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

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Abstract

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.

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Abbreviations

AVSD:

Atrioventricular septal defect

CHD:

Congenital heart defect

DS:

Down syndrome

AVB:

Atrioventricular block

SND:

Sinus node dysfunction

PM:

Pacemaker

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Acknowledgements

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

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Authors and Affiliations

  1. Pediatric Cardiology and Cardiac Arrhythmia Unit, Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children’s Hospital and Research Institute, Piazza Sant’Onofrio, 4, 00050, Rome, Italy

    Corrado Di Mambro, Camilla Calvieri, Massimo Stefano Silvetti, Ilaria Tamburri, Salvatore Giannico, Anwar Baban & Fabrizio Drago

  2. Pediatric Cardiac Surgery Unit, Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children’s Hospital and Research Institute, Rome, Italy

    Sonia Albanese, Gianluca Brancaccio & Adriano Carotti

  3. Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children’s Hospital and Research Institute, Rome, Italy

    Fiore Salvatore Iorio

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  1. Corrado Di Mambro
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  2. Camilla Calvieri
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  3. Massimo Stefano Silvetti
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Correspondence to Corrado Di Mambro.

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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.

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

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Di Mambro, C., Calvieri, C., Silvetti, M.S. et al. Bradyarrhythmias in Repaired Atrioventricular Septal Defects: Single-Center Experience Based on 34 Years of Follow-Up of 522 Patients. Pediatr Cardiol 39, 1590–1597 (2018). https://doi.org/10.1007/s00246-018-1934-4

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  • DOI: https://doi.org/10.1007/s00246-018-1934-4

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