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September 17, 2012Clinical Genetics

A study of the SCN5A gene in a cohort of 76 patients with Brugada syndrome

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Why the study?

Does SCN5A mutation status correlate with clinical phenotype and prognosis in patients with Brugada syndrome?

Population

76 non-related patients with Brugada syndrome

Comparison

Direct sequencing and multiplex… vs Wild-type probands

Design

Cohort

Key result

In patients with Brugada syndrome, SCN5A mutations were associated with a trend toward more spontaneous type I ECGs compared to wild-type probands (87.5% vs 52.9%, p=0.06).

Authors

EGEsperanza García-MolinaJLJavier LacunzaFEFrancisco Ruiz Espejo

Discussion

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Overview

SCN5A mutations should not guide risk stratification in Brugada syndrome; leaves open genotype-ECG correlations in larger cohorts.

Study Design

Type

Cohort (n=76)

Structured PICO

Does SCN5A mutation status correlate with clinical phenotype and prognosis in patients with Brugada syndrome?

P
Population
76 non-related patients with Brugada syndrome evaluated for SCN5A gene mutations and genotype-phenotype correlations.
E
Exposure
Direct sequencing and multiplex ligation-dependent probe amplification (MLPA) of the SCN5A gene
C
Comparator
Wild-type probands (patients without SCN5A mutations)
O
Outcome
Genotype-phenotype correlation (clinical characteristics, family risk profile, spontaneous type I Brugada ECG, syncope, history of arrhythmia)surrogate

Main Result

Absolute Event Rate: 87.5% vs 52.9%

p-value: p=0.06

In a cohort of Brugada syndrome patients, SCN5A mutations were found in 10.5% and were associated with familial disease and a trend toward spontaneous type I ECG, but not with increased arrhythmic events.

Limitations

  • Small sample size requiring larger studies to evaluate the role of polymorphisms

Cite This Study

García-Molina et al. (2012) conducted a cohort in Brugada syndrome (n=76). SCN5A gene mutations vs. Wild-type (no SCN5A mutations) was evaluated on Spontaneous type I Brugada ECG (p=0.06). In patients with Brugada syndrome, SCN5A mutations were associated with a trend toward more spontaneous type I ECGs compared to wild-type probands (87.5% vs 52.9%, p=0.06).

synapsesocial.com/papers/6a9e6c1b4bbd51f2272cc1dbhttps://doi.org/10.1111/cge.12017
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