Prolonged Right Ventricular Ejection Delay in Brugada Syndrome Depends on the Type of SCN5A Variant - Electromechanical Coupling Through Tissue Velocity Imaging as a Bridge Between Genotyping and Phenotyping

Sophie C H Van Malderen, Dorien Daneels, Dirk Kerkhove, Uschi Peeters, Dominic A M J Theuns, Steven Droogmans, Guy Van Camp, Caroline Weytjens, Martine Biervliet, Maryse Bonduelle, Sonia Van Dooren, Pedro Brugada

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

BACKGROUND: Patients with Brugada syndrome (BrS) and a history of syncope or sustained ventricular arrhythmia have longer right ventricular ejection delays (RVEDs) than asymptomatic BrS patients. Different types ofSCN5Avariants leading to different reductions in sodium current (INa) may have different effects on conduction delay, and consequently on electromechanical coupling (i.e., RVED). Thus, we investigated the genotype-phenotype relationship by measuring RVED to establish whether BrS patients carrying more severeSCN5Avariants leading to premature protein truncation (T) and presumably 100%INareduction have a longer RVED than patients carrying missense variants (M) with different degrees ofINareduction.Methods and Results:There were 34 BrS patients (mean [±SD] age 43.3±12.9 years; 52.9% male) carrying anSCN5Avariant and 66 non-carriers in this cross-sectional study. Patients carrying aSCN5Avariant were divided into T-carriers (n=13) and M-carriers (n=21). Using tissue velocity imaging, RVED and left ventricular ejection delay (LVED) were measured as the time from QRS onset to the onset of the systolic ejection wave at the end of the isovolumetric contraction. T-carriers had longer RVEDs than M-carriers (139.3±15.1 vs. 124.8±11.9 ms, respectively; P=0.008) and non-carriers (127.7±17.3 ms, P=0.027). There were no differences in LVED among groups.

CONCLUSIONS: Using the simple, non-invasive echocardiographic parameter RVED revealed a more pronounced 'electromechanical' delay in BrS patients carrying T variants ofSCN5A.

Original languageEnglish
Pages (from-to)53-61
Number of pages9
JournalCirculation Journal
Volume82
Issue number1
Early online date4 Aug 2017
DOIs
Publication statusPublished - 25 Dec 2017

Keywords

  • Brugada syndrome
  • Conduction delay
  • Right ventricular ejection delay
  • SCN5A

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