Brugada syndrome

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Brugada syndrome

ORPHA:130Disease

Also called Ventricular fibrillation, Brugada type

What it is

A cardiac disorder characterized on electrocardiogram (ECG) by ST segment elevation with a coved aspect on the right precordial leads, and a clinical susceptibility to ventricular tachyarrhythmias and sudden death occurring in the absence of overt myocardial abnormalities.

Key facts

Prevalence
1-5 / 10 000 (Europe)
Age of onset
Adult, Childhood
Inheritance
Autosomal dominant, Not applicable
Classified as
Disease

Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.

Signs and symptoms

These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.

Genes

SCN5ADisease-causing germline mutation(s)
ABCC9Candidate gene tested
AKAP9Candidate gene tested
CACNA1CCandidate gene tested
CACNA2D1Candidate gene tested
CACNB2Candidate gene tested
GPD1LCandidate gene tested
HCN4Candidate gene tested
KCND3Candidate gene tested
KCNE3Candidate gene tested
KCNE5Candidate gene tested
KCNJ8Candidate gene tested
PKP2Candidate gene tested
RANGRFCandidate gene tested
SCN10ACandidate gene tested
SCN1BCandidate gene tested
SCN2BCandidate gene tested
SCN3BCandidate gene tested
SCNN1ACandidate gene tested
SEMA3ACandidate gene tested
SLMAPCandidate gene tested
TRPM4Candidate gene tested

ICD-10 codes

I49.8filed under a broader ICD-10 category — shared with 7 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

GARD 1030MEDDRA 10059027MESH D053840MONDO 0015263OMIM 601144OMIM 611777OMIM 611875OMIM 611876OMIM 612838OMIM 613119OMIM 613120OMIM 613123OMIM 616399UMLS C1142166

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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