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. 2022 Jul 13;108(15):1194-1199.
doi: 10.1136/heartjnl-2021-320411.

Familial risk of atrioventricular block in first-degree relatives

Affiliations

Familial risk of atrioventricular block in first-degree relatives

Johnni Resdal Dyssekilde et al. Heart. .

Abstract

Objective: Rare cases of genetically inherited atrioventricular block (AVB) have been reported; however, the heredity of AVB remains unknown. We aimed to assess the heredity of AVB.

Design, setting and participants: Using data from the Danish Civil Registration Registry, we established a nationwide cohort of individuals with parental links. Data were merged with information from the Danish Pacemaker and Implantable Cardioverter Defibrillator Registry, containing information on all pacemaker implantations performed in Denmark during the study period, to identify patients who received a first-time pacemaker because of AVB.

Results: A total of 4 648 204 individuals had parental links and a total of 26 880 consecutive patients received a first-time pacemaker due to AVB. Overall, the adjusted rate ratio (RR) of pacemaker implantation due to AVB was 2.1 (95% CI 1.8 to 2.5) if a father, mother or sibling had AVB compared with the risk in the general population. The adjusted RR was 2.2 (1.7-2.9) for offspring of mothers with AVB, 1.9 (1.5-2.4) for offspring of fathers with AVB and 3.5 (2.3-5.4) for siblings to a patient with AVB. The risk increased inversely proportionally with the age of the index case at the time of pacemaker implantation. The corresponding adjusted RRs were 15.8 (4.8-52.3) and 10.0 (3.3-30.4) if a mother or father, respectively, had a pacemaker implantation before 50 years.

Conclusion and relevance: First-degree relatives to a patient with AVB carry an increased risk of AVB with the risk being strongly inversely associated with the age of the index case at pacemaker implantation. These findings indicate a genetic component in the development of AVB in families with an early-onset disease.

Keywords: bradycardia; pacemaker, artificial.

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Conflict of interest statement

Competing interests: MKC has received lecture fees from Sanofi. JBJ reports personal fees from Medtronic, personal fees from Biotronik, outside the submitted work. JCN is supported by a grant from the Novo Nordisk Foundation, Denmark (NNF16OC0018658). HB is supported by the Innovation Fund Denmark (IFD) and NordForsk (through the funding to PM Heart (90580)). HKJ is supported by a grant from the Novo Nordisk Foundation, Denmark (NNF18OC0031258) and received lecture fees from Abbott, Denmark, and Biosense Webster, Europe.

Figures

Figure 1
Figure 1
The sex- and age distribution among patients at the time of pacemaker implantation from the Danish Pacemaker and Implantable Cardioverter Defibrillator Registry.
Figure 2
Figure 2
Flow chart of patient selection. Some mothers and fathers with atrioventricular block (AVB) also had siblings and consequently these individuals are registered as a parent as well as a sibling with AVB.
Figure 3
Figure 3
Adjusted rate ratio (RR) of atrioventricular block for first-degree relatives as function of age of the index relative.

Comment in

References

    1. 2015. DPaIRAr, 2015. Available: https://ssl.icddata.dk/download/Danish_Pacemaker_and_ICD_Register_Annual...
    1. Kumar S, Baldinger SH, Gandjbakhch E, et al. . Long-Term arrhythmic and Nonarrhythmic outcomes of lamin A/C mutation carriers. J Am Coll Cardiol 2016;68:2299–307. 10.1016/j.jacc.2016.08.058 - DOI - PubMed
    1. Liu G, Yang Z, Chen W, et al. . Novel missense variant in TTN cosegregating with familial atrioventricular block. Eur J Med Genet 2020;63:103752 10.1016/j.ejmg.2019.103752 - DOI - PubMed
    1. Rudbeck-Resdal J, Nielsen JC, Bundgaard H, et al. . Appropriate use of genetics in a young patient with atrioventricular block and family history of sudden cardiac death. HeartRhythm Case Rep 2019;5:169–72. 10.1016/j.hrcr.2018.12.004 - DOI - PMC - PubMed
    1. Zaytseva AK, Karpushev AV, Kiselev AM, et al. . Characterization of a novel SCN5A genetic variant A1294G associated with mixed clinical phenotype. Biochem Biophys Res Commun 2019;516:777–83 10.1016/j.bbrc.2019.06.080 - DOI - PubMed

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