title: Long-Term Follow-Up of Probands With Brugada Syndrome.
abstract: This study analyzes the natural history of a large cohort of probands with Brugada syndrome (BrS) to assess the predictive value of different clinical and electrocardiographic parameters for the development of ventricular fibrillation (VF) or sudden cardiac death (SCD) during a long-term follow-up. Baseline characteristics of 289 consecutive probands (203 men; mean age 45 ± 16 years) with a Brugada type 1 electrocardiogram were analyzed. After a mean follow-up of 10.1 ± 4.6 years, 29 malignant arrhythmias occurred. On multivariate analysis, a history of VF and syncopal episodes, fragmented QRS (f-QRS), spontaneous type 1 electrocardiogram, and early repolarization pattern were significantly associated with later occurrence of VF/SCD. In patients with drug-induced BrS, the accentuation or de novo appearance of f-QRS in other leads was always associated with VF/SCD. Cerebrovascular events occurred in 8 patients with atrial fibrillation (15.1%), most of them (75%) presenting as the first clinical manifestation. The time-to-diagnosis was found to be significantly shorter in those patients who directly came to our center than in those who referred to our center for a second opinion. In conclusion, systematic use of the pharmacologic challenge in patients with unexplained cardiovascular symptoms and/or atrial fibrillation might significantly improve the identification of BrS with a shortening of the time-to-diagnosis. The CHA2DS2VASc score might be inappropriate for predicting transient ischemic attack or stroke in BrS. This study confirms the independent predictive value of previous VF and syncopal episodes, f-QRS, type 1 electrocardiogram, and early repolarization pattern. In BrS a sufficiently long follow-up is necessary before conclusions on prognosis are apparent.
authors.affiliation: Cardiovascular Department, Heart Rhythm Management Center, Postgraduate Program in Cardiac Electrophysiology and Pacing, Universitair Ziekenhuis Brussel, Vrije Universiteit Brussel, Brussels, Belgium. Electronic address: carlodeasmundis@me.com.
authors.forename: Carlo
authors.initials: C
authors.lastname: de Asmundis
authors.affiliation: Cardiovascular Department, Heart Rhythm Management Center, Postgraduate Program in Cardiac Electrophysiology and Pacing, Universitair Ziekenhuis Brussel, Vrije Universiteit Brussel, Brussels, Belgium.
authors.forename: Giacomo
authors.initials: G
authors.lastname: Mugnai
authors.affiliation: Cardiovascular Department, Heart Rhythm Management Center, Postgraduate Program in Cardiac Electrophysiology and Pacing, Universitair Ziekenhuis Brussel, Vrije Universiteit Brussel, Brussels, Belgium.
authors.forename: Gian-Battista
authors.initials: GB
authors.lastname: Chierchia
authors.affiliation: Cardiovascular Department, Heart Rhythm Management Center, Postgraduate Program in Cardiac Electrophysiology and Pacing, Universitair Ziekenhuis Brussel, Vrije Universiteit Brussel, Brussels, Belgium.
authors.forename: Juan
authors.initials: J
authors.lastname: Sieira
authors.affiliation: Cardiovascular Department, Heart Rhythm Management Center, Postgraduate Program in Cardiac Electrophysiology and Pacing, Universitair Ziekenhuis Brussel, Vrije Universiteit Brussel, Brussels, Belgium.
authors.forename: Giulio
authors.initials: G
authors.lastname: Conte
authors.affiliation: Cardiovascular Department, Heart Rhythm Management Center, Postgraduate Program in Cardiac Electrophysiology and Pacing, Universitair Ziekenhuis Brussel, Vrije Universiteit Brussel, Brussels, Belgium.
authors.forename: Moises
authors.initials: M
authors.lastname: Rodriguez-Mañero
authors.affiliation: Cardiovascular Department, Heart Rhythm Management Center, Postgraduate Program in Cardiac Electrophysiology and Pacing, Universitair Ziekenhuis Brussel, Vrije Universiteit Brussel, Brussels, Belgium.
authors.forename: Gudrun
authors.initials: G
authors.lastname: Pappaert
authors.affiliation: Cardiac Surgery Department, Universitair Ziekenhuis Brussel, Vrije Universiteit Brussel, Brussels, Belgium.
authors.forename: Jens
authors.initials: J
authors.lastname: Czapla
authors.affiliation: Cardiac Surgery Department, Universitair Ziekenhuis Brussel, Vrije Universiteit Brussel, Brussels, Belgium.
authors.forename: Jan
authors.initials: J
authors.lastname: Nijs
authors.affiliation: Cardiac Surgery Department, Universitair Ziekenhuis Brussel, Vrije Universiteit Brussel, Brussels, Belgium.
authors.forename: Mark
authors.initials: M
authors.lastname: La Meir
authors.affiliation: Cardiovascular Department, Heart Rhythm Management Center, Postgraduate Program in Cardiac Electrophysiology and Pacing, Universitair Ziekenhuis Brussel, Vrije Universiteit Brussel, Brussels, Belgium.
authors.forename: Ruben
authors.initials: R
authors.lastname: Casado
authors.affiliation: Cardiovascular Department, Heart Rhythm Management Center, Postgraduate Program in Cardiac Electrophysiology and Pacing, Universitair Ziekenhuis Brussel, Vrije Universiteit Brussel, Brussels, Belgium.
authors.forename: Erwin
authors.initials: E
authors.lastname: Ströker
authors.affiliation: Cardiovascular Department, Heart Rhythm Management Center, Postgraduate Program in Cardiac Electrophysiology and Pacing, Universitair Ziekenhuis Brussel, Vrije Universiteit Brussel, Brussels, Belgium.
authors.forename: Valentina
authors.initials: V
authors.lastname: De Regibus
authors.affiliation: Cardiovascular Department, Heart Rhythm Management Center, Postgraduate Program in Cardiac Electrophysiology and Pacing, Universitair Ziekenhuis Brussel, Vrije Universiteit Brussel, Brussels, Belgium.
authors.forename: Pedro
authors.initials: P
authors.lastname: Brugada
mesh_terms: D000328:Adult; D000368:Aged; D001281:Atrial Fibrillation / Q000453:epidemiology*; D001530:Belgium / Q000453:epidemiology; D053840:Brugada Syndrome / Q000175:diagnosis / Q000453:epidemiology* / Q000503:physiopathology / Q000628:therapy; D016757:Death, Sudden, Cardiac / Q000453:epidemiology* / Q000517:prevention & control; D017147:Defibrillators, Implantable; D057210:Delayed Diagnosis; D004562:Electrocardiography; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D011446:Prospective Studies; D018570:Risk Assessment; D020521:Stroke / Q000453:epidemiology*; D014693:Ventricular Fibrillation / Q000453:epidemiology*