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Magnetic Resonance Imaging Risk Factors for Ventricular Arrhythmias in Tetralogy of Fallot

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Abstract

Sudden cardiac death (SCD) is the most common cause of late mortality in tetralogy of Fallot (TOF). Pulmonary regurgitation (PR) was previously found to be the most common hemodynamic abnormality associated with ventricular arrhythmias (VA), but cardiovascular magnetic resonance (CMR)-based studies did not show this association. The aim of this study is to investigate the risk factors for VA in TOF using CMR. Electronic records of TOF patients and their CMR studies between July 2006 and October 2018 in one center were retrospectively reviewed. Demographic, clinical and CMR data of patients were collected. Outcome was defined as sustained ventricular tachycardia (VT), aborted SCD and SCD. From a total of 434 TOF patients with complete CMR studies, 19 (4.4%) patients developed a positive outcome (12 sustained VT, 4 aborted SCD, 3 SCD) at a median age of 24 years. The number of surgical interventions was significantly greater in patients who developed VA. Right ventricular volumes were significantly larger in patients who suffered a positive outcome. Odds ratio for developing VA was 6.905 for RVEDVI ≥ 160 ml/m2 and 6.141 for RVESVI ≥ 80 ml/m2 (P = 0.0014 and 0.0012, respectively). Event-free survival was longer in patients with smaller right ventricular volumes. In conclusion, right ventricular dimensions are the most significant factors associated with the development of VA in TOF. The number of surgical interventions is also related to an increased risk.

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References

  1. Hoffman JI, Kaplan S (2002) The incidence of congenital heart disease. J Am Coll Cardiol 39(12):1890–1900

    Article  Google Scholar 

  2. Khairy P, Ionescu-Ittu R, Mackie AS, Abrahamowicz M, Pilote L, Marelli AJ (2010) Changing mortality in congenital heart disease. J Am Coll Cardiol 56(14):1149–1157

    Article  Google Scholar 

  3. Al Habib HF, Jacobs JP, Mavroudis C, Tchervenkov CI, O'Brien SM, Mohammadi S et al (2010) Contemporary patterns of management of tetralogy of Fallot: data from the Society of Thoracic Surgeons Database. Ann Thorac Surg 90(3):813–820

    Article  Google Scholar 

  4. Nollert G, Fischlein T, Bouterwek S, Böhmer C, Klinner W, Reichart B (1997) Long-term survival in patients with repair of tetralogy of Fallot: 36-year follow-up of 490 survivors of the first year after surgical repair. J Am Coll Cardiol 30(5):1374–1383

    Article  CAS  Google Scholar 

  5. Murphy JG, Gersh BJ, Mair DD, Fuster V, McGoon MD, Ilstrup DM et al (1993) Long-term outcome in patients undergoing surgical repair of tetralogy of Fallot. N Engl J Med 329(9):593–599

    Article  CAS  Google Scholar 

  6. 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):975–981

    Article  CAS  Google Scholar 

  7. Khairy P, Aboulhosn J, Gurvitz MZ, Opotowsky AR, Mongeon F-P, Kay J et al (2010) Arrhythmia burden in adults with surgically repaired tetralogy of Fallot. Circulation 122(9):868

    Article  Google Scholar 

  8. 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):211–216

    Article  CAS  Google Scholar 

  9. Valente AM, Gauvreau K, Assenza GE, Babu-Narayan SV, Schreier J, Gatzoulis MA et al (2014) Contemporary predictors of death and sustained ventricular tachycardia in patients with repaired tetralogy of Fallot enrolled in the INDICATOR cohort. Heart 100(3):247–253

    Article  Google Scholar 

  10. Buechel ER, Dave HH, Kellenberger CJ, Dodge-Khatami A, Pretre R, Berger F et al (2005) 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 26(24):2721–2727

    Article  Google Scholar 

  11. Oosterhof T, van Straten A, Vliegen HW, Meijboom FJ, van Dijk AP, Spijkerboer AM et al (2007) Preoperative thresholds for pulmonary valve replacement in patients with corrected tetralogy of Fallot using cardiovascular magnetic resonance. Circulation 116(5):545–551

    Article  Google Scholar 

  12. Bokma JP, Geva T, Sleeper LA, Babu Narayan SV, Wald R, Hickey K et al (2018) A propensity score-adjusted analysis of clinical outcomes after pulmonary valve replacement in tetralogy of Fallot. Heart 104(9):738

    Article  Google Scholar 

  13. Harrild DM, Berul CI, Cecchin F, Geva T, Gauvreau K, Pigula F et al (2009) Pulmonary valve replacement in tetralogy of Fallot: impact on survival and ventricular tachycardia. Circulation 119(3):445–451

    Article  Google Scholar 

  14. Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM et al (2018) AHA/ACC guideline for the management of adults with congenital heart disease. J Am Coll Cardiol. 73:81–192

    Article  Google Scholar 

  15. Priori SG, Blomström-Lundqvist C, Mazzanti A, Blom N, Borggrefe M, Camm J et al (2015) 2015 ESC guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. The Task Force for the Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death of the European Society of Cardiology (ESC) Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC). Eur Heart J 36(41):2793–2867

    Article  Google Scholar 

  16. Haas NA, Schirmer KR (2017) Guidelines for the management of congenital heart diseases in childhood and adolescence. Cardiol Young 27(S3):S1–S105

    Article  Google Scholar 

  17. Davlouros PA, Kilner PJ, Hornung TS, Li W, Francis JM, Moon JCC et al (2002) Right ventricular function in adults with repaired tetralogy of Fallot assessed with cardiovascular magnetic resonance imaging. J Am Coll Cardiol 40(11):2044–2052

    Article  Google Scholar 

  18. 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):1068–1074

    Article  Google Scholar 

  19. Rebergen SA, Chin JG, Ottenkamp J, van der Wall EE, de Roos A (1993) Pulmonary regurgitation in the late postoperative follow-up of tetralogy of Fallot. Volumetric quantitation by nuclear magnetic resonance velocity mapping. Circulation 88(5):2257–2266

    Article  CAS  Google Scholar 

  20. Buddhe S, Shah A, Lai WW (2015) Progression of right ventricular dilation in repaired tetralogy of Fallot. J Magn Reson Imaging 41(3):730–737

    Article  Google Scholar 

  21. Rutz T, Ghandour F, Meierhofer C, Naumann S, Martinoff S, Lange R et al (2017) Evolution of right ventricular size over time after tetralogy of Fallot repair: a longitudinal cardiac magnetic resonance study. Eur Heart J Cardiovasc Imaging 18(3):364–370

    Article  Google Scholar 

  22. Wald RM, Valente AM, Gauvreau K, Babu-Narayan SV, Assenza GE, Schreier J et al (2015) Cardiac magnetic resonance markers of progressive RV dilation and dysfunction after tetralogy of Fallot repair. Heart 101(21):1724

    Article  CAS  Google Scholar 

  23. Ponikowski P, Voors AA, Anker SD, Bueno H, Cleland JGF, Coats AJS et al (2016) 2016 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure. The Task Force for the diagnosis and treatment of acute and chronic heart failure of the European Society of Cardiology (ESC) Developed with the special contribution of the Heart Failure Association (HFA) of the ESC. Eur Heart J 37(27):2129–2200

    Article  Google Scholar 

  24. Wald RM, Haber I, Wald R, Valente AM, Powell AJ, Geva T (2009) Effects of regional dysfunction and late gadolinium enhancement on global right ventricular function and exercise capacity in patients with repaired tetralogy of Fallot. Circulation 119(10):1370–1377

    Article  Google Scholar 

  25. Babu-Narayan SV, Kilner PJ, Li W, Moon JC, Goktekin O, Davlouros PA et al (2006) Ventricular fibrosis suggested by cardiovascular magnetic resonance in adults with repaired tetralogy of Fallot and its relationship to adverse markers of clinical outcome. Circulation 113(3):405–413

    Article  CAS  Google Scholar 

  26. Cochet H, Iriart X, Allain-Nicolaï A, Camaioni C, Sridi S, Nivet H et al (2019) Focal scar and diffuse myocardial fibrosis are independent imaging markers in repaired tetralogy of Fallot. Eur Heart J Cardiovasc Imaging 20:990–1003

    Article  Google Scholar 

  27. Hanneman K, Crean AM, Wintersperger BJ, Thavendiranathan P, Nguyen ET, Kayedpour C et al (2017) The relationship between cardiovascular magnetic resonance imaging measurement of extracellular volume fraction and clinical outcomes in adults with repaired tetralogy of Fallot. Eur Heart J Cardiovasc Imaging 19(7):777–784

    Article  Google Scholar 

  28. Priromprintr B, Silka MJ, Rhodes J, Batra AS (2016) A prospective 5-year study of the frequency of arrhythmias during serial exercise testing and clinical follow-up after Melody valve implant. Heart Rhythm 13(11):2135–2141

    Article  Google Scholar 

  29. Meierhofer C, Tavakkoli T, Kühn A, Ulm K, Hager A, Müller J et al (2017) Importance of non-invasive right and left ventricular variables on exercise capacity in patients with tetralogy of Fallot hemodynamics. Pediatr Cardiol 38(8):1569–1574

    Article  Google Scholar 

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Acknowledgements

JR is supported by a governmental educational grant from the Egyptian Ministry of Higher Education.

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Authors and Affiliations

  1. Congenital Heart Disease and Pediatric Cardiology, German Heart Center, Technical University of Munich, Munich, Germany

    Judy Rizk, Nerejda Shehu, Heiner Latus, Peter Ewert, Heiko Stern & Christian Meierhofer

  2. Department of Cardiology, Faculty of Medicine, Alexandria University, El-Khartoum Square, Alexandria, 21521, Egypt

    Judy Rizk

  3. Radiology, German Heart Center, Technical University of Munich, Munich, Germany

    Stefan Martinoff

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  1. Judy Rizk
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  2. Nerejda Shehu
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Correspondence to Judy Rizk.

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Rizk, J., Shehu, N., Latus, H. et al. Magnetic Resonance Imaging Risk Factors for Ventricular Arrhythmias in Tetralogy of Fallot. Pediatr Cardiol 41, 862–868 (2020). https://doi.org/10.1007/s00246-020-02323-0

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  • DOI: https://doi.org/10.1007/s00246-020-02323-0

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