Successful Ablation of Hemodynamically Stable Monomorphic VT in a 5-Year-Old Boy with Brugada's Syndrome

Objectives: Brugada's syndrome (BrS) is an inherited arrhythmic disease that may cause potentially fatal ventricular tachyarrhythmias, mainly in males between their fourth and fifth decades of life. Currently, the published data on children and adolescents with BrS and arrhythmic events (AEs) and their management are limited.

Methods: Case presentation: we report a 5- year-old boy who presented with symptoms of fatigue and abdominal pain with vomiting combined with signs of viral infection of the upper airways without fever in the emergency room. A monomorphic ventricular tachycardia (VT) with inferior axis, left bundle-branch-block morphology, tachycardia cycle length of 260 ms was documented. VT was resistant to drugs (amiodaron, β-blocker), as well as to several episodes of electrical cardioversions, and lasted for several hours before it terminated spontaneously. Further diagnostic examinations (echocardiography, catheter examination with biopsy) did not reveal any pathological findings. MRI revealed mild noncompaction cardiomyopathy of the LV apex and the left lateral wall. Epinephrin-challenge was pathological (QTc 550 ms) and ajmalin challenge showed BrS type-1 ECG. He was initially treated with β-blockers and a loop recorder (ILR) was implanted. After documentation of nonsymptomatic recurrent episodes of nonsustained VT on ILR, an electrophysiological study was performed. In the right ventricular outflow tract, fractionated electrograms could be documented. Positive pacemapping (12/12 leads) and a local early activation of −36 ms indicated this location as the potential origin of the VT. Subsequently, endocardial radiofrequency ablation (RFA) was successful at this site. After RFA, no more VT or PVCs were inducible. In the follow-up period of actually 4 months, no VT was documented by ILR. Meanwhile, the genetic testing showed three different polymorphisms (Gen-locations: KCNH2, SCN1B, SCN5A), the results of the genetic examination of the family members are still pending.

Conclusion: In conclusion, we assume that in children with hemodynamically stable monomorphic VT and BrS type-1 ECG, radiofrequency ablation can be a therapeutical option and can probably delay the implantation of an ICD.

No conflict of interest has been declared by the author(s).

Publication History

Article published online:
21 February 2021