Hostname: page-component-78c5997874-t5tsf Total loading time: 0 Render date: 2024-11-17T18:24:03.622Z Has data issue: false hasContentIssue false
  • English
  • Français

Outcomes of adults with repaired tetralogy of Fallot from the national Scottish Cohort

Published online by Cambridge University Press:  24 February 2021

Richard J. Dobson ,
Nitish Ramparsad ,
Niki L. Walker ,
Alex McConnachie  and
Mark H. D. Danton Open the ORCID record for Mark H. D. Danton [Opens in a new window]
Richard J. Dobson
Affiliation:
Scottish Adult Congenital Services, Golden Jubilee National Hospital, Glasgow, Scotland, UK
Nitish Ramparsad
Affiliation:
Robertson Centre for Biostatistics, University of Glasgow, Glasgow, Scotland, UK
Niki L. Walker
Affiliation:
Scottish Adult Congenital Services, Golden Jubilee National Hospital, Glasgow, Scotland, UK
Alex McConnachie
Affiliation:
Robertson Centre for Biostatistics, University of Glasgow, Glasgow, Scotland, UK
Mark H. D. Danton*
Affiliation:
Scottish Adult Congenital Services, Golden Jubilee National Hospital, Glasgow, Scotland, UK
*
Author for correspondence: M. Danton, Department of the Scottish Adult Congenital Service, Golden Jubilee National Hospital, Agamemnon St, Clydebank, ScotlandG81 4DY, UK. Tel: +44 141 201 000; Fax: +44 141 201 8544. E-mail: Mark.Danton@glasgow.ac.uk
Get access Rights & Permissions [Opens in a new window]

Abstract

Background:

The adult population of repaired tetralogy of Fallot is increasing and at risk of pre-mature death and arrhythmia. This study evaluates risk factors for adverse outcome and the effect of pulmonary valve replacement within a national cohort.

Methods:

A retrospective cohort study of 341 adult repaired tetralogy of Fallot (16–72 years) managed through a single national service was undertaken incorporating over 1200 patient-years of follow-up. Demographics, cardiopulmonary exercise testing, cardiac magnetic resonance, reintervention (including pulmonary valve replacement), and clinical events were analysed. The influence of these parameters on a primary outcome (death or arrhythmia) was evaluated.

Results:

Compared with an age-/gender-matched population, patients experienced a reduced survival, particularly males over 55 years (standardised mortality ratio : 6.12, 95% CI: 1.64–15.66, p = 0.004). Cox proportional hazards modelling identified increased indexed right ventricle (RV) end-diastolic volume (hazard ratio (HR): 2.86, 95% CI: 1.4–5.85, p = 0.004) and female gender (HR (male): 0.37, 95% CI: 0.14–0.98, p = 0.045) to be predictors significantly associated with the primary outcome. Pulmonary valve replacement undertaken at indexed RV end-diastolic volume = 145 ml/m2 reduced RV volumes and QRS duration but did not improve cardiopulmonary exercise testing nor NYHA class. Pulmonary valve replacement during cohort period was associated with increased risk of primary outcome (HR: 2.82, 95% CI: 1.36–5.86, p = 0.005).

Conclusions:

Although the majority of adult tetralogy of Fallot were asymptomatic in NYHA 1, cardiopulmonary exercise testing revealed important deficits. Tetralogy of Fallot survival was reduced compared to the general population. Female gender and increasing RV end-diastolic volume predicted adverse events. Pulmonary valve replacement reduced RV volumes and QRS duration but did not improve primary outcome.

Keywords

TetralogyFallotadultcongenitalheart
Type
Original Article
Information
Cardiology in the Young , Volume 31 , Issue 8 , August 2021 , pp. 1306 - 1314
Check for updates
Copyright
© The Author(s), 2021. Published by Cambridge University Press

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Park, CS, Lee, JR, Lim, H-G, Kim, W-H, Kim, YJ. The long-term result of total repair for tetralogy of Fallot. Eur J Cardiothorac Surg 2010; 38: 311–317.CrossRefGoogle Scholar
Gatzoulis, MA, Balaji, S, Webber, SA, et al. Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study. Lancet 2000; 356: 975–981.CrossRefGoogle ScholarPubMed
Knauth, AL, Gauvreau, K, Powell, AJ, et al. Ventricular size and function assessed by cardiac MRI predict major adverse clinical outcomes late after tetralogy of Fallot repair. Heart 2008; 94: 211–216.10.1136/hrt.2006.104745CrossRefGoogle ScholarPubMed
Valente, AM, Gauvreau, K, Assenza, GE, et al. Contemporary predictors of death and sustained ventricular tachycardia in patients with repaired tetralogy of Fallot enrolled in the INDICATOR cohort. Heart 2014; 100: 247–253.CrossRefGoogle ScholarPubMed
Diller, G-P, Kempny, A, Liodakis, E, et al. Left ventricular longitudinal function predicts life-threatening ventricular arrhythmia and death in adults with repaired tetralogy of Fallot. Circulation 2012; 125: 2440–2446.CrossRefGoogle ScholarPubMed
Ghai, A, Silversides, C, Harris, L, Webb, GD, Sui, SC, Therrien, J. Left ventricular dysfunction is a risk factor for sudden cardiac death in adults late after repair of tetralogy of Fallot. J Am Coll Cardiol 2002; 40: 1675–1680.CrossRefGoogle ScholarPubMed
Geva, T, Sandweiss, BM, Gauvreau, K, Gauvreau, K, Lock, JE, Powell, AJ. Factors associated with impaired clinical status in long-term survivors of tetralogy of Fallot repair evaluated by magnetic resonance imaging. JACC 2004; 43: 1068–1074.CrossRefGoogle Scholar
Egbe, AC, Kothapalli, S, Borlaug, BA, et al. Mechanism and risk factors for death in adults with tetralogy of Fallot. Am J Cardiol 2019; 124: 803−807.CrossRefGoogle Scholar
Stout, KK, Daniels, CJ, Baoulhosn, 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: e698–e800. doi: 10.1161/CIR.0000000000000603.Google Scholar
Geva, T, Mulder, B, Gauvreau, K, et al. Preoperative predictors of death and sustained ventricular tachycardia after pulmonary valve replacement in patients with repaired tetralogy of Fallot enrolled in the INDICATOR cohort. Circulation 2018; 138; 2106–2115. doi: 10.1161/CIRCULATIONAHA.118.034740.CrossRefGoogle ScholarPubMed
Baumgartner, H, Bonhoeffer, P, DeGroot, NMS, et al. ESC Guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J 2010; 31: 2915–2957.CrossRefGoogle Scholar
Le Ven, F, Bibeau, K, De Larochelliere, E, et al. Cardiac morphology and function reference values derived from a large subset of healthy young Caucasian adults by magnetic resonance imaging. Eur Heart J – Cardiovasc Imaging 2016; 17: 981–990.CrossRefGoogle ScholarPubMed
Wasserman, K. Principles of Exercise Testing and Interpretation: Including Pathophysiology and Clinical Applications, 4th ed. Lippincott Williams & kins, Philadelphia, 2005.Google Scholar
Clark, AL, Poole-Wilson, PA, Coats, AJS. Relation between ventialtion and carbon dioxide production in patients with chronic heart failure. JACC 1992; 20: 1326–1332. CrossRefGoogle Scholar
Korn, EL, Graubard, BI, Midthune, D. Time-to-event analysis of longitudinal follow-up of a survey: choice of the time-scale. Am J Epidemiol 1997; 145: 72–80.Google ScholarPubMed
López-Ratón, M, Rodríguez-Álvarez, MX, Cadarso-Suárez, C, Gude-Sampedro, F. OptimalCutpoints: an R package for selecting optimal cutpoints in diagnostic tests. J Stat Software 2014; 61: 1–36.CrossRefGoogle Scholar
Finkelstein, D, Muzikansky, A, Schoenfeld, D. Comparing survival of a sample to that of a standard population. J Natl Cancer Inst 2003; 95: 1434–1439.CrossRefGoogle ScholarPubMed
Kahn, HA, Sempos, CT. Statistical Methods in Epidemiology. Oxford University Press, Oxford, 1989.Google Scholar
R Core Team. R: A Language and Environment for Statistical Computing Vienna, R Foundation for Statistical Computing, Austria, 2017.Google Scholar
Shen, Y, Zhang, X, Ma, W, et al. VE/VCO2 slope and its prognostic value in patients with chronic heart failure. Exp Ther Med 2015; 9: 1407–1412.CrossRefGoogle ScholarPubMed
Rapp, D, Scharhag, J, Wagenpfeil, S, Scholl, J. Reference values for peak oxygen uptake: cross-sectional analysis of cycle ergometry-based cardiopulmonary exercise tests of 10 090 adult German volunteers from the Prevention First Registry. BMJ Open 2018; 8: e018697. doi: 10.1136/bmjopen-2017-018697.CrossRefGoogle ScholarPubMed
Petersen, SE, Aung, N, Sanghvi, MM, et al. Reference ranges for cardiac structure and function using cardiovascular magnetic resonance (CMR) in Caucasians from the UK Biobank population cohort. J Cardiovasc Magn Reson 2017; 19: 1–19. doi: 10.1186/s12968-017-0327-9.CrossRefGoogle ScholarPubMed
Kawel-Boehm, N, Maceira, A, Valsanggiacomo, ER, et al. Normal values for cardiovascular magnetic resonance in adults and children J Cardiovasc Magn Reson 2015; 17: 29. doi: 10.1186/s12968-015-0111-7.CrossRefGoogle ScholarPubMed
Quail, MA, Frigiola, A, Giardini, A, et al. Impact of pulmonary valve replacement in tetralogy of Fallot with pulmonary regurgitation: a comparison of intervention and nonintervention. Ann Thorac Surg 2012; 94: 1619–1626.CrossRefGoogle ScholarPubMed
Frigiola, A, Hughes, M, Turner, M, et al. Physiological and phenotypic characteristics of late survivors of tetralogy of Fallot repair who are free from pulmonary valve replacement. Circulation 2013; 128: 1861–1868.CrossRefGoogle ScholarPubMed
Kempny, A, Dimopoulos, K, Uebing, A, et al. Reference values for exercise limitations among adults with congenital heart disease. Relation to activities of daily life—single centre experience and review of published data. Eur Heart J 2012; 33: 1386–1396.CrossRefGoogle ScholarPubMed
Inuzuki, R, Diller, G-P, Borgia, F, et al. Comprehensive use of cardiopulmonary exercise testing identifies adults with congenital heart disease at increased mortality risk in the medium term. Circulation 2012; 125: 250–259.CrossRefGoogle Scholar
Arena, R, Sietsema, KE. Cardiopulmonary exercise testing in the clinical evaluation of patients with heart and lung disease. Circulation 2011: 123; 668–680.CrossRefGoogle ScholarPubMed
Gitt, AK, Wasserman, K, Kilowski, C, et al. Exercise Anaerobic threshold and ventilatory efficiency identify heart failure patients for high risk of early death. Circulation 2002; 106: 3079–3084.CrossRefGoogle ScholarPubMed
Mouws, EMJP, Roos-Hesselink, JW, Bogers, AJJC, de Groot, NMS. Coexistence of tachyarrhythmias in patients with Tetralogy of Fallot. Heart Rhythm 2018; 15: 503–511.CrossRefGoogle ScholarPubMed
Bokma, JP, Winter, MM, Vehmeijer, JT, et al. QRS fragmentation is superior to QRS duration in predicting mortality in adults with Tetralogy of Fallot. Heart 2017; 103: 666–671.CrossRefGoogle ScholarPubMed
Diller, G-P, Kempny, A, Alonso-Gonzalez, R, et al. Survival prospects and circumstances of death in contemporary adult congenital heart disease patients under follow-up at a large tertiary centre. Circulation 2015; 132: 2118–2125.CrossRefGoogle Scholar
O’Byrne, ML, Glatz, AC, Mercer-Rosa, L, et al. Trends in pulmonary valve replacement in children and adults with Tetralogy of Fallot. Am J Cardiol 2015; 115: 118–124.CrossRefGoogle ScholarPubMed
Egbe, AC, Vallabhajosyula, S, Connolly, HM, Trends and outcomes of pulmonary valve replacement in tetralogy of Fallot. Int J Cardiol 2020; 299: 136–139.10.1016/j.ijcard.2019.07.063CrossRefGoogle Scholar
Tretter, JT, Reddington, AN. Risk factors and biomarkers of poor outcomes. Time to throw out right ventricular volumes in repaired Tetralogy of Fallot? Lessons from the INDICATOR cohort. Circulation 2018; 138: 2116–2118.CrossRefGoogle ScholarPubMed
Geva, T. Indications for pulmonary valve replacement in repaired tetralogy of Fallot the quest continues Circulation 2013; 128: 1855–1857.CrossRefGoogle ScholarPubMed
Greutmann, M, Tetralogy of Fallot, pulmonary valve replacement and right ventricular volumes: are we chasing the right target. Eur Heart J 2016; 37: 836–839.CrossRefGoogle Scholar
Bokma, JP, Winter, MM, Oosterhof, T, et al. Preoperative thresholds for mid-to-late haemodynamic and clinical outcomes after pulmonary valve replacement in tetralogy of Fallot. Eur Heart J 2016; 37: 829–835. doi: 10.1093/eurheartj/ehv550.CrossRefGoogle ScholarPubMed
Oosterhof, T, van Straten, A, Vliegen, HW, et al. Preoperative thresholds for pulmonary valve replacement in patients with corrected tetralogy of Fallot using cardiovascular magnetic resonance. Circulation 2007; 116: 545–551.CrossRefGoogle ScholarPubMed
Heng, EL, Gatzoulis, MA, Uebing, A, et al. Immediate and midterm cardiac remodeling after surgical pulmonary valve replacement in adults with repaired tetralogy of Fallot. Circulation 2017; 136: 1703–1713. doi: 10.1161/CIRCULATIONAHA.117.027402.CrossRefGoogle ScholarPubMed
Geva, T, Gauvreau, K, Powell, AJ, et al. Randomized trial of pulmonary valve replacement with and without right ventricular remodeling surgery. Circulation 2010; 122: S201–S208.CrossRefGoogle ScholarPubMed
Cheung, EW, Wong, WH, Cheung, YF, Meta-analysis of pulmonary valve replacement after operative repair of tetralogy of Fallot. Am J Cardiol 2010; 106: 552–557.CrossRefGoogle ScholarPubMed
Gengsakul, A, Harris, L, Bradley, TJ, et al. The impact of pulmonary valve replacement after tetralogy of Fallot repair: a matched comparison. Eur J Cardiothorac Surg 2007; 32: 462–468.CrossRefGoogle Scholar
Ferraz Cavalcanti, PE, Oliveira Sá, MPB, Santos, CA, 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: 2227–2243.CrossRefGoogle ScholarPubMed
Frigiola, A, Tsang, V, Bull, C, et al. Biventricular response after pulmonary valve replacement for right ventricular outflow tract dysfunction. Is age a predictor of outcome? Circulation 2008; 118(suppl 1): S182–S190.CrossRefGoogle Scholar
Harrild, DM, Berul, CI, Cecchin, F, et al. Pulmonary valve replacement in tetralogy of Fallot impact on survival and ventricular tachycardia Circulation 2009; 119: 445–451.10.1161/CIRCULATIONAHA.108.775221CrossRefGoogle ScholarPubMed
Bokma, JP, Geva, T, Sleeper, LA, et al. A propensity score-adjusted analysis of clinical outcomes after pulmonary valve replacement in tetralogy of Fallot. Heart 2018; 104: 738–744.CrossRefGoogle ScholarPubMed
Caldaroni, F, Lo Rito, M, Chessa, M, et al. Surgical ablation of ventricular tachycardia in patients with repaired tetralogy of Fallot. Eur J Cardiothorac Surg 2019; 55: 845–850.CrossRefGoogle Scholar
Therrien, J, Siu, SC, Harris, L, et al. Impact of pulmonary valve replacement on arrhythmia propensity late after repair of tetralogy of Fallot. Circulation 2001; 103: 2489–2494.CrossRefGoogle ScholarPubMed
Rotes, AS, Eidem, BW, Connolly, HM, et al. Long-term follow-up after pulmonary valve replacement in repaired tetralogy of Fallot. Am J Cardiol 2014; 114: 901–908.CrossRefGoogle Scholar
Khairy, P, Aboulhosn, J, Gurvitz, MZ, et al. For the alliance for adult research in congenital cardiology (AARCC). Arrhythmia burden in adults with surgically repaired Tetralogy of Fallot. Circulation 2010; 122: 868–875.CrossRefGoogle ScholarPubMed