AEPC 2022 - 55th Annual Meeting of the Association for European Paediatric and Congenital Cardiology , Geneve, İsviçre, 25 - 28 Mayıs 2022, ss.485-486
BACKGROUND AND AIM: Brugada syndrome(BrS), an inherited arrhythmogenic disease which can
cause syncope or sudden cardiac death(SCD), is characterized by ST-segment elevation in right
precordial leads. Sodium channel blocking drugs(Ajmaline, Flecainide) are used to reveal ECG
findings in unidentified forms. In this study, we aimed to share our clinical experience of pediatric
patients who were suspected of BrS and performed Ajmaline provocation test (APT).
METHODS: The data of 78 patients who underwent APT between 2013-2021 were analyzed
retrospectively. In the test protocol, Ajmaline was given to each patient as a six to ten-minute
infusion (1mg/kg). 12-channel ECG recordings were taken at the beginning of the test and at twominute
intervals. During this whole period, the test was considered positive in case of the
appearance of type-1 Brugada sign, especially on lead V1 and V2.
RESULTS: 51/78(65%) patients included in the study were male. The mean age at admission was
13.02±4.34(0.98-21.27)years and the mean body weight was 47.06±19.48(9-95)kg. On admission 15
patients(19.2%) had syncope, 15(19.2%) had palpitations and 15(19.2%) had a history of cardiac
arrest. 20(25.6%) patients had a family history of sudden death. 12 lead basic ECG, suspected
Brugada sign was observed in 23 patients, borderline long QT interval in 3 patients, and right bundle
branch block in 2 patients. Echocardiographically revealed normal cardiac findings except
asymmetric septal hypertrophy in one patient and mitral valve prolapse in another. APT was positive
in 13/78 (16.6%) patients (Table-1) and there were no arrhythmias or adverse events during testing.
In 7 (53.8%) of the 13 patients with a positive test result, ICD was implanted (syncope (n=6) and
cardiac arrest (n=1)). During to mean 5 years follow-up period, there was no major arrhythmic event
in these 13 patients.
CONCLUSIONS: APT test can be performed safely and effectively in suspected BrS, aborted cardiac
arrest, and a family history of sudden death. In positive cases; medical treatment±ICD decision
should be made by evaluating together with other clinical findings and risk factors.
Keywords: Brugada syndrome, pediatric, Ajmaline, provocation test,