Submission Details

Submitter:

Classification:
Disputed Evidence
GENCC:100005
Gene:
Disease:
short QT syndrome
Mode Of Inheritance:
Autosomal dominant
Evaluated Date:
10/27/2020
Evidence/Notes:

SCN5A encodes the alpha subunit of the cardiac voltage-gated sodium channel. Genetic evidence supporting its relationship with SQTS is derived from a single case in which a rare SCN5A variant was discovered (PMID 22490985). The patient, however, had a type 1 Brugada pattern with a relatively short QT interval and the Expert Panel regarded this phenotype as being concordant with Brugada syndrome and not SQTS.

PubMed IDs:
22490985
Public Report:
Assertion Criteria:
Submitter Submitted Date:
12/05/2025

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