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.

Bradyarrhythmias in repaired atrioventricular septal defects: single-center experience based on 34 years of follow-up of 522 patients / Di Mambro, Corrado; Calvieri, Camilla; Silvetti, Massimo Stefano; Tamburri, Ilaria; Giannico, Salvatore; Baban, Anwar; Albanese, Sonia; Brancaccio, Gianluca; Carotti, Adriano; Iorio, Fiore Salvatore; Drago, Fabrizio. - In: PEDIATRIC CARDIOLOGY. - ISSN 0172-0643. - 39:8(2018), pp. 1590-1597. [10.1007/s00246-018-1934-4]

Bradyarrhythmias in repaired atrioventricular septal defects: single-center experience based on 34 years of follow-up of 522 patients

Calvieri, Camilla
Secondo
Writing – Original Draft Preparation
;
Iorio, Fiore Salvatore
Penultimo
Supervision
;
2018

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.
2018
atrioventricular block; atrioventricular septal defect; congenital heart disease; down syndrome; pacemaker implantation; sinus node dysfunction; atrioventricular block; bradycardia; child; child, preschool; cohort studies; female; follow-up studies; heart septal defects; humans; infant; kaplan-meier estimate; male; pacemaker, artificial; quality of life; retrospective studies; sick sinus syndrome; time factors; vascular surgical procedures; pediatrics, perinatology and child health; cardiology and cardiovascular medicine
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Bradyarrhythmias in repaired atrioventricular septal defects: single-center experience based on 34 years of follow-up of 522 patients / Di Mambro, Corrado; Calvieri, Camilla; Silvetti, Massimo Stefano; Tamburri, Ilaria; Giannico, Salvatore; Baban, Anwar; Albanese, Sonia; Brancaccio, Gianluca; Carotti, Adriano; Iorio, Fiore Salvatore; Drago, Fabrizio. - In: PEDIATRIC CARDIOLOGY. - ISSN 0172-0643. - 39:8(2018), pp. 1590-1597. [10.1007/s00246-018-1934-4]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/1266551
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