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Impact of right atrium dimension on adverse outcome after pulmonary valve replacement in repaired Tetralogy of Fallot patients

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Abstract

The hemodynamic impact of residual pulmonary regurgitation (PR) in repaired Tetralogy of Fallot (rTOF) has been well demonstrated. However, markers driving the decision making process to indicate the ideal timing of pulmonary valve replacement (PVR) are still uncertain. Furthermore, very few studies have included the right atrium (RA) dilatation as a preoperative risk factor for post-PVR clinical adverse outcome. The aim of this study was to investigate the impact of pre-PVR right atrial dilation on adverse outcomes in rTOF. We retrospectively reviewed from our CMR database all rTOF patients who underwent CMR study before and after PVR. Detailed clinical and surgical history were collected, in addition to imaging data. The composite primary and secondary post-PVR end points were also recorded. The study cohort consisted of 41 patients (mean age at PVR repair 27.4 ± 10 years). As expected, end-diastolic and end-systolic right ventricle (RV) volumes significantly decreased after PVR (p < 0.001). The RV reverse remodeling, defined by ΔRVEDVi and ΔRVESVi, was associated with both pre-PVR RVEDVi and RVESVi. The higher the pre-PVR RV volumes, more the RV reverse remodeling will be obtained post-surgery. Patients who experienced an adverse outcome were older at pre-PVR, they had a higher Nt-ProBNP, worse VO2/kg/min, more significant tricuspid regurgitation and more dilated RA. The association with the RA dilatation persists and becomes even more significant if we exclude patients who had tricuspid repair beside RVOT surgical reconstruction. Besides RV volumes and function, RA dimensions may play a pivotal role in the decision making of TOF patients.

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Abbreviations

CMR:

Cardiovascular magnetic resonance imaging

CHD:

Congenital heart disease

CPET:

Cardiopulmonary exercise test

LA:

Left atrium

LASVi:

Left atrial endsystolic volume index

LGE:

Late Gadolinium Enhancement

LVEDVi:

Left ventricular enddiastolic volume index

PRF:

Pulmonary regurgitation fraction

PVR:

Pulmonary valve replacement

RA:

Right atrium

RV:

Right ventricle

RASVi:

Right atrial endsystolic volume index

RVEDVi:

Right ventricular end-diastolic volume index

RVOT:

Right ventricle outflow tract

TOF:

Tetralogy of Fallot

References

  1. 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 

  2. Murphy JG, Gersh BJ, Mair DD, Fuster V, McGoon MD, Ilstrup DM, McGoon DC, Kirklin JW, Danielson GK (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 

  3. Gatzoulis MA, Balaji S, Webber SA, Siu SC, Hokanson JS, Poile C, Rosenthal M, Nakazawa M, Moller JH, Gillette PC, Webb GD, Redington AN (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 

  4. Bouzas B, Kilner PJ, Gatzoulis MA (2005) Pulmonary regurgitation: not a benign lesion. Eur Heart J 26(5):433–439

    Article  Google Scholar 

  5. 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 

  6. Knauth AL, Gauvreau K, Powell AJ, Landzberg MJ, Walsh EP, Lock JE, del Nido PJ, Geva T (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 

  7. 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 

  8. Oosterhof T, Meijboom FJ, Vliegen HW, Hazekamp MG, Zwinderman AH, Bouma BJ, van Dijk AP, Mulder BJ (2006) Long-term follow-up of homograft function after pulmonary valve replacement in patients with tetralogy of Fallot. Eur Heart J 27(12):1478–1484

    Article  Google Scholar 

  9. Vliegen HW, van Straten A, de Roos A, Roest AA, Schoof PH, Zwinderman AH, Ottenkamp J, van der Wall EE, Hazekamp MG (2002) Magnetic resonance imaging to assess the hemodynamic effects of pulmonary valve replacement in adults late after repair of tetralogy of Fallot. Circulation 106(13):1703–1707

    Article  Google Scholar 

  10. Oosterhof T, van Straten A, Vliegen HW, Meijboom FJ, van Dijk AP, Spijkerboer AM, Bouma BJ, Zwinderman AH, Hazekamp MG, de Roos A, Mulder BJ (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 

  11. Lee C, Kim YM, Lee CH, Kwak JG, Park CS, Song JY, Shim WS, Choi EY, Lee SY, Baek JS (2012) Outcomes of pulmonary valve replacement in 170 patients with chronic pulmonary regurgitation after relief of right ventricular outflow tract obstruction: implications for optimal timing of pulmonary valve replacement. J Am Coll Cardiol 60(11):1005–1014

    Article  Google Scholar 

  12. Ferraz-Cavalcanti PE, Sá MP, Santos CA, Esmeraldo IM, De-Escobar RR, De-Menezes AM, de Azevedo OM, de Vasconcelos Silva FP, Lins RF, Lima RDC (2013) 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 62(23):2227–2224

    Article  Google Scholar 

  13. Geva T, Gauvreau K, Powell AJ, Cecchin F, Rhodes J, Geva J, Del-Nido P (2010) Randomized trial of pulmonary valve replacement with and without right ventricular remodeling surgery. Circulation 122(11 Suppl):S201–S208

    Article  Google Scholar 

  14. Therrien J, Provost Y, Merchant N, Williams W, Colman J, Webb G (2005) Optimal timing for pulmonary valve replacement in adults after tetralogy of Fallot repair. Am J Cardiol 95(6):779–782

    Article  Google Scholar 

  15. Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM, Crumb SR, Dearani JA, Fuller S, Gurvitz M, Khairy P, Landzberg MJ, Saidi A, Valente AM, Van Hare GFP (2019) 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 139(14):e698–e780

    Google Scholar 

  16. Ait Ali L, Lurz P, Ripoli A, Rossi G, Kister T, Aquaro GD, Passino C, Bonhoeffer P (2019) Festa P implications of atrial volumes in surgical corrected tetralogy of Fallot on clinical adverse events. Int J Cardiol 283:107–111

    Article  Google Scholar 

  17. Corno AF, Festa P (2009) Tetralogy of Fallot. Congenital heart defects: decision making for cardiac surgery volume 3 CT-scan and MRI. Springer, Berlin

    Book  Google Scholar 

  18. Babu-Narayan SV, Kilner PJ, Li W, Moon JC, Goktekin O, Davlouros PA, Khan M, Ho SY, Pennell DJ, Gatzoulis MA (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 

  19. Maceira AM, Cosín-Sales J, Roughton M, Prasad SK, Pennell DJ (2013) Reference right atrial dimensions and volume estimation by steady state free precession cardiovascular magnetic resonance. J Cardiovasc Magn Reson 15:29

    Article  Google Scholar 

  20. Bokma JP, Geva T, Sleeper LA, Babu Narayan SV, Wald R, Hickey K, Jansen K, Wassall R, Lu M, Gatzoulis MA, Mulder BJ, Valente AM (2018) A propensity score-adjusted analysis of clinical outcomes after pulmonary valve replacement in tetralogy of Fallot. Heart 104(9):738–744

    Article  Google Scholar 

  21. Therrien J, Siu SC, McLaughlin PR, Liu PP, Williams WG, Webb GD (2000) Pulmonary valve replacement in adults late after repair of tetralogy of Fallot: are we operating too late? J Am Coll Cardiol 36:1670–1675

    Article  CAS  Google Scholar 

  22. van Straten A, Vliegen HW, Hazekamp MG, Bax JJ, Schoof PH, Ottenkamp J, van der Wall EE, de Roos A (2004) Right ventricular function after pulmonary valve replacement in patients with tetralogy of Fallot. Radiology 233:824–829

    Article  Google Scholar 

  23. Buechel ER, Dave HH, Kellenberger CJ, Dodge-Khatami A, Pretre R, Berger F, Bauersfeld U (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 26:2721–2727

    Article  Google Scholar 

  24. Khambadkone S, Coats L, Taylor A, Boudjemline Y, Derrick G, Tsang V, Cooper J, Muthurangu V, Hegde SR, Razavi RS, Pellerin D, Deanfield J, Bonhoeffer P (2005) Percutaneous pulmonary valve implantation in humans: results in 59 consecutive patients. Circulation 112:1189–1197

    Article  Google Scholar 

  25. Geva T, Mulder B, Gauvreau K, Babu-Narayan SV, Wald RM, Hickey K, Powell AJ, Gatzoulis MA, Valente AM (2018) 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 138(19):2106–2115

    Article  Google Scholar 

  26. Henkens IR, van Straten A, Schalij MJ et al (2007) Predicting outcome of pulmonary valve replacement in adult tetralogy of Fallot patients. Ann Thorac Surg 83:907–911

    Article  Google Scholar 

  27. Redington AN (2014) Low cardiac output due to acute right ventricular dysfunction and cardiopulmonary interactions in congenital heart disease (2013 Grover Conference series). Pulm Circ 4(2):191–199

    Article  Google Scholar 

  28. Sandeep B, Huang X, Xu F, Su P, Wang T, Sun X (2019) Etiology of right ventricular restrictive physiology early after repair of tetralogy of Fallot in pediatric patients. J Cardiothorac Surg 14(1):84

    Article  Google Scholar 

  29. DiLorenzo M, Hwang WT, Goldmuntz E, Ky B, Mercer-Rosa L (2018) Diastolic dysfunction in tetralogy of Fallot: comparison of echocardiography with catheterization. Echocardiography 35(10):1641–1648

    Article  Google Scholar 

  30. Rudski LG, Lai WW, Afilalo J, Hua L, Handschumacher MD, Chandrasekaran K, Solomon SD, Louie EK, Schiller NB (2010) Guidelines for the echocardiographic assessment of the right heart in adults: a report from the American Society of Echocardiography endorsed by the European Association of Echocardiography, a registered branch of the European Society of Cardiology, and the Canadian Society of Echocardiography. J Am Soc Echocardiogr 23(7):685–713

    Article  Google Scholar 

  31. Kutty S, Valente AM, White MT, Hickey K, Danford DA, Powell AJ, Geva T (2018) Usefulness of pulmonary arterial end-diastolic forward flow late after tetralogy of Fallot repair to predict a "restrictive" right ventricle. Am J Cardiol 121(11):1380–1386

    Article  Google Scholar 

  32. Mouratoglou SA, Dimopoulos K, Kamperidis V, Feloukidis C, Kallifatidis A, Pitsiou G, Stanopoulos I, Grosomanidis V, Hadjimiltiades S, Karvounis H, Giannakoulas G (2018) Right atrial function predicts clinical outcome in patients with precapillary pulmonary hypertension. J Am Soc Echocardiogr 31(10):1137–1145

    Article  Google Scholar 

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Acknowledgements

Many thanks to Susan Elizabeth Gwynne for her precious help in the manuscript editing

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Authors

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LA contribute to research design, data analysis, drafting the paper and approved the submitted manuscript. CM and PF contribute to research design, the acquisition and data interpretation and the paper drafting and approved the submitted manuscript, SS contribute to analysis of the data, revised critically and approved the submitted manuscript, DF, VP, GS and LA contribute to the data interpretation, critically drafting the paper and approved the submitted manuscript. CP contribute to data acquisition revised critically and approved the submitted manuscript.

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Correspondence to Lamia Ait-ALi.

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Ait-ALi, L., Marrone, C., Salvadori, S. et al. Impact of right atrium dimension on adverse outcome after pulmonary valve replacement in repaired Tetralogy of Fallot patients. Int J Cardiovasc Imaging 36, 1973–1982 (2020). https://doi.org/10.1007/s10554-020-01891-9

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