Журнал синдрома Дауна и хромосомных аномалий
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Sinus Node Dysfunction in People having Down Syndrome with Structurally Normal Hearts: A Case Report

Rachael Cordina2,4*

Down Syndrome (DS) is the most common chromosomal abnormality in live born infants. Although limited research suggests that autonomic dysfunction may be more common in the setting of DS, a predisposition to cardiac conduction abnormalities is not well recognized. We performed a retrospective review of records at our tertiary referral centre from Jan 2018 to Jan 2021 to identify people who had Down’s syndrome and required permanent pacemaker implantation for cardiac conduction disease. Inclusion criteria were age ≥ 18 years, and without a structural cardiac abnormality. Three individuals were identified aged between 41 and 59 years; two were female and one was male. All presented with recurrent syncope with sinus pauses (2-10 seconds) identified on Holter monitor (n=2) or loop recorder (n=1). All three patients underwent dual chamber permanent pacemaker insertion under general anesthesia with no short or long term complications over the follow-up period (11-46 months). One of the three patients had mild self-limiting pre-syncopal episodes three months post-pacemaker implantation which resolved following the commencement of 100 mcg fludrocortisone daily. Young people with DS may be predisposed to sinus node dysfunction; rhythm monitoring is recommended in the setting of suspicious symptoms. People who have Down Syndrome may have an increased risk for sinus node dysfunction compared with the general population. Heart rhythm assessment should be considered in the setting of suspicious symptoms.