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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.
- Source :
-
Circulation journal : official journal of the Japanese Circulation Society [Circ J] 2017 Dec 25; Vol. 82 (1), pp. 53-61. Date of Electronic Publication: 2017 Aug 04. - Publication Year :
- 2017
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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 (I <subscript>Na</subscript> ) 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%I <subscript>Na</subscript> reduction have a longer RVED than patients carrying missense variants (M) with different degrees ofI <subscript>Na</subscript> reduction.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.<br />Conclusions: Using the simple, non-invasive echocardiographic parameter RVED revealed a more pronounced 'electromechanical' delay in BrS patients carrying T variants ofSCN5A.
- Subjects :
- Adult
Brugada Syndrome diagnostic imaging
Codon, Nonsense
Cross-Sectional Studies
Echocardiography
Electrochemical Techniques
Female
Genotype
Heart Conduction System diagnostic imaging
Heart Conduction System physiopathology
Humans
Male
Middle Aged
Mutation, Missense
Phenotype
Time Factors
Ventricular Dysfunction, Right diagnostic imaging
Brugada Syndrome physiopathology
NAV1.5 Voltage-Gated Sodium Channel genetics
Ventricular Dysfunction, Right physiopathology
Subjects
Details
- Language :
- English
- ISSN :
- 1347-4820
- Volume :
- 82
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- Circulation journal : official journal of the Japanese Circulation Society
- Publication Type :
- Academic Journal
- Accession number :
- 28781330
- Full Text :
- https://doi.org/10.1253/circj.CJ-16-1279