Objective: To investigate the correlation between SCN5A gene mutation and arrhythmia in children. Methods: Clinical data of 8 patients with arrhythmia complicated with SCN5A gene mutation diagnosed and treated in our hospital from Jan. 2018 to Jun. 2020 were retrospectively analyzed. Results: Among the 8 patients

7 were males and 1 was female

with age of 5. 5 ( from 2. 5 to 7. 0) years. Syncope was the main clinical manifestation

with an incidence of 87. 5% (7 / 8). One case of syncope was related to sinus arrest and atrial flutter

and 6 cases were related to ventricular tachycardia. Combined with electrocardiogram and gene examination in 6 cases of ventricular tachycardia

4 cases were diagnosed as Brugada syndrome (Brs)

1 case was type 3 long QT interval syndrome (LQT3)

and 1 case was Brs complicated with LQT3. Three patients had significant decrease of left ventricular function. Conclusion: SCN5A gene mutation is associated with malignant arrhythmia in children

which needs to be paid attention to. If necessary

genome sequencing should be carried out as soon as possible for accurate treatment.