How is Brugada syndrome 6 inherited?

Brugada syndrome 6 follows an autosomal dominant inheritance pattern. This means inheriting just one copy of the altered gene from either parent can cause the condition.

How is Brugada syndrome 6 diagnosed?

Information about diagnosis is currently limited for this condition.

Are there treatments for Brugada syndrome 6?

Information about treatment is currently limited for this condition.

What are the potential complications of Brugada syndrome 6?

Currently, there isn't a lot of information about the complications specifically related to Brugada syndrome 6. However, since it affects the heart's rhythm, there could be risks associated with heart rhythm problems. It's important to stay in touch with a healthcare provider to monitor any changes.

How might Brugada syndrome 6 affect daily life?

Information about how Brugada syndrome 6 impacts daily life is limited right now. Because it affects the heart's electrical system, patients may need to be cautious with activities that could stress the heart. Regular check-ups with a doctor can help manage any concerns.

What is the prognosis for someone with Brugada syndrome 6?

At this time, there isn't enough information to provide a clear prognosis for individuals with Brugada syndrome 6. Since research is ongoing, understanding how this condition progresses is still being studied. It's essential to work closely with a healthcare team for personalized guidance.

Should family members be tested for Brugada syndrome 6?

Since Brugada syndrome 6 is caused by a genetic mutation, family members may want to consider genetic testing. This can help identify if they carry the same mutation and understand their risks. Consulting with a genetic counselor can provide valuable information and support for family planning.

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Brugada syndrome 6 - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

Brugada syndrome 6

MONDO:0013145

Also known as:BRGDA6Brugada syndrome caused by mutation in KCNE3Brugada syndrome type 6

Overview

Any Brugada syndrome in which the cause of the disease is a mutation in the KCNE3 gene.

GeneticsAutosomal dominant · KCNE3 (chr 11)

Clinical Presentation

Built from Human Phenotype Ontology (HPO) phenotype annotations and GeneReviews clinical descriptions. Features represent findings documented across known cases — not an exhaustive list of possible symptoms.

Features include always present findings: ST segment elevation. 3 total HPO annotations.

Organ System	Phenotype Count	Example Features
Heart and blood vessels	2	Ventricular fibrillation, Cardiac arrest

Genetic Landscape

Assembled from HGNC gene records, ClinGen gene-disease validity evidence, and ClinVar pathogenic variant data. Validity classifications reflect the strength of published evidence linking each gene to this disease.

KCNE3 encodes potassium voltage-gated channel subfamily E regulatory subunit 3 (103 aa). Ancillary protein that functions as a regulatory subunit of the voltage-gated potassium (Kv) channel complex composed of pore-forming and potassium-conducting alpha subunits and of regulatory beta subunits. Highest expression in Whole Blood (18.2 TPM) and Small Intestine Terminal Ileum (12.5 TPM).

Brugada syndrome 6 is associated with mutations in the KCNE3 gene on chromosome 11.

KCNE3 is classified as a druggable target (Ion Channel category) with score 0.0.

GeneReviews

Diagnosis

Drawn from GeneReviews diagnostic guidelines, newborn screening panel status (RUSP), and ClinGen gene-disease validity classifications. Reflects current clinical evidence and consensus where available.

GeneReviews

Diagnostic Criteria

Brugada syndrome is a channelopathy, caused by genetic changes in transmembrane ion channels that create action potentials, in this case leading to an increased risk of cardiac arrhythmia .

Suggestive Findings

Brugada syndrome should be suspected in individuals with any of the following findings:

Recurrent syncope
Ventricular fibrillation

Treatment

Compiled from FDA-approved drug records, orphan drug designations, and FDA Adverse Event Reporting System (FAERS) safety data. GeneReviews management guidance is included where available.

No approved treatments are currently available for Brugada syndrome 6. The disease remains an area of unmet medical need.

GeneReviews

Management Guidelines

Evaluations Following Initial Diagnosis

To establish the extent of disease and needs in an individual diagnosed with Brugada syndrome, the following evaluations (if not performed as part of the evaluation that led to the diagnosis) are recommended:

Electrocardiogram (EKG)

Prognosis

Based on GeneReviews surveillance recommendations, HPO phenotype frequency and severity annotations, and published prevalence estimates from Orphanet and OMIM.

GeneReviews

Surveillance Guidelines

At-risk individuals with a family history of Brugada syndrome or a known pathogenic variant should undergo EKG monitoring every one to two years beginning at birth . The presence of type 1 EKG changes should be further investigated.

Source: GeneReviews — "Brugada Syndrome"

Phenotype severity distribution: 1 always present feature.

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

No clinical trials have been registered for Brugada syndrome 6.

112 publications have been identified in PubMed for Brugada syndrome 6. Research spans Clinical Trial Publication (21%), Basic Science / Preclinical (21%), and Epidemiology / Natural History (16%).

Research Type	Count	% of Total
Clinical study results	24	21%

Data assembled from 6 of 12 sources · Last updated Sep 19, 2026, 5:33 AM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

OMIM

Online Mendelian Inheritance in Man

OMIM:613119

GARD

Genetic and Rare Diseases Info Center

GARD:15619

Questions for Your Care Team

→Should I pursue genetic testing for KCNE3, and what would the results mean for my family?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

FAQ

Frequently Asked Questions

Common questions about Brugada syndrome 6

Brugada syndrome 6 is a rare condition that is part of the Brugada syndrome group. In this condition, a mutation in the KCNE3 gene is the known cause. Brugada syndromes typically affect the heart's rhythm and electrical system, and Brugada syndrome 6 is identified by this specific genetic change. Being aware of the genetic background of Brugada syndrome 6 provides insight into its classification and helps distinguish it from other types of Brugada syndrome. Information about other clinical details, including the overall impact on daily life, is limited at this time.

Information about symptoms is currently limited for this condition.

Brugada syndrome 6 is caused by a mutation in the KCNE3 gene. The mutation affects the heart's electrical system and is a known factor in triggering the condition. Genetic factors play a significant role in the development of conditions in the Brugada syndrome spectrum. Associated genes include KCNE3.

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Kisho

Brugada syndrome 6

Overview

Clinical Presentation

Genetic Landscape

Diagnosis

Diagnostic Criteria

Suggestive Findings

Treatment

Management Guidelines

Evaluations Following Initial Diagnosis

Prognosis

Surveillance Guidelines

Research Landscape

Medical References

External Resources

OMIM

GARD

Questions for Your Care Team

Questions for Your Care Team

FAQ

Frequently Asked Questions

Clinical Guidance

Explore clinical features

Clinical Guidance

Penetrance

Differential Diagnosis

Treatment of Manifestations

Clinical Guidance

Therapies Under Investigation

Clinical Trials

Recent Publications

Brugada syndrome 6

Overview

Clinical Presentation

Genetic Landscape

Diagnosis

Diagnostic Criteria

Suggestive Findings

Treatment

Management Guidelines

Evaluations Following Initial Diagnosis

Prognosis

Surveillance Guidelines

Research Landscape

Medical References

External Resources

OMIM

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

FAQ

Frequently Asked Questions

What is Brugada syndrome 6?

What are the symptoms of Brugada syndrome 6?

What causes Brugada syndrome 6?

How is Brugada syndrome 6 inherited?

How is Brugada syndrome 6 diagnosed?

Are there treatments for Brugada syndrome 6?

What are the potential complications of Brugada syndrome 6?

How might Brugada syndrome 6 affect daily life?

What is the prognosis for someone with Brugada syndrome 6?

Should family members be tested for Brugada syndrome 6?

Clinical Guidance

Explore clinical features

Clinical Guidance

Penetrance

Differential Diagnosis

Treatment of Manifestations

Clinical Guidance

Therapies Under Investigation

Clinical Trials

Recent Publications