Risk of cardiac tachyarrhythmia in patients with repaired tetralogy of Fallot: a multicenter cardiac MRI based study

  • Niek E. G. BeurskensEmail author
  • Quint A. J. Hagdorn
  • Thomas M. Gorter
  • Rolf M. F. Berger
  • Karin M. Vermeulen
  • Joost P. van Melle
  • Tjark E. Ebels
  • George K. Lui
  • Scott R. Ceresnak
  • Frandics P. Chan
  • Tineke P. Willems
Original Paper


Cardiac tachyarrhythmias are the leading cause of morbidity and mortality in patients with repaired tetralogy of Fallot (TOF). We evaluated risk factors for sustained ventricular tachyarrhythmia (VT) and atrial tachyarrhythmia (ATA) in these patients. Patients (n = 319) who underwent cardiac magnetic resonance (CMR) imaging at two tertiary centers between 2007 and 2016 were assessed. Potential risk markers, based on history, cardiac magnetic resonance imaging (CMR), electrocardiography (ECG) and echocardiography, were analyzed for prediction of the primary endpoint of VT, and the secondary endpoint of ATA. During a follow-up of 3.5 (0.9–6.1) years, 20 (6.3%) patients reached the primary endpoint, and 30 (9.4%) the secondary endpoint. Multivariable cox hazards regression identified right ventricular (RV) end-diastolic volume (Hazard ratio [HR] 2.03, per 10 ml/m2 increase; p = 0.02), RV end-systolic volume (HR 3.04, per 10 ml/m2 increase; p = 0.04), RV mass (HR 1.88, per 10 g/m2 increase; p = 0.02), and RV ejection fraction (HR 6.06, per 10% decrease; p = 0.02) derived from CMR to be independent risk factors of VT. In addition, QRS-duration (HR 1.70, per 10 ms increase; p = 0.001) and body mass index (BMI: HR 1.8, per 5 kg/m2 increase; p = 0.02) were independent markers of VT. Older age at TOF repair (HR 1.33, per 2 months increase; p = 0.03) and BMI (HR 1.76, per 5 kg/m2 increase; p < 0.001) independently predicted ATA. RV systolic dysfunction, hypertrophy and dilatation on CMR, together with QRS prolongation, and obesity are predictive of VT in TOF patients. Older age at TOF repair and obesity were associated with the occurrence of ATA.


Tetralogy of Fallot Cardiac magnetic resonance imaging Cardiac tachyarrhythmia Risk stratification ICD implantation 


Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.


  1. 1.
    Bacha EA, Scheule AM, Zurakowski D, Erickson LC, Hung J, Lang P et al (2001) Long-term results after early primary repair of tetralogy of Fallot. J Thorac Cardiovasc Surg 122(1):154–161CrossRefGoogle Scholar
  2. 2.
    Chiu SN, Wang JK, Chen HC, Lin MT, Wu ET, Chen CA et al (2012) Long-term survival and unnatural deaths of patients with repaired tetralogy of Fallot in an Asian cohort. Circ Cardiovasc Qual Outcomes 5(1):120–125CrossRefGoogle Scholar
  3. 3.
    Hickey EJ, Veldtman G, Bradley TJ, Gengsakul A, Manlhiot C, Williams WG et al (2009) Late risk of outcomes for adults with repaired tetralogy of Fallot from an inception cohort spanning four decades. Eur J Cardiothorac Surg 35(1):156–164; discussion 164CrossRefGoogle Scholar
  4. 4.
    Geva T, Sandweiss BM, Gauvreau K, Lock JE, Powell AJ (2004) Factors associated with impaired clinical status in long-term survivors of tetralogy of Fallot repair evaluated by magnetic resonance imaging. J Am Coll Cardiol 43(6):1068–1074CrossRefGoogle Scholar
  5. 5.
    Khairy P, Aboulhosn J, Gurvitz MZ, Opotowsky AR, Mongeon FP, Kay J et al (2010) Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: a multi-institutional study. Circulation 122(9):868–875CrossRefGoogle Scholar
  6. 6.
    Khairy P, Dore A, Poirier N, Marcotte F, Ibrahim R, Mongeon FP et al (2009) Risk stratification in surgically repaired tetralogy of Fallot. Expert Rev Cardiovasc Ther 7(7):755–762CrossRefGoogle Scholar
  7. 7.
    Valente AM, Gauvreau K, Assenza GE, Babu-Narayan SV, Schreier J, Gatzoulis MA et al (2014) Predictors of death and sustained ventricular tachycardia in patients with repaired tetralogy of Fallot enrolled in the INDICATOR cohort. Heart 100(3):247–253CrossRefGoogle Scholar
  8. 8.
    Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA et al (2008) ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J Am Coll Cardiol 52(23):e143–e263CrossRefGoogle Scholar
  9. 9.
    Mooij CF, de Wit CJ, Graham DA, Powell AJ, Geva T (2008) Reproducibility of MRI measurements of right ventricular size and function in patients with normal and dilated ventricles. J Magn Reson Imaging 28(1):67–73CrossRefGoogle Scholar
  10. 10.
    Geva T (2011) Repaired tetralogy of Fallot: the roles of cardiovascular magnetic resonance in evaluating pathophysiology and for pulmonary valve replacement decision support. J Cardiovasc Magn Reson 13:9CrossRefGoogle Scholar
  11. 11.
    Jaspers K, Freling HG, van Wijk K, Romijn EI, Greuter MJ, Willems TP (2013) Improving the reproducibility of MR-derived left ventricular volume and function measurements with a semi-automatic threshold-based segmentation algorithm. Int J Cardiovasc Imaging 29(3):617–623CrossRefGoogle Scholar
  12. 12.
    Gorter TM, van Melle JP, Freling HG, Ebels T, Bartelds B, Pieper PG (2015) Pulmonary regurgitant volume is superior to fraction using background-corrected phase contrast MRI in determining the severity of regurgitation in repaired tetralogy of Fallot. Int J Cardiovasc Imaging 31(6):1169–1177CrossRefGoogle Scholar
  13. 13.
    Haycock GB, Schwartz GJ, Wisotsky DH (1978) Geometric method for measuring body surface area: a height-weight formula validated in infants, children, and adults. J Pediatr 93(1):62–66CrossRefGoogle Scholar
  14. 14.
    Baumgartner H, Bonhoeffer P, Groot de NMS, Haan de F, Deanfield JE, Nazzareno G (2010) ESC Guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J 31:2915–2957CrossRefGoogle Scholar
  15. 15.
    Folino AF, Daliento L (2005) Arrhythmias after tetralogy of fallot repair. Indian Pacing Electrophysiol J 5(4):312–324Google Scholar
  16. 16.
    Kawut SM, Barr RG, Lima JA, Praestgaard A, Johnson WC, Chahal H et al (2012) Right ventricular structure is associated with the risk of heart failure and cardiovascular death: the Multi-Ethnic Study of Atherosclerosis (MESA)—right ventricle study. Circulation 126(14):1681–1688CrossRefGoogle Scholar
  17. 17.
    Gatzoulis MA, Balaji S, Webber SA, Siu SC, Hokanson JS, Poile C et al (2000) Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study. Lancet 356(9234):975–981CrossRefGoogle Scholar
  18. 18.
    Knauth AL, Gauvreau K, Powell AJ, Landzberg MJ, Walsh EP, Lock JE et al (2008) Ventricular size and function assessed by cardiac MRI predict major adverse clinical outcomes late after tetralogy of Fallot repair. Heart 94(2):211–216CrossRefGoogle Scholar
  19. 19.
    Bokma JP, de Wilde KC, Vliegen HW, van Dijk AP, van Melle JP, Meijboom FJ et al (2017) Value of cardiovascular magnetic resonance imaging in noninvasive risk stratification in tetralogy of Fallot. JAMA Cardiol 2(6):678–683CrossRefGoogle Scholar
  20. 20.
    Abel ED, Litwin SE, Sweeney G (2008) Cardiac remodeling in obesity. Physiol Rev 88(2):389–419CrossRefGoogle Scholar
  21. 21.
    Jouven X, Desnos M, Guerot C, Ducimetière P (1999) Predicting sudden death in the population: the Paris Prospective Study I. Circulation 99(15):1978–1983CrossRefGoogle Scholar
  22. 22.
    Wong CY, O’Moore-Sullivan T, Leano R, Hukins C, Jenkins C, Marwick TH (2006) Association of subclinical right ventricular dysfunction with obesity. J Am Coll Cardiol 47(3):611–616CrossRefGoogle Scholar
  23. 23.
    Stritzke J, Markus MR, Duderstadt S, Lieb W, Luchner A, Döring A et al (2009) The aging process of the heart: obesity is the main risk factor for left atrial enlargement during aging the MONICA/KORA (monitoring of trends and determinations in cardiovascular disease/cooperative research in the region of Augsburg) study. J Am Coll Cardiol 54(21):1982–1989CrossRefGoogle Scholar

Copyright information

© Springer Nature B.V. 2018

Authors and Affiliations

  • Niek E. G. Beurskens
    • 1
    Email author
  • Quint A. J. Hagdorn
    • 2
  • Thomas M. Gorter
    • 1
  • Rolf M. F. Berger
    • 2
  • Karin M. Vermeulen
    • 3
  • Joost P. van Melle
    • 1
  • Tjark E. Ebels
    • 4
  • George K. Lui
    • 5
  • Scott R. Ceresnak
    • 6
  • Frandics P. Chan
    • 7
  • Tineke P. Willems
    • 8
  1. 1.Department of Cardiology, University Medical Center GroningenUniversity of GroningenGroningenThe Netherlands
  2. 2.Center for Congenital Heart Diseases, Beatrix Children’s Hospital, University Medical Center GroningenUniversity of GroningenGroningenThe Netherlands
  3. 3.Department of Epidemiology, University Medical Center GroningenUniversity of GroningenGroningenThe Netherlands
  4. 4.Department of Cardiothoracic Surgery, University Medical Center GroningenUniversity of GroningenGroningenThe Netherlands
  5. 5.Departments of Medicine and Pediatrics, Divisions of Cardiovascular Medicine and Pediatric CardiologyStanford University School of MedicinePalo AltoUSA
  6. 6.Division of Pediatric CardiologyStanford University School of MedicinePalo AltoUSA
  7. 7.Department of Radiology, Stanford University Medical CenterStanford University School of MedicinePalo AltoUSA
  8. 8.Department of Radiology, University Medical Center GroningenUniversity of GroningenGroningenThe Netherlands

Personalised recommendations