Skip to main content

Advertisement

Log in

Predictors of Atrial Arrhythmia in Adults with Repaired Tetralogy of Fallot

  • Research
  • Published:
Pediatric Cardiology Aims and scope Submit manuscript

Abstract

Tetralogy of Fallot (TOF), the most common cyanotic congenital heart disease in adults, has excellent long-term survival. However, many patients (30–45%) develop late arrhythmias. Previous studies have identified predictors of arrhythmia (atrial or ventricular) using clinical markers that predate arrhythmia onset by many years. Our objective was to develop a predictive model for incident atrial arrhythmias within two years of clinical evaluation and diagnostic testing. A single-center nested unmatched case-control study of 174 adults with repaired TOF. We included only patients with results from ECG and echocardiogram data in the required time interval (3–24 months before first arrhythmia for cases; 24 months of follow-up for controls). A predictive multivariable model for risk of incident atrial arrhythmia was developed using logistic regression with a least absolute shrinkage and selection operator (LASSO). Of 41 demographic, surgical, and diagnostic variables, six were selected as having predictive value for atrial arrhythmia based on cross validation. The factors with the greatest predictive value in decreasing order were moderate / severe tricuspid regurgitation (adjusted odds ratio (OR) 149.42), QRS fragmentation (OR 28.08), severe pulmonary regurgitation (OR 8.22), RV systolic dysfunction (OR 2.95), 1st degree AV block (OR 2.59), and age at time of surgical repair (OR 1.02). Predictors for atrial arrhythmia in our study suggested abnormal right ventricle anatomical function and electrophysiologic properties (conduction and repolarization) as the primary underlying substrate.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2

Similar content being viewed by others

Data Availability

No datasets were generated or analysed during the current study.

Abbreviations

AF:

atrial fibrillation

AFL:

atrial flutter

AT:

atrial tachycardia

AVNRT:

atrioventricular nodal reentrant tachycardia

BTT shunt:

Blalock-Thomas-Taussig shunt

CHB:

complete heart block

CHF:

congestive heart failure

cMRI:

cardiac magnetic resonance imaging

IART:

intra-atrial reentry tachycardia

ICD:

implantable cardioverter-defibrillator

LA:

left atrial

LV:

left ventricular

LVEF:

left ventricular ejection fraction

OR:

odds ratio

PAC:

premature atrial contraction

PVC:

premature ventricular contraction

PVR:

pulmonary valve replacement

RA:

right atrial

RV:

right ventricular

RVED:

right ventricular ejection fraction

RVEDVi:

indexed RV end diastolic volume

RVOT:

right ventricular outflow tract

SCA:

sudden cardiac arrest

SSS:

sick sinus syndrome

SVT:

supraventricular tachycardia

TA:

transannular

TOF:

Tetralogy of Fallot

TR:

tricuspid regurgitation

TTE:

transthoracic echocardiogram

VF:

ventricular fibrillation

VSD:

ventricular septal defect

VT:

ventricular tachycardia

WACH:

Washington Adult Congenital Heart

References

  1. Bailliard F, Anderson RH (2009) Tetralogy of Fallot. Orphanet J Rare Dis. 13;4:2. https://doi.org/10.1186/1750-1172-4-2. PMID: 19144126; PMCID: PMC2651859

  2. Monaco M, Williams I (2012) Tetralogy of Fallot: fetal diagnosis to surgical correction. Minerva Pediatr 64(5):461–470 PMID: 22992529

    PubMed  CAS  Google Scholar 

  3. Downing TE, Kim YY (2015) Tetralogy of Fallot: General Principles of Management. Cardiol Clin. 33(4):531 – 41, vii-viii. https://doi.org/10.1016/j.ccl.2015.07.002. Epub 2015 Aug 29. PMID: 26471818

  4. Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA, Del Nido P, Fasules JW, Graham TP Jr, Hijazi ZM, Hunt SA, King ME, Landzberg MJ, Miner PD, Radford MJ, Walsh EP, Webb GD (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-e263. https://doi.org/10.1016/j.jacc.2008.10.001. PMID: 19038677

  5. Smith CA, McCracken C, Thomas AS, Spector LG, St Louis JD, Oster ME, Moller JH, Kochilas L (2019) Long-term outcomes of tetralogy of Fallot: a study from the Pediatric Cardiac Care Consortium. JAMA Cardiol 4(1):34–41. https://doi.org/10.1001/jamacardio.2018.4255PMID: 30566184; PMCID: PMC6439686

    Article  PubMed  Google Scholar 

  6. Krieger EV, Zeppenfeld K, DeWitt ES, Duarte VE, Egbe AC, Haeffele C, Lin KY, Robinson MR, Sillman C, Upadhyay S, American Heart Association Adults With Congenital Heart Disease Committee of the Council on Lifelong Congenital Heart Disease and Heart Health in the Young and Council on Clinical Cardiology (2022) Arrhythmias in repaired tetralogy of Fallot: A Scientific Statement from the American Heart Association. Circ Arrhythm Electrophysiol 15(11):e000084. https://doi.org/10.1161/HAE.0000000000000084Epub 2022 Oct 20. PMID: 36263773

    Article  PubMed  Google Scholar 

  7. Pinsker BL, Serfas JD, Krasuski RA (2022) Burden and impact of Arrhythmias in Repaired Tetralogy of Fallot. Curr Cardiol Rep 24(3):225–234. https://doi.org/10.1007/s11886-022-01638-zEpub 2022 Feb 9. PMID: 35138576

    Article  Google Scholar 

  8. Harrison DA, Siu SC, Hussain F, MacLoghlin CJ, Webb GD, Harris L (2001) Sustained atrial arrhythmias in adults late after repair of tetralogy of Fallot. Am J Cardiol. 87(5):584-8. https://doi.org/10.1016/s0002-9149(00)01435-1. PMID: 11230843

  9. Cohen MI, Khairy P, Zeppenfeld K, Van Hare GF, Lakkireddy DR, Triedman JK Preventing Arrhythmic Death in Patients With Tetralogy of Fallot (2021) JACC Review Topic of the Week. J Am Coll Cardiol. 77(6):761–771. https://doi.org/10.1016/j.jacc.2020.12.021. PMID: 33573746

  10. Khairy P, Aboulhosn J, Gurvitz MZ, Opotowsky AR, Mongeon FP, Kay J, Valente AM, Earing MG, Lui G, Gersony DR, Cook S, Ting JG, Nickolaus MJ, Webb G, Landzberg MJ, Broberg CS, Alliance for Adult Research in Congenital Cardiology (AARCC) (2010) Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: a multi-institutional study. Circulation 122(9):868–875 Epub 2010 Aug 16. PMID: 20713900

    Article  Google Scholar 

  11. 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 – 81. https://doi.org/10.1016/S0140-6736(00)02714-8. PMID: 11041398

  12. Pinsker BL, Serfas JD, Awerbach JD, Dizon S, Spector ZZ, Campbell MJ, Krasuski RA (2022) Utility of Cardiac magnetic resonance imaging in Predicting Atrial Arrhythmias in Repaired Tetralogy of Fallot. Am J Cardiol 174:151–157 Epub 2022 Apr 23. PMID: 35473783

    Article  PubMed  Google Scholar 

  13. Ait Ali L, Lurz P, Ripoli A, Rossi G, Kister T, Aquaro GD, Passino C, Bonhoeffer P, Festa P (2019) Implications of atrial volumes in surgical corrected Tetralogy of Fallot on clinical adverse events. Int J Cardiol. 283:107–111. doi: 10.1016/j.ijcard.2019.02.018. Epub 2019 Feb 11. PMID: 30819586

  14. Lang RM, Bierig M, Devereux RB, Flachskampf FA, Foster E, Pellikka PA, Picard MH, Roman MJ, Seward J, Shanewise JS, Solomon SD, Spencer KT, Sutton MS, Stewart WJ, Chamber Quantification Writing Group; American Society of Echocardiography’s Guidelines and Standards Committee; European Association of Echocardiography (2005); Recommendations for chamber quantification: a report from the American Society of Echocardiography’s Guidelines and Standards Committee and the Chamber Quantification Writing Group, developed in conjunction with the European Association of Echocardiography, a branch of the European Society of Cardiology. J Am Soc Echocardiogr. 18(12):1440-63. https://doi.org/10.1016/j.echo.2005.10.005. PMID: 16376782

  15. Tibshirani R (1996) Regression shrinkage and selection via the lasso. J Roy Stat Soc: Ser B (Methodol) 58:267–288

    MathSciNet  Google Scholar 

  16. Kowarik A, Templ M (2016) Imputation with the r package vim. J Stat Softw 74(7). https://doi.org/10.18637/jss.v074.i07

  17. Friedman J, Hastie T, Tibshirani R (2010) Regularization paths for generalized linear models via coordinate descent. J Stat Softw 33(1). https://doi.org/10.18637/jss.v033.i01

  18. Kimura Y, Fukuda K, Nakano M, Hasebe Y, Fukasawa K, Chiba T, Miki K, Tatebe S, Miyata S, Ota H, Kimura M, Adachi O, Saiki Y, Shimokawa H (2018) Prognostic Significance of PR Interval Prolongation in Adult Patients With Total Correction of Tetralogy of Fallot. Circ Arrhythm Electrophysiol. 11(11):e006234. https://doi.org/10.1161/CIRCEP.118.006234. PMID: 30571179

  19. Spies F, Knecht S, Zeljkovic I, Reichlin T, Madaffari A, Osswald S, Sticherling C, Kühne M (2021) First-degree atrioventricular block in patients with atrial fibrillation and atrial flutter: the prevalence of intra-atrial conduction delay. J Interv Card Electrophysiol 61(2):421–425. https://doi.org/10.1007/s10840-020-00838-3Epub 2020 Jul 30. PMID: 32734408; PMCID: PMC8324594

    Article  PubMed  Google Scholar 

  20. Wijesekera VA, Raju R, Precious B, Berger AJ, Kiess MC, Leipsic JA, Grewal J (2016) Congenit Heart Dis 11(6):606–614. https://doi.org/10.1111/chd.12354Epub 2016 May 26. PMID: 27225732 Sequential Right and Left Ventricular Assessment in Post-Tetralogy of Fallot Patients with Significant Pulmonary Regurgitation

  21. Izumi G, Takeda A, Yamazawa H, Nagai A, Sasaki D, Sato I, Kato N, Tachibana T (2022) Usefulness of prolonged PR interval to Predict Atrial Tachyarrhythmia Development following Surgical repair of tetralogy of Fallot. Am J Cardiol 184:127–132 Epub 2022 Sep 17. PMID: 36127179

    Article  PubMed  Google Scholar 

  22. Ait Ali L, Trocchio G, Crepaz R, Stuefer J, Stagnaro N, Siciliano V, Molinaro S, Sicari R, Festa P (2016) Left ventricular dysfunction in repaired tetralogy of Fallot: incidence and impact on atrial arrhythmias at long term-follow up. Int J Cardiovasc Imaging 32(9):1441–1449 Epub 2016 Jun 18. PMID: 27318953

    Article  PubMed  Google Scholar 

  23. Buntharikpornpun R, Jaruratanasirikul S, Roymanee S, Jarutach J, Wongwaitaweewong K, Sangthong R (2021) Correlation between fragmented QRS and ventricular function from Cardiac magnetic resonance in patients with repaired tetralogy of Fallot. Pediatr Cardiol 42(8):1713–1721 Epub 2021 Jun 10. PMID: 34110460

    Article  PubMed  Google Scholar 

  24. 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 GF (2019) 2018 AHA/ACC Guideline for the management of adults with congenital heart disease: executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice guidelines. Circulation 139(14):e637–e697. https://doi.org/10.1161/CIR.0000000000000602

    Article  PubMed  Google Scholar 

  25. Sachdeva R, Valente AM, Armstrong AK, Cook SC, Han BK, Lopez L, Lui GK, Pickard SS, Powell AJ, Bhave NM, Sachdeva R, Valente AM, Pickard SS, Baffa JM, Banka P, Cohen SB, Glickstein JS, Kanter JP, Kanter RJ, Kim YY, Kipps AK, Latson LA, Lin JP, Parra DA, Rodriguez FH 3rd, Saarel EV, Srivastava S, Stephenson EA, Stout KK, Zaidi AN, ACC/AHA/ASE/HRS/ISACHD/SCAI/SCCT/SCMR /SOPE 2020 Appropriate Use Criteria for Multimodality Imaging During the Follow-Up Care of Patients With Congenital Heart Disease: A Report of the American College of Cardiology Solution Set Oversight Committee and Appropriate Use Criteria Task Force, American Heart Association, American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and Society of Pediatric chocardiography. J Am Coll Cardiol 75(6):657–703. doi: https://doi.org/10.1016/j.jacc.2019.10.002. Epub 2020 Jan 6. PMID: 31918898

  26. Moak JP (2017) Ablation of Atrial Arrhythmias in Postoperative Congenital Heart Disease Patients: Have We Reached the Upper Limit of Success or Is It Time for a Paradigm Shift in Strategy? Circ Arrhythm Electrophysiol. 10(12):e006021. https://doi.org/10.1161/CIRCEP.117.006021. PMID: 29247035

Download references

Funding

Not applicable.

Author information

Authors and Affiliations

Authors

Contributions

ZEF - made substantial contributions to the conception of the work; acquisition, analysis, and interpretation of data, and writing of the MSAMA - made substantial contributions to the conception of the work; acquisition, analysis, and interpretation of data, and writing of the MSSI - made substantial contributions to the conception of the work; interpretation of data, and writing of the MSJPM - made substantial contributions to the conception of the work; analysis, and interpretation of data, and writing of the MS.

Corresponding author

Correspondence to Jeffrey P. Moak.

Ethics declarations

Competing Interests

The authors declare no competing interests.

Additional information

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Falk, Z.E., Aldous, A.M., Ito, S. et al. Predictors of Atrial Arrhythmia in Adults with Repaired Tetralogy of Fallot. Pediatr Cardiol (2024). https://doi.org/10.1007/s00246-024-03436-6

Download citation

  • Received:

  • Accepted:

  • Published:

  • DOI: https://doi.org/10.1007/s00246-024-03436-6

Keywords

Navigation