Background: Brugada syndrome (BrS) is considered a purely electrical disease with variable electrical substrates. Variable rates of mechanical abnormalities have been also reported. Whether exists a link between electrical and mechanical abnormalities has never been previously explored. This investigational physiopathological study aimed to determine the relationship between the substrate size/location, as exposed by ajmaline provocation, and the severity of mechanical abnormalities, as assessed by cardiac magnetic resonance in patients with BrS.Methods: Twenty-four consecutive high-risk patients with BrS (mean age, 3811 years, 17 males), presenting with malignant syncope and documented polymorphic ventricular tachycardia/ventricular fibrillation, and candidate to implantable cardioverter defibrillator implantation, underwent cardiac magnetic resonance and electroanatomic maps. During each examination, ajmaline test (1 mg/kg over 5 minutes) was performed. Cardiac magnetic resonance findings were compared with 24 age, sex, and body surface area-matched controls. In patients with BrS, the correlation between the electrical substrate extent and right ventricular regional mechanical abnormalities before/after ajmaline challenge was analyzed.Results: After ajmaline, patients with BrS showed a reduction of right ventricular (RV) ejection fraction (P<0.001), associated with decreased transversal displacement (U, P<0.001) and longitudinal strain (epsilon, P<0.001) localized at RV outflow tract. In patients with BrS significant preajmaline/postajmaline changes of transversal displacement (U, P<0.001) and longitudinal strain (epsilon, P<0.001) were found. In the control group, no mechanical changes were observed after ajmaline. The electrical substrate consistently increased after ajmaline from 1.72.8 cm(2) to 14.27.3 cm(2) (P<0.001), extending from the RV outflow tract to the neighboring segments of the RV anterior wall. Postajmaline RV ejection fraction inversely correlated with postajmaline substrate extent (r=-0.830, P<0.001). In patients with BrS and normal controls, cardiac magnetic resonance detected neither myocardial fibrosis nor RV outflow tract morphological abnormalities.Conclusions: BrS is a dynamic RV electromechanical disease, where functional abnormalities correlate with the maximal extent of the substrate size. These findings open new lights on the physiopathology of the disease.Registration: URL: https://clinicaltrial.gov; Unique identifier: NCT03524079.
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