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European Heart Journal 2001 22(5):400-409; doi:10.1053/euhj.2000.2366
Copyright © 2001 by the European Society of Cardiology.
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Diagnosis and long-term follow-up of the Brugada syndrome in patients with idiopathic ventricular fibrillation

C.A Remmea,b,f1, E.F.D Wevera, A.A.M Wildea,b, R Derksena and R.N.W Hauera

a Department of Cardiology, Heart-Lung Centre Utrecht, The Netherlands
b Experimental and Molecular Cardiology Group, Academic Medical Centre, University of Amsterdam, The Netherlands

revised July 3, 2000; accepted July 12, 2000

Abstract

Aims Some patients with idiopathic ventricular fibrillation may suffer from the Brugada syndrome. The diagnostic criteria for the Brugada syndrome are uncertain and arbitrarily set. Therefore, we studied the prevalence of the Brugada syndrome using various diagnostic criteria and long-term follow-up in 37 idiopathic ventricular fibrillation patients.

Methods and Results Idiopathic ventricular fibrillation was diagnosed after thorough clinical evaluation in 37 survivors of an out-of-hospital cardiac arrest referred to our institute (UMC Utrecht). Retrospectively, nine patients (24%, group I) were classified as potentially having the Brugada syndrome based on the presence of (in)complete right bundle branch block and ST-segment elevation in leads V1–V3of ≥1mm. Only three patients (8%, group II) showed (in)complete right bundle branch block and ≥2mm ST-segment elevation. With the intermittent presence of these ECG features and/or their (re)appearance with class I antiarrhythmic drugs included as criteria, the percentage of the Brugada syndrome was attenuated in group I (2/37; 5%) and group II (1/37; 3%). Sixteen (43%) of all idiopathic ventricular fibrillation patients (mean follow-up 77±41 months) had a recurrent episode of syncope, ventricular tachyarrhythmias or sudden death. Recurrence rate was 3/9 (33%) in Brugada patients group I, 2/3 (66%) in group II and 13/28 (46%) in patients without the Brugada syndrome (P=ns).

Conclusions Depending on the diagnostic criteria used, the Brugada syndrome was observed in 3% to 24% of patients with idiopathic ventricular fibrillation, underlining the importance of defining the precise diagnostic criteria in these patients. For all idiopathic ventricular fibrillation patients, the ventricular tachyarrhythmia recurrence rate was substantial during an average follow-up of more than 6 years.

Key Words: Sudden cardiac death, idiopathic ventricular fibrillation, Brugada syndrome, follow-up

f1 Correspondence: C. A. Remme, MD, Laboratory of Experimental Cardiology, Academic Medical Centre, Room M0-052, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands.


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