Long-term evolution of echocardiography parameters in Brugada syndrome patients

Background Brugada syndrome is a heritable disorder with a high risk of sudden death. Although being a primary electrical disorder, subtle structural changes have repeatedly been described. Little is known about the long-term structural evolution in Brugada syndrome. Purpose We aimed to assess the evolution of echocardiographic parameters in Brugada syndrome and the presence of markers of structural evolution. Methods Brugada syndrome patients with minimum two complete transthoracic echocardiographic examinations were included. Clinical data were collected retrospectively. We analyzed all available echocardiographic examinations and assessed the evolution of cardiac parameters over time and its association with patient characteristics. Results The study included 113 Brugada syndrome patients [47 (33-55) years, 59 (52%) female, spontaneous type 1 ECG in 26 (23%)], with a total of 258 echocardiographies, collected over a period of 6.8 (6.6-7.1) years. Proximal and distal right ventricular outflow tract and basal right ventricular diameter dilated at a rate of 0.2 (0.1-0.3) mm/year (p=0.004), 0.4 (0.3-0.5) mm/year (p<0.001) and 0.6 (0.4-0.8) mm/year (p<0.001) respectively (Figure). Left ventricular mechanical dispersion increased from 38 (±11) ms to 42 (±12) ms (p=0.001). Neither male sex, nor presenting a spontaneous type 1 ECG pattern were associated with worse structural evolution. Conclusion We observed progressive right ventricular outflow tract and basal right ventricle dilation and an increasing contractile dyssynchrony in Brugada syndrome, potentially indicating a progressive structural affection. Further research is warranted to unravel underlying mechanisms and possible clinical implications of progressive structural changes in Brugada syndrome.