Objectives Pedro and Joseph Brugada introduced the Brugada syndrome in 1992. This potential fatal syndrome occurs in children as well, but few paediatricians are familiar. Paediatric patients presumably diagnosed with Brugada syndrome need guidance through electrophysiological investigations, may need proper anaesthesia during investigation or therapy, and resuscitation after electrophysiological challenge, ablation therapy or device implantation may occur.

Methods We report on the clinical experience with paediatric patients suspected of Brugada syndrome in our Hospital, that were electrophysiologically investigated by Brugada himself with sodium channel blocking agents. Some were treated by ablation therapy, pacemaker or implantable cardioverter defibrillator. These children need monitoring and a standby anesthesiologist.

Results We review Brugada syndrome in children: etiology, clinical features and typical ECG. We emphasise on the importance of psychophysical preparation of the patient and safety management during provocation testing. Anaesthesia may be necessary: we describe the best anticipating attitude if Brugada syndrome or VT/VF occurs after challenge. Several drugs are found to trigger Brugada syndrome and are contraindicated. Possible definitive treatment strategies for the child with Brugada syndrome are discussed. We add practical tips for patients and parents.

Conclusions Brugada syndrome is increasingly recognized, also in paediatric patients. Awareness of all paediatric critical care physicians and anaesthesiologists should improve. In suspection of Brugada syndrome, prompt cardiac evaluation, electrophysiological investigation and adapted therapy are mandatory. Paediatric intensivist, anaesthesiologist and cardiologist should collaborate closely, and all should be informed on a regular base on characteristics, treatment, anticipating techniques and reanimation of a patient with Brugada syndrome.