Altered right ventricular electromechanical conduction in Brugada syndrome

Student thesis: Doctoral Thesis

Abstract

This thesis showed that simple, non-invasive echocardiographic parameters representing conduction delay (right ventricular ejection delay (RVED), RV contraction duration (RVCdur)) and mechanical dispersion (SD, Delta-RVCdur) are prolonged in male patients with Brugada syndrome (BS) along with an unchanged myocardial shortening amplitude. Moreover, the increased conduction delay and mechanical dispersion in BS males is more pronounced in those with previous malignant arrhythmic events.
Whereas up to now only the ‘repolarization’ hypothesis provided an explanation for the predominant male malignant phenotype in BS, our group has now supported this male predominance mechanically through different parameters of conduction delay: RVED, RVCdur, SD and Delta-RVCdur.
Furthermore, we established that BS patients carrying more severe SCN5A variants leading to premature protein truncation (T) and presumably 100% INa reduction, had a longer RVED than patients carrying missense variants (M) with different degrees of INa reduction.
In conclusion, we report a high number of patients with coexisting BS and skeletal sodium channel myotonia. Our findings suggest a possible impact of SCN4A variants on the pathophysiological mechanism underlying the development of a type 1 ECG pattern and of malignant arrhythmia symptoms in some patients with BS
Date of Award28 Jun 2018
Original languageEnglish
Awarding Institution
  • Vrije Universiteit Brussel
SupervisorSteven Droogmans (Promotor), Pedro Brugada (Co-promotor), Brigitte Velkeniers-Hoebanckx (Jury), Bernard Cosyns (Jury), Jan Poelaert (Jury), Carlo De Asmundis (Jury), Tamas Szili-Torok (Jury), Yves De Greef (Jury) & Julie De Backer (Jury)

Keywords

  • Brugada Syndrome
  • echocardiography

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