long QT syndrome
Findings
No curated finding names long QT syndrome yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
A condition that is characterized by episodes of fainting (syncope) and varying degree of ventricular arrhythmia as indicated by the prolonged QT interval. The inherited forms are caused by mutation of genes encoding cardiac ion channel proteins. The two major forms are Romano-Ward syndrome (also known as long QT syndrome 1) and Jervell-Lange Nielsen syndrome.
Definition from the Mondo Disease Ontology (MONDO:0002442), read 2026-09-29. CC BY 4.0.
Features
1 feature
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, hp/releases/2026-09-01.
- ArrhythmiaMondoHP:0011675
Genes
17 genes
Germline variants in the gene encoding each protein, as classified by GenCC submitters, each in their own words. Limited, disputed and refuted submissions are listed too.
- CALM1HGNC:1442
- Definitive · ClinGen · Autosomal dominant · 2018
- HGNC:1445HGNC:1445
- Definitive · ClinGen · Autosomal dominant · 2018
- HGNC:1449HGNC:1449
- Definitive · ClinGen · Autosomal dominant · 2018
- KCNH2HGNC:6251
- Definitive · ClinGen · Autosomal dominant · 2018
- KCNQ1HGNC:6294
- Definitive · ClinGen · Autosomal dominant · 2018
- TRDNHGNC:12261
Where it sits
- A kind of
- Narrower terms (1)
Other names
1 name
Resolves to: long QT syndrome
- Also called
- ventricular arrhythmia associated with long QT syndrome