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

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.

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.

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.

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.