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long QT syndrome 10 (LQT10) - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

long QT syndrome 10

MONDO:0012737

Also known as:LQT10SCN4B long QT syndromelong QT syndrome caused by mutation in SCN4B

Overview

Any long QT syndrome in which the cause of the disease is a mutation in the SCN4B gene.

GeneticsAutosomal dominant · SCN4B (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 sometimes findings: Atrial fibrillation and Sudden cardiac death. 5 total HPO annotations.

Organ System	Phenotype Count	Example Features
Heart and blood vessels	3	Atrial fibrillation, Sudden cardiac death, Atrioventricular block

Explore clinical features

Genetic Landscape

SCN4B function has not been fully characterized.

Long QT syndrome 10 is associated with mutations in the SCN4B gene on chromosome 11.

Diagnosis

Genetic testing for SCN4B is available. Testing is considered confirmatory for diagnosis.

Biomarker and diagnostic research for long QT syndrome 10 has been reported in the published literature.

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 long QT syndrome 10.

203 publications have been identified in PubMed for long QT syndrome 10. Kisho has analyzed 104 by research type. Research spans Epidemiology / Natural History (25%), Review / Meta-Analysis (20%), and Clinical Trial Publication (14%).

Research Type	Count	% of Total
Disease patterns and progression	26	25%

Data assembled from 5 of 12 sources · Last updated Sep 20, 2026, 4:03 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

OMIM

Online Mendelian Inheritance in Man

OMIM:611819

GARD

Genetic and Rare Diseases Info Center

GARD:10436

Questions for Your Care Team

→Should I pursue genetic testing for SCN4B, 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?

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long QT syndrome 10

Overview

Clinical Presentation

Explore clinical features

Genetic Landscape

Diagnosis

Research Landscape

Medical References

External Resources

OMIM

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

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