Brugada Syndrome is an autosomal dominant disease with variable expression. In approximately 20% of cases, the underlying cause of Brugada syndrome is a genetic defect in the SCN5A
gene, which encodes
the sodium channel.
Brugada Syndrome manifests with syncope and cardiac arrest,
typically occurring in the
third and fourth
decade of life. The syndrome is characterised
by a typical
electrocardiogram (ECG) pattern of ST
segment elevation in leads V1 to V3,
and incomplete or complete right bundle branch
block. ICD is indicated
in patients with symptoms suggestive
of, or evidence of arrhythmias.
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