Pediatric Cardiology

, Volume 39, Issue 8, pp 1590–1597 | Cite as

Bradyarrhythmias in Repaired Atrioventricular Septal Defects: Single-Center Experience Based on 34 Years of Follow-Up of 522 Patients

  • Corrado Di MambroEmail author
  • Camilla Calvieri
  • Massimo Stefano Silvetti
  • Ilaria Tamburri
  • Salvatore Giannico
  • Anwar Baban
  • Sonia Albanese
  • Gianluca Brancaccio
  • Adriano Carotti
  • Fiore Salvatore Iorio
  • Fabrizio Drago
Original Article


Atrioventricular Septal Defect (AVSD) is a rare congenital heart defect (CHD) often associated with genetic syndromes, most commonly Down syndrome (DS). Over the last four decades, surgical repair has increased survival and improved quality of life in these patients. The prevalence of bradyarrhythmias namely, atrioventricular block (AVB) and sinus node dysfunction (SND) in AVSD is partially known. 522 cases with both partial and complete AVSD (38.7% with DS), undergoing intracardiac repair from 1982 to 2016 at our institution, were reviewed from our system database. 38 (7.3%) patients received permanent PM implantation for AVB (early or late) or SND. On one hand, AVB requiring PM was found in 26 (4.98%). This was further subdivided into early-onset 14 (2.6%) and late-onset AVB 12 (2.2%) (median 4 [IQR 1–7] years). On the other hand, 12 (2.3%) experienced late SND requiring PM (median 11 [IQR 3.5–15.2] years). Early and late AVB were independent from the type of AVSD (partial or complete), whereas the late SND was remarkably observed in complete AVSD compared to partial AVSD (p = 0.017). We classified the cohort into two main categories: DS (202, 38.7%) and non-DS (320, 61.3%). At Kaplan–Meier survival analysis, DS was significantly associated with late-onset bradyarrhythmias (p = 0.024). At Cox regression analysis, we identified DS as an independent predictor of PM implantation (HR 2.17). In conclusion, about 7% of repaired AVSD patients need PM implantation during follow-up. There are no differences in early and late AVB occurrence according to the type of AVSD. There is a higher incidence of late SND in repaired complete AVSD, with a later timing onset in patients with associated DS. Moreover, DS seems to be an independent predictor of PM implantation.


Congenital heart disease Atrioventricular septal defect Atrioventricular block Sinus node dysfunction Down syndrome Pacemaker implantation 



Atrioventricular septal defect


Congenital heart defect


Down syndrome


Atrioventricular block


Sinus node dysfunction





The authors would like to thank Dr. Elisa Del Vecchio for her valuable collaboration in the editorial revision.

Compliance with Ethical Standards

Conflict of interest

The authors have no conflict of interest.

Ethical Approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. This article does not contain any studies with animals performed by any of the authors.

Informed Consent

Informed consent was obtained from all individual participants included in the study.


  1. 1.
    Calkoen EE, Hazekamp MG, Blom NA et al (2016) Atrioventricular septal defect: from embryonic development to long-term follow-up. Int J Cardiol 202:784–795CrossRefGoogle Scholar
  2. 2.
    Christensen N, Andersen H, Garne E et al (2013) Atrioventricular septal defects among infants in Europe: a population-based study of prevalence, associated anomalies, and survival. Cardiol Young 23:560–567CrossRefGoogle Scholar
  3. 3.
    Lillehei CW, Sellers RD, Bonnabeau RC, Eliot RS (1963) Chronic postsurgical complete heart block. With particular reference to prognosis, management, and a new P-wave pacemaker. J Thorac Cardiovasc Surg 46:436–456PubMedGoogle Scholar
  4. 4.
    Bonatti V, Agnetti A, Squarcia U (1998) Early and late postoperative complete heart block in pediatric patients submitted to open-heart surgery for congenital heart disease. Pediatr Med Chir 20:181–186PubMedGoogle Scholar
  5. 5.
    Gross GJ, Chiu CC, Hamilton RM, Kirsh JA, Stephenson EA (2006) Natural history of postoperative heart block in congenital heart disease: implications for pacing intervention. Heart Rhythm 3:601–604CrossRefGoogle Scholar
  6. 6.
    Anderson JB, Czosek RJ, Knilans TK, Meganathan K, Heaton P (2012) Postoperative heart block in children with common forms of congenital heart disease: results from the KID database. J Cardiovasc Electrophysiol 23:1349–1354CrossRefGoogle Scholar
  7. 7.
    Weindling SN, Saul JP, Gamble WJ, Mayer JE, Wessel D, Walsh EP (1998) Duration of complete atrioventricular block after congenital heart disease surgery. Am J Cardiol 82:525–527CrossRefGoogle Scholar
  8. 8.
    Laurens P, Gavelle P, Piwnica A, Farge C, Dubost C, Maurice P (1983) Severe postoperative heart blocks appearing late. 16 cases. Arch Mal Coeur Vaiss 76:1132–1139PubMedGoogle Scholar
  9. 9.
    Nishimura RA, Callahan MJ, Holmes DR Jr et al (1984) Transient atrioventricular block after open-heart surgery for congenital heart disease. Am J Cardiol 53:198–201CrossRefGoogle Scholar
  10. 10.
    Godman MJ, Roberts NK, Izukawa T (1974) Late postoperative conduction disturbances after repair of ventricular septal defect and tetralogy of Fallot. Analysis by his bundle recordings. Circulation 49:214–221CrossRefGoogle Scholar
  11. 11.
    Moss AJ, Klyman G, Emmanouilides GC (1972) Late onset complete heart block. Newly recognized sequela of cardiac surgery. Am J Cardiol 30:884–887CrossRefGoogle Scholar
  12. 12.
    Banks MA, Jenson J, Kugler JD (2001) Late development of atrioventricular block after congenital heart surgery in Down syndrome. Am J Cardiol 88:A7:86–89CrossRefGoogle Scholar
  13. 13.
    Lange R, Guenther T, Busch R, Hess J, Schreiber C (2007) The presence of Down syndrome is not a risk factor in complete atrioventricular septal defect repair. J Thorac Cardiovasc Surg 134:304–310CrossRefGoogle Scholar
  14. 14.
    Al-Hay AA, MacNeill SJ, Yacoub M, Shore DF, Shinebourne EA (2003) Complete atrioventricular septal defect, Down syndrome, and surgical outcome: risk factors. Ann Thorac Surg 75:412–421CrossRefGoogle Scholar
  15. 15.
    Saffirio C, Marino B, Formigari R (2008) Better surgical prognosis for patients with Down syndrome. J Thorac Cardiovasc Surg 135:230CrossRefGoogle Scholar
  16. 16.
    Giroud JM, Jacobs JP, Spicer D et al (2010) Report from the international society for nomenclature of paediatric and congenital heart disease: creation of a visual encyclopedia illustrating the terms and definitions of the international pediatric and congenital cardiac code. World J Pediatr Congenit Heart Surg 1:300–313CrossRefGoogle Scholar
  17. 17.
    Formigari R, Michielon G, Digilio MC et al (2009) Genetic syndromes and congenital heart defects: how is surgical management affected? Eur J Cardiothorac Surg 35:606–614CrossRefGoogle Scholar
  18. 18.
    Niu MC, Dickerson HA, Moore JA et al (2018) Heterotaxy syndrome and associated arrhythmias in pediatric patients. Heart Rhythm 15:548–554CrossRefGoogle Scholar
  19. 19.
    Gregoratos G, Abrams J, Epstein AE American College of Cardiology/American Heart Association Task Force on Practice Guidelines/North American Society for Pacing and Electrophysiology Committee to Update the 1998 Pacemaker Guidelines et al (2002) ACC/AHA/NASPE 2002 guideline update for implantation of cardiac pacemakers and antiarrhythmia devices: summary article: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (ACC/AHA/NASPE Committee to Update the 1998 Pacemaker Guidelines). Circulation 106:2145–2161CrossRefGoogle Scholar
  20. 20.
    Epstein AE, DiMarco JP, Ellenbogen KA American College of Cardiology Foundation; American Heart Association Task Force on Practice Guidelines; Heart Rhythm Society et al (2013) 2012 ACCF/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines and the Heart Rhythm Society. Circulation 127:e283–e352CrossRefGoogle Scholar
  21. 21.
    Batra AS, Wells WJ, Hinoki KW, Stanton RA, Silka MJ (2003) Late recovery of atrioventricular conduction after pacemaker implantation for complete heart block associated with surgery for congenital heart disease. J Thorac Cardiovasc Surg 125:1291–1293CrossRefGoogle Scholar
  22. 22.
    Liberman L, Pass RH, Hordof AJ, Spotnitz HM (2008) Late onset of heart block after open heart surgery for congenital heart disease. Pediatr Cardiol 29:56–59CrossRefGoogle Scholar
  23. 23.
    Brignole M, Auricchio A, Baron-Esquivias G et al (2013) 2013 ESC guidelines on cardiac pacing and cardiac resynchronization therapy: the task force on cardiac pacing and resynchronization therapy of the European Society of Cardiology (ESC). Developed in collaboration with the European Heart Rhythm Association (EHRA). Europace 15:1070–1118CrossRefGoogle Scholar
  24. 24.
    Goldman BS, Williams WG, Hill T et al (1985) Permanent cardiac pacing after open heart surgery: congenital heart disease. Pacing Clin Electrophysiol 8:732–739CrossRefGoogle Scholar
  25. 25.
    Alexi-Meskishvili V, Ishino K, Dähnert I et al (1996) Correction of complete atrioventricular septal defects with the double-patch technique and cleft closure. Ann Thorac Surg 62:519–524CrossRefGoogle Scholar
  26. 26.
    Devlin PJ, Backer CL, Eltayeb O, Mongé MC, Hauck AL, Costello JM (2016) Repair of partial atrioventricular septal defect: age and outcomes. Ann Thorac Surg 102:170–177CrossRefGoogle Scholar
  27. 27.
    Delaney JW, Moltedo JM, Dziura JD, Kopf GS, Snyder CS (2006) Early postoperative arrhythmias after pediatric cardiac surgery. J Thorac Cardiovasc Surg 131:1296–1300CrossRefGoogle Scholar
  28. 28.
    St Louis JD, Jodhka U, Jacobs JP et al (2014) Contemporary outcomes of complete atrioventricular septal defect repair: analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database. J Thorac Cardiovasc Surg 148:2526–2531CrossRefGoogle Scholar
  29. 29.
    Tucker EM, Pyles LA, Bass JL, Moller JH (2007) Permanent pacemaker for atrioventricular conduction block after operative repair of perimembranous ventricular septal defect. J Am Coll Cardiol 50:1196–1200CrossRefGoogle Scholar
  30. 30.
    Murray LE, Smith AH, Flack EC, Crum K, Owen J, Kannankeril PJ (2017) Genotypic and phenotypic predictors of complete heart block and recovery of conduction after surgical repair of congenital heart disease. Heart Rhythm 14:402–409CrossRefGoogle Scholar
  31. 31.
    Morris CD, Magilke D, Reller M (1992) Down’s syndrome affects results of surgical correction of complete atrioventricular canal. Pediatr Cardiol 13:80–84CrossRefGoogle Scholar
  32. 32.
    Blom NA, Ottenkamp J, Deruiter MC, Wenink ACG, Gittenberger-De Groot AC (2005) Development of the cardiac conduction system in atrioventricular septal defect in human trisomy 21. Pediatr Res 58:516–520CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • Corrado Di Mambro
    • 1
    Email author return OK on get
  • Camilla Calvieri
    • 1
  • Massimo Stefano Silvetti
    • 1
  • Ilaria Tamburri
    • 1
  • Salvatore Giannico
    • 1
  • Anwar Baban
    • 1
  • Sonia Albanese
    • 2
  • Gianluca Brancaccio
    • 2
  • Adriano Carotti
    • 2
  • Fiore Salvatore Iorio
    • 3
  • Fabrizio Drago
    • 1
  1. 1.Pediatric Cardiology and Cardiac Arrhythmia Unit, Department of Pediatric Cardiology and Cardiac SurgeryBambino Gesù Children’s Hospital and Research InstituteRomeItaly
  2. 2.Pediatric Cardiac Surgery Unit, Department of Pediatric Cardiology and Cardiac SurgeryBambino Gesù Children’s Hospital and Research InstituteRomeItaly
  3. 3.Department of Pediatric Cardiology and Cardiac SurgeryBambino Gesù Children’s Hospital and Research InstituteRomeItaly

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