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Case Reports
. 2025 Jul 23;30(20):104144.
doi: 10.1016/j.jaccas.2025.104144.

Cardio-Embolic Stroke in a 14-Year-Old Boy Due to SCN5A-Associated Atrial Standstill

Ali Bahbah  1 , Jay Sengupta  2 , Morgan Kelly  3 , Elizabeth Bade  3 , Dawn Witt  4 , Charles Gornick  5 , Joao Cavalcante  5 , John Zakaib  5
Affiliations
Case Reports

Cardio-Embolic Stroke in a 14-Year-Old Boy Due to SCN5A-Associated Atrial Standstill

Ali Bahbah et al. JACC Case Rep. .

Abstract

Background: Atrial standstill is a rare pathologic phenomenon characterized by absence of electrical and mechanical activity of one or both atria. SCN5A gene mutations are associated with a myriad of clinical cardiac phenotypes including sinus node dysfunction and atrial standstill.

Case summary: We present a case of a 14-year-old boy who suffered acute embolic stroke in the setting of SNC5A-associated atrial standstill. The patient required thrombolysis and interventional thrombectomy, after which he recovered completely.

Discussion: To our knowledge, this is the first report of acute embolic stroke as the initial presentation of SCN5A-associated partial atrial standstill in the pediatric population. SCN5A-associated atrial standstill carries a significant risk of stroke and mortality in pediatric patients who are not identified and managed appropriately.

Take-home messages: Atrial standstill can present with serious cardioembolic complications in pediatric patients. Prevention using oral anticoagulation should be considered in patients with partial atrial standstill, especially when associated with SCN5A variants and in the absence of atrial pacing.

Keywords: SCN5A; atrial standstill; pacemaker; pediatrics; stroke.

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Conflict of interest statement

Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Figures

None
Graphical abstract
Figure 1
Figure 1
Patient's Initial Electrocardiogram at Presentation, Showing Regular Junctional Rhythm With Absent P Waves
Figure 2
Figure 2
Computed Tomography Angiography Computed tomography angiography showing acute occlusion of cervical internal carotid (A) artery up to M1 segment of the middle cerebral artery (B).
Figure 3
Figure 3
Brain Magnetic Resonance Imaging Showing Acute Infarct of the Right Basal Ganglia
Figure 4
Figure 4
Patient’s Telemetry During Hospitalization First 2 recordings showing junctional rhythm, absent P waves, and prolonged pause. Bottom recording showing normal sinus rhythm, indicating the transient nature of atrial standstill.
Figure 5
Figure 5
Patient's Holter Monitoring After Discharge Showing Junctional Rhythm and Sinus Pauses Up to 8 Seconds
Figure 6
Figure 6
Genetic Testing Showing Positive Result for a Heterozygous Pathogenic SCN5A Gene Variant, the Same as the One Carried by His Paternal Uncle as Well as Another SCN5A Heterozygous Variant of Undetermined Significance
See this image and copyright information in PMC

References

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