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

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 bradyarrhythmi...

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Bibliographic Details
Published in:Pediatric cardiology 2018-12, Vol.39 (8), p.1590-1597
Main Authors: 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
Format: Article
Language:English
Subjects:
Analysis
Arrhythmia
Atrioventricular Block - etiology
Bradycardia - etiology
Cardiac Surgery
Cardiology
Child
Child, Preschool
Cohort Studies
Congenital heart disease
Down syndrome
Female
Follow-Up Studies
Genetic aspects
Genetic disorders
Heart Septal Defects - physiopathology
Heart Septal Defects - surgery
Humans
Infant
Kaplan-Meier Estimate
Male
Medicine
Medicine & Public Health
Original Article
Pacemaker, Artificial - statistics & numerical data
Quality of Life
Retrospective Studies
Sick Sinus Syndrome - complications
Sick Sinus Syndrome - etiology
Time Factors
Vascular Surgery
Vascular Surgical Procedures - adverse effects
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Summary: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.
ISSN:0172-0643
1432-1971
DOI:10.1007/s00246-018-1934-4