaging 2014; 7 (
1
): 190–197.10.1161/CIRCIMAGING.113.000553CrossRefGoogle Scholar
Pe, FC, Mp, S, Ca, S, et al. Pulmonary valve replacement after operative repair of tetralogy of Fallot: meta-analysis and meta-regression of 3,118 patients from 48 studies. J Am Coll Cardiol 2013; 62 (
23
): 2227–2243.Google Scholar
Dm, H, Ci, B, C., F, et al. Pulmonary valve replacement in tetralogy of Fallot: impact on survival and ventricular tachycardia. Circulation 2009; 119 (
3
): 445–451.Google Scholar
Marelli, A, Beauchesne, L, Colman, J, et al. Canadian Cardiovascular Society 2022 guidelines for cardiovascular interventions in adults with congenital heart disease. Can J Cardiol 2022; 38 (
7
): 862–896.Google Scholar
Zoghbi, WA, Adams, D, Bonow, RO, et al. Recommendations for noninvasive evaluation of native valvular regurgitation: a report from the American Society of Echocardiography developed in collaboration with the society for cardiovascular magnetic resonance. J Am Soc Echocardiogr 2017; 30 (
4
): 303–371.10.1016/j.echo.2017.01.007CrossRefGoogle ScholarPubMed
Feltes, TF, Bacha, E, Beekman, RH, et al. Indications for cardiac catheterization and intervention in pediatric cardiac disease: a scientific statement from the American Heart Association. Circulation 2011; 123 (
22
): 2607–2652.10.1161/CIR.0b013e31821b1f10CrossRefGoogle ScholarPubMed
King, ME, Braun, H, Goldblatt, A, Liberthson, R, Weyman, AE. Interventricular septal configuration as a predictor of right ventricular systolic hypertension in children: a cross-sectional echocardiographic study. Circulation 1983; 68 (
1
): 68–75.10.1161/01.CIR.68.1.68CrossRefGoogle ScholarPubMed
Hagdorn, QAJ, Beurskens, NEG, Gorter, TM, et al. Sex differences in patients with repaired tetralogy of Fallot support a tailored approach for males and females: a cardiac magnetic resonance study. Int J Cardiovasc Imaging 2020; 36 (
10
): 1997–2005.10.1007/s10554-020-01900-xCrossRefGoogle ScholarPubMed
Mertens, LL. Right ventricular remodelling after tetralogy of Fallot repair: new insights from longitudinal follow-up data. Eur Heart J - Cardiovasc Imaging 2017; 18 (
3
): 371–372.10.1093/ehjci/jew299CrossRefGoogle ScholarPubMed
Bhat, M, Mercer-Rosa, L, Fogel, MA, et al. Longitudinal changes in adolescents with TOF: implications for care. Eur Heart J Cardiovasc Imaging 2017; 18 (
3
): 356–363.10.1093/ehjci/jew272CrossRefGoogle ScholarPubMed
Blais, S, Marelli, A, Vanasse, A, et al. Comparison of long-term outcomes of valve-sparing and transannular patch procedures for correction of tetralogy of Fallot. JAMA Netw Open 2021; 4 (
7
): e2118141.10.1001/jamanetworkopen.2021.18141CrossRefGoogle ScholarPubMed
Cocomello, L, Sinha, S, Gonzalez Corcia, MC, Baquedano, M, Benedetto, U, Caputo, M. Determinants of QRS duration in patients with tetralogy of Fallot after pulmonary valve replacement. J Card Surg 2021; 36 (
6
): 1958–1968.10.1111/jocs.15469CrossRefGoogle ScholarPubMed
Buddhe, S, Shah, A, Lai, WW. Progression of right ventricular dilation in repaired tetralogy of Fallot. J Magn Reson Imaging JMRI 2015; 41 (
3
): 730–737.10.1002/jmri.24610CrossRefGoogle ScholarPubMed
Wijesekera, VA, Raju, R, Precious, B, et al. Sequential right and left ventricular assessment in posttetralogy of Fallot patients with significant pulmonary regurgitation. Congenit Heart Dis 2016; 11 (
6
): 606–614.10.1111/chd.12354CrossRefGoogle ScholarPubMed
Greutmann, M, Ruperti, J, Schwitz, F, et al. High variability of right ventricular volumes and function in adults with severe pulmonary regurgitation late after tetralogy of Fallot repair. Am J Cardiol 2022; 166: 88–96.10.1016/j.amjcard.2021.11.022CrossRefGoogle ScholarPubMed
T., R, F., G, M., C, et al. Evolution of right ventricular size over time after tetralogy of Fallot repair: a longitudinal cardiac magnetic resonance study. Eur Heart J Cardiovasc Imaging 2017; 18 (
3
): 364–370.Google Scholar
Latus, H, Stammermann, J, Voges, I, et al. Impact of right ventricular pressure load after repair of tetralogy of Fallot. J Am Heart Assoc Cardiovasc Cerebrovasc Dis 2022; 11 (
7
): e022694.10.1161/JAHA.121.022694CrossRefGoogle ScholarPubMed
van der Ven, JPG, Sadighy, Z, Valsangiacomo Buechel, ER, et al. Multicentre reference values for cardiac magnetic resonance imaging derived ventricular size and function for children aged 0–18 years. Eur Heart J - Cardiovasc Imaging 2020; 21 (
1
): 102–113.10.1093/ehjci/jez164CrossRefGoogle ScholarPubMed
Gnanappa, GK, Celermajer, DS, Zhu, D, Puranik, R, Ayer, J. Severe right ventricular dilatation after repair of tetralogy of Fallot is associated with increased left ventricular preload and stroke volume. Eur Heart J Cardiovasc Imaging 2019; 20 (
9
): 1020–1026.10.1093/ehjci/jez035CrossRefGoogle ScholarPubMed
Davlouros, PA, Kilner, PJ, Hornung, TS, et al. Right ventricular function in adults with repaired tetralogy of Fallot assessed with cardiovascular magnetic resonance imaging. J Am Coll Cardiol 2002; 40 (
11
): 2044–2052.10.1016/S0735-1097(02)02566-4CrossRefGoogle ScholarPubMed
Khalaf, A, Tani, D, Tadros, S, Madan, S. Right- and left-ventricular strain evaluation in repaired pediatric tetralogy of Fallot patients using magnetic resonance tagging. Pediatr Cardiol 2013; 34 (
5
): 1206–1211.10.1007/s00246-013-0631-6CrossRefGoogle ScholarPubMed
Kempny, A, Diller, GP, Orwat, S, et al. Right ventricular-left ventricular interaction in adults with tetralogy of Fallot: a combined cardiac magnetic resonance and echocardiographic speckle tracking study. Int J Cardiol 2012; 154 (
3
): 259–264.10.1016/j.ijcard.2010.09.031CrossRefGoogle ScholarPubMed
Luijnenburg, SE, Helbing, WA, Moelker, A, et al. 5-year serial follow-up of clinical condition and ventricular function in patients after repair of tetralogy of Fallot. Int J Cardiol 2013; 169 (
6
): 439–444.10.1016/j.ijcard.2013.10.013CrossRefGoogle ScholarPubMed
Baumgartner, H, De Backer, J, Babu-Narayan, SV, et al. 2020 ESC guidelines for the management of adult congenital heart disease. Eur Heart J 2021; 42 (
6
): 563–645.10.1093/eurheartj/ehaa554CrossRefGoogle ScholarPubMed
Goo, HW. Changes in right ventricular volume, volume load, and function measured with cardiac computed tomography over the entire time course of tetralogy of Fallot. Korean J Radiol 2019; 20 (
6
): 956–966.10.3348/kjr.2018.0891CrossRefGoogle ScholarPubMed
Buechel, ERV, Dave, HH, Kellenberger, CJ, et al. Remodelling of the right ventricle after early pulmonary valve replacement in children with repaired tetralogy of Fallot: assessment by cardiovascular magnetic resonance. Eur Heart J 2005; 26 (
24
): 2721–2727.10.1093/eurheartj/ehi581CrossRefGoogle ScholarPubMed
Ruperti-Repilado, FJ, Haag, N, Fischer, T, et al. Impact of pulmonary valve replacement on ventricular function and cardiac events in patients with tetralogy of Fallot. A retrospective cohort study. Rev Esp Cardiol Engl Ed 2024; 77: 408–417.10.1016/j.recesp.2023.11.010CrossRefGoogle ScholarPubMed
Sabate Rotes, A, Eidem, BW, Connolly, HM, et al. Long-term follow-up after pulmonary valve replacement in repaired tetralogy of Fallot. Am J Cardiol 2014; 114 (
6
): 901–908.10.1016/j.amjcard.2014.06.023CrossRefGoogle ScholarPubMed
Loss et al. supplementary material
Loss et al. supplementary material
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