Incidence, Risk Factors, and Outcomes of Atrial Arrhythmias in Adult Patients With Atrioventricular Septal Defect

Jacquemart E, Bessière F, Combes N, Ladouceur M, Iserin L, Gardey K, Henaine R, Dulac A, Cohen S, Belli E, Jannot AS, Chevalier P, Ly R, Clavier S, Legendre A, Petit J, Maltret A, Di Filippo S, Hascoët S, Marijon E, Waldmann V.JACC Clin Electrophysiol. 2022 Mar;8(3):331-340. doi: 10.1016/j.jacep.2021.09.004. Epub 2021 Oct 27.PMID: 35331427


Take Home Points

  • Retrospective analysis of 391 patients diagnosed with AVSD (61% women) from 3 French centres (2001-2020). 22% of the cohort had trisomy 21. (Table 1)
  • 85% of the cohort had undergone reparative surgery.
  • A relatively small proportion (n=58, 15%) of the cohort were born with a complete AVSD (100% repaired).
  • Mean age at enrolment 36.3 ± 16.3 years and mean duration of follow up 17 years after surgical repair.
  • One quarter of patients (n=98) developed at least 1 atrial arrhythmia at a mean age of 39 years old.
  • Lifetime risks for developing atrial arrhythmia to ages 20, 40, 60 years were 3.7%, 17.8% and 55.3 respectively.
  • The commonest arrhythmia until the age of 45 years was IART/Focal atrial tachyarrhythmia. At beyond 45 years old, AF was the commonest arrhythmia.
  • Independent risks associated with developing atrial arrhythmias were age; number of cardiac operations; left or right atrial dilation and moderate or severe left AV valve regurgitation (Figure 1).
  • Those patients experiencing atrial arrhythmias had a higher subsequent risk of needing a pacemaker (42% vs 9%; P<0.001) ; developing heart failure (25% vs 1%; P<0.001) and experiencing a stroke (11% vs 3%; P=0.007).

Dr Damien Cullington

Commentary by Dr. Damien Cullington (Liverpool, UK), section editor of ACHD Journal Watch:

Patients born with an AVSD are commonly seen in the ACHD clinic – AVSD comprises 7% of all congenital cardiac lesions. Most patients fare well following their surgical repair but there are a number of important issues for the team to watch for over time – mostly these relate to monitoring of valvar regurgitation and possibly stenosis (usually the left valve) and the surveillance of/treatment of atrial arrhythmias or progressive AV block. This analysis is welcomed to help guide and inform the ACHD team and their patients about what chronic risk is posed by atrial arrhythmias to patients with AVSD – mostly those whom have undergone surgical repair. The characteristics of the study cohort are seen in Table 1. It is not documented what antiarrhythmic therapies patients were receiving during their follow up and if this may have adjusted outcomes.


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Table 1


Atrial arrhythmias

Over a mean FU of 17.3 ± 14.2 years after first surgical repair, one quarter of patients (n=98) presented with at least one episode of atrial arrhythmia (Figure 1). Until the age of 45 years, IART/FAT was the commonest arrhythmia. After the age of 45 years old, AF was commoner. At the end of follow up, approximately three quarters of patients (n=75, 77%) with a history of experiencing atrial arrhythmias subsequently had persistent or permanent atrial arrhythmia. Table 2 shows risk factors for developing atrial arrhythmia. The prevalence of atrial arrhythmia in different phenotypic subgroups is shown in Figure 2.


Table 2 – Risk factors for development of atrial arrhythmias


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Figure 1


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Figure 2


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  • It is unclear from the data presented how many patients underwent ablation for atrial arrhythmias during follow up and how this modified their outcomes.
  • It is unclear from the data presented what medical treatments patients received and how this may have modified outcomes.


Atrial arrhythmias occur frequently in patients with AVSD with over half experiencing such phenomena by the age of 60 years old. Whilst it is important to understand risks for developing atrial arrhythmias, further research is needed to understand how they could be optimally prevented. It would be insightful to understand how medical therapy (i.e. antiarrhythmics) and upfront ablative intervention helps to modify long term outcomes.