Maurizio Pieroni1, Pasquale Notarstefano2, Antonio Oliva3, Oscar Campuzano4, Pasquale Santangeli5, Monica Coll6, Martina Nesti2, Andrea Carnevali7, Aureliano Fraticelli2, Anna Iglesias6, Simone Grassi3, Ramon Brugada8, Leonardo Bolognese2. 1. Cardiovascular Department, San Donato Hospital, Arezzo, Italy. Electronic address: mauriziopieroni@yahoo.com. 2. Cardiovascular Department, San Donato Hospital, Arezzo, Italy. 3. Institute of Public Health, Legal Medicine Section, Catholic University, Rome, Italy. 4. Cardiovascular Genetics Centre, University of Girona-IDIBGI, Girona, Spain; Centro Investigación Biomédica en Red, Enfermedades Cardiovasculares (CIBERCV), Madrid, Spain; Medical Science Department, School of Medicine, University of Girona, Girona, Spain. 5. Cardiovascular Division, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania. 6. Cardiovascular Genetics Centre, University of Girona-IDIBGI, Girona, Spain. 7. Pathology Department, San Donato Hospital, Arezzo, Italy. 8. Cardiovascular Genetics Centre, University of Girona-IDIBGI, Girona, Spain; Centro Investigación Biomédica en Red, Enfermedades Cardiovasculares (CIBERCV), Madrid, Spain; Medical Science Department, School of Medicine, University of Girona, Girona, Spain; Cardiology Service, Hospital Josep Trueta, University of Girona, Girona, Spain.
Abstract
BACKGROUND: The prevalence and significance of structural abnormalities in Brugada syndrome (BrS) are still largely debated. OBJECTIVES: The authors investigated the relationship between genetic background, electroanatomic abnormalities, and pathologic substrate in BrS. METHODS: They performed 3-dimensional electroanatomic unipolar and bipolar mapping in 30 patients with BrS. Twenty patients underwent 3-dimensional electroanatomic unipolar and bipolar mapping-guided right ventricular outflow tract (RVOT) endomyocardial biopsy. Programmed ventricular stimulation and genetic analysis were performed in all patients. RESULTS: Low-voltage areas (LVAs) were observed at unipolar map in 93% of patients and at bipolar map in 50% of cases. Unipolar LVAs were always larger than bipolar LVAs, were always colocalized, and in all cases included RVOT. Disease-causing mutations were detected in 10 (33%) patients. Programmed ventricular stimulation was positive in 16 cases (53%). In 75% of patients, RVOT histology showed pathologic findings with myocardial inflammation in 80% of them. Among patients with abnormal bipolar map submitted to endomyocardial biopsy, 9 (81%) showed evidence of myocardial inflammation. Conversely, bipolar map was abnormal in 83% of patients with myocardial inflammation. Myocardial inflammation was also more prevalent among inducible patients (83% vs. 25% in noninducible; p = 0.032). CONCLUSIONS: BrS is characterized by electroanatomical and structural abnormalities localized to RVOT with a gradient of the pathologic substrate from epicardium to endocardium possibly driven by myocardial inflammation. These findings reclassify BrS as a combination of structural and electrical defects opening the way to new risk stratification and therapeutic strategies.
BACKGROUND: The prevalence and significance of structural abnormalities in Brugada syndrome (BrS) are still largely debated. OBJECTIVES: The authors investigated the relationship between genetic background, electroanatomic abnormalities, and pathologic substrate in BrS. METHODS: They performed 3-dimensional electroanatomic unipolar and bipolar mapping in 30 patients with BrS. Twenty patients underwent 3-dimensional electroanatomic unipolar and bipolar mapping-guided right ventricular outflow tract (RVOT) endomyocardial biopsy. Programmed ventricular stimulation and genetic analysis were performed in all patients. RESULTS: Low-voltage areas (LVAs) were observed at unipolar map in 93% of patients and at bipolar map in 50% of cases. Unipolar LVAs were always larger than bipolar LVAs, were always colocalized, and in all cases included RVOT. Disease-causing mutations were detected in 10 (33%) patients. Programmed ventricular stimulation was positive in 16 cases (53%). In 75% of patients, RVOT histology showed pathologic findings with myocardial inflammation in 80% of them. Among patients with abnormal bipolar map submitted to endomyocardial biopsy, 9 (81%) showed evidence of myocardial inflammation. Conversely, bipolar map was abnormal in 83% of patients with myocardial inflammation. Myocardial inflammation was also more prevalent among inducible patients (83% vs. 25% in noninducible; p = 0.032). CONCLUSIONS: BrS is characterized by electroanatomical and structural abnormalities localized to RVOT with a gradient of the pathologic substrate from epicardium to endocardium possibly driven by myocardial inflammation. These findings reclassify BrS as a combination of structural and electrical defects opening the way to new risk stratification and therapeutic strategies.
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