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long QT syndrome type 5
Pathology:
- KCNE1 mutants form channels that open slowly & close rapidly, thereby diminishing K+ currents
Genetics:
- associated with defects in KCNE1
Clinical manifestations:
- syncope & sudden death in response to exercise or emotional stress
Special laboratory:
- electrocardiogram
a) prolonged QT interval
b) polymorphic ventricular arrhythmias
Interactions
disease interactions
Related
Jervell & Lange-Nielsen syndrome
General
long QT syndrome
Database Correlations
OMIM 176261
MORBIDMAP 176261