EIGHTH INTERNATIONAL WORKSHOP ON CARDIAC ARRHYTHMIAS Methods: Brugada–type ECG in healthy men included in the Quebec Cardiovascular Study was assessed by systematic review of all (4374 men, 46±8 years old) ECGs done between 1974 and 1985. Classification of BS was made according to three different repolarization patterns: Type 1 is characterized by a coved ST-SE ≥2mm followed by a negative T-wave. Type 2 represents a high take-off ST-SE (≥2mm) with a descending ST-SE (remaining ≥1mm above the baseline) followed by a positive T-wave. Type 3 shows a right precordial ST-SE <1mm. Type 1 Type 2 Type 3 Results: No patient was found with the typical Brugada-type 1 pattern. Type 2 was present in 30 men (0.7%) and type 3 in 57 men (1.3%). After a follow- up of 14±4 years, 7% died from cardiovascular (CV) mortality including 3% from SCD. No CV mortality occurred in pts with the ECG BS-pattern. Conclusion: The typical BS-type 1 pattern is rare in native French Canadian. The BS-type 2 and 3 is much more prevalent but it does not appear to be associated with a high risk of SCD or bad prognosis in asymptomatic subjects. 11.4 BRUGADA SYNDROME. NATIONAL REGISTRY OF CUBA 2003 F. Dortic´ os, M. Dorantes, J. Castro, R. Zayas, M.A. Qui˜ nones, F. Torn´ es. Servicio de arritmias y estimulaci´ on el´ ectrica, Instituto de Cardiolog´ıa y Cirug´ıa Cardiovascular, Ciudad de la Habana, Cuba The Brugada syndrome is the most frequent cause of sudden cardiac death due to electrical causes in young individuals with no evidence of structural heart disease. It is characterized by ST segment elevation in right precordial leads, RBBB morphology and an increased predisposition to life-threatening arrhythmic events. This study has characterized all cases diagnosed during the period 1995–2002 in Cuba. Twenty-two apparently healthy individuals with the above mentioned ECG signs were studied, 10 symptomatic and 12 asymptomatic. Nineteen were male and 3 female, mean age of 44 years. Phar- macological testing with sodium channel blockers proved very useful (positive in all cases). The H-V interval was normal; life-threatening arrhythmic events were induced during programmed electrical stimulation in 3 symptomatic and 2 asymptomatic patients. Those and all other symptomatic patients received an implantable cardio-defibrillator (ICD). Clinical recurrences of life-threatening events occurred in 4 cases. Treatment with antiarrhythmic drugs was an exception. The clinical history and sequential ECG tracings are of great value for the diagnosis of this syndrome, however, minimal, transient and occult forms should be sort. The ICD is the treatment of choice. 11.5 DIAGNOSTIC VALUE OF AJMALINE TESTING IN ARRHYTHMOGENIC RIGHT VENTRICULAR DYSPLASIA-CARDIOMYOPATHY S. Peters, S. Denecke, M. Tr¨ ummel, B. Koehler. Klinikum Quedlinburg, Cardiology, Germany Right bundle branch block and coved ST elevation in V1-V3 were first described in a young patient with arrhythmogenic right ventricular dysplasia- cardiomyopathy (ARVD/C) after aborted sudden death in 1989. We performed ajmaline testing with 1mg/kg in 78 patients with ARVD/C and in a control group of 5 patients without structural heart disease. in 12 patients with ARVD/C (19%) a positive Brugada phenomenon was induced. Besides an increase in PQ and QTc interval there was an increase in QRS duration of up to 20 msec in the control group. The increase of QRS duration was 30–40 msec in ARVD/C without Brugada phenomenon and more than 40msec in ARVD/C with provocable Brugada phenomenon. Conclusions: the value of ajmaline testing in ARVD/C includes: discrim- ination of ARVD/C from a control group by the amount of QRS duration increase (diagnostic criterion) and provocation of Brugada phenomenon (risk stratification). 11.6 ELECTROPHYSIOLOGIC AND PROGNOSTIC CHARACTERISTICS OF DIFFERENT CATEGORIES OF PATIENTS AFFECTED BY THE BRUGADA SYNDROME. A PROSPECTIVE SINGLE CENTRE STUDY E. Marras 1 , P. Delise 1 , N. Sitta 1 , L. Coro’ 1 , M. Fantinel 2 , L. Sciarra 1 , F. Pianu 1 . 1 Operative Units of Cardiology, Hospital of Conegliano, Treviso, and 2 Feltre, Belluno, Italy Brugada Syndrome may be complicated by sudden death. The identification of patients at risk is an unresolved problem. Methods: from 1998 to 2003 we collected 38 patients (38 M, 8 F , mean age 41±12 years) with the type 1 or type 2 ecg Brugada pattern. Patients were divided into 3 groups: A. 11 patients with cardiac arrest (1) or syncope (10); B. 17 patients without symptoms but with a familiar history of symptomatic Brugada Syndrome or of juvenile (<45 years) sudden death; C. 10 patients without symptoms and no familiar history of symptomatic Brugada Syndrome or juvenile sudden death. 16 patients underwent the electrophysiologic study (EPS). After hospital discharge patients were prospectively followed for 36±18 months. Results: EPS induced sVT/FV in 58% (4/7) of Gr.A, 16% (1/6) of Gr. B and 30% (1/3) of Gr. C (Gr. A vs Gr. B+C, p=0.35). 5 pts (3 in Gr.A, 1 in Gr. B and 1 in Gr.C) received an ICD. During follow up 1 patient (of Gr.B, with EPS+, with an implanted ICD) had a resuscitated VF, while no patient died. Positive predictive value of EPS was 16% (1/6), negative predictive value was 100% (10/10). Considering all patients which had a resuscitated cardiac arrest and/or sudden death subjects at high risk of our series resulted 2/38 (7%). Conclusions: with the limits of the small number of patients and of a short follow up, our data suggest that 1. The risk of sudden death in the Brugada syndrome is low but not irrelevant (7%); 2. The positive predictive value of EPS is low but its negative predictive value is high; 3. Asymptomatic patients with a familial history of sudden death may be at high risk. 11.7 RISK STRATIFICATION IN THE BRUGADA SYNDROME. NON INVASIVE MARKERS J. Stanley, J. Alvarez, M. Dorantes, J. Castro, D. Barrera, R. Stusser, E. Rivas, F. Dortic´ os. Instituto de Cardiolog´ıa y Cirug´ıa Cardiovascular, Ciudad de la Habana, Cuba Since the Brugada syndrome has been recognized as a separate entity, in- vasive and non-invasive markers have been searched for detecting the risk of life-threatening arrhythmic events, particularly in asymptomatic individuals where the first manifestation may be sudden cardiac death. This study has compared the power of various non-invasive markers in the risk stratification of ventricular fibrillation (VF) and sudden cardiac death. Late potentials, QT interval, QT dispersion and heart rate variability were analyzed over a 2 year period, in 20 patients (17 male and 3 female, mean age of 44 years) with the Brugada syndrome (symptomatic or asymptomatic), compared with 20 normal individuals similar in age and sex. Late potentials were present in 80% of patients versus 5% in the control group; 88% of symptomatic patients, all with recurrent episodes, had late potentials, and in 4 of 5 patients stimulation induced VF or polymorphic ventricular tachycardia. Late potentials were the most important non-invasive marke rs for risk stratification and inducibility, with an incidence significantly higher in patients (p<0.0005) than in controls. Invasive and non-invasive markers should be considered integrally, for a better approach to the reality. 11.8 UTILITY OF THE I.V. PROCAINAMIDE TEST TO UNMASK A CONCEALED FORM OF BRUGADA SYNDROME J. Champagne, G. O’Hara, F. Molin, F. Philippon, M. Chahine, P. Poirier, L. Charbonneau, M. Gilbert. Quebec Heart Institute, Quebec City, Canada Brugada syndrome (BS) is characterized by an ECG showing a right bundle branch block (RBBB) pattern with a ST-segment elevation (SE) in leads V1 to V3. The ECG manifestations of BS are often transient in many patients (pts) and Class 1 antiarrhythmics such as procainamide (P) can unmask the ST-SE in pts with normal baseline ECG. The role of the P test in different clinical settings is not well defined. Methods: P (10 mg/kg I.V), in combination with higher V1-V2 lead positioning, was administered to 43 pts without structural heart disease (45±15 years old; 70% male). Twenty pts had previous syncope or palpitations and showed an incomplete RBBB in V1 without ST-SE, unexplained sudden death (SD) or BS in family members was present in 9 pts and 14 pts were investigated for idiopathic ventricular fibrillation (VF). Results: the P test was positive (ST-SE >2mm in leads V1-V2) in 8 pts (19%). BS was diagnosed in 4 pts with clinical history of syncope or Europace Supplements, Vol. 4, October 2003 A19 Downloaded from https://academic.oup.com/europace/article-abstract/4/Supplement_1/A19/483540 by guest on 30 July 2018