SNOMED CT

Last uploaded: January 31, 2024
Preferred Name

Familial long QT syndrome

Synonyms

Familial long QT syndrome (disorder)

Inherited long QT syndrome

Definitions

A rare group of genetic, cardiac rhythm diseases with characteristics of a prolongation of the QT interval at basal electrocardiography (ECG) and by a high risk of life-threatening arrhythmias. The two cardinal manifestations are syncopal episodes, which may lead to cardiac arrest and sudden cardiac death, and electrocardiographic abnormalities: prolongation of the QT interval and T wave abnormalities. Inheritance may be autosomal dominant or autosomal recessive and depends on the genes involved.

ID

http://purl.bioontology.org/ontology/SNOMEDCT/442917000

Active

1

altLabel

Familial long QT syndrome (disorder)

Congenital long QT syndrome

Inherited long QT syndrome

Associated finding of

http://purl.bioontology.org/ontology/SNOMEDCT/1269318001

CASE SIGNIFICANCE ID

900000000000020002

CTV3ID

XUjIl

cui

C1141890

definition

A rare group of genetic, cardiac rhythm diseases with characteristics of a prolongation of the QT interval at basal electrocardiography (ECG) and by a high risk of life-threatening arrhythmias. The two cardinal manifestations are syncopal episodes, which may lead to cardiac arrest and sudden cardiac death, and electrocardiographic abnormalities: prolongation of the QT interval and T wave abnormalities. Inheritance may be autosomal dominant or autosomal recessive and depends on the genes involved.

DEFINITION STATUS ID

900000000000073002

Effective time

20100131

Has finding site

http://purl.bioontology.org/ontology/SNOMEDCT/80891009

INACTIVATION INDICATOR

900000000000483008

notation

442917000

prefLabel

Familial long QT syndrome

Subset member

6011000124106~MAPRULE~IFA 422348008 | Andersen Tawil syndrome |

6011000124106~MAPADVICE~IF ANDERSEN TAWIL SYNDROME CHOOSE Q87.89 | CONSIDER ADDITIONAL CODE TO IDENTIFY SPECIFIC CONDITION OR DISEASE | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT

447562003~MAPRULE~TRUE

6011000124106~MAPRULE~IFA 719907006 | Timothy syndrome type 2 (disorder) |

6011000124106~MAPPRIORITY~4

6011000124106~MAPADVICE~MAP SOURCE CONCEPT CANNOT BE CLASSIFIED WITH AVAILABLE DATA

6011000124106~MAPTARGET~

6011000124106~MAPCATEGORYID~447638001

6011000124106~MAPGROUP~1

6011000124106~MAPGROUP~2

447562003~MAPTARGET~I49.8

900000000000508004~ACCEPTABILITYID~900000000000549004

447562003~MAPGROUP~1

6011000124106~MAPTARGET~Q87.89

447562003~CORRELATIONID~447561005

6011000124106~MAPCATEGORYID~447637006

900000000000509007~ACCEPTABILITYID~900000000000548007

6011000124106~MAPADVICE~IF ANDERSEN TAWIL SYNDROME CHOOSE I45.81 | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT

447562003~MAPADVICE~ALWAYS I45.8

900000000000508004~ACCEPTABILITYID~900000000000548007

447562003~MAPPRIORITY~1

6011000124106~MAPTARGET~Q89.7

447562003~MAPCATEGORYID~447637006

447562003~MAPADVICE~ALWAYS I49.8

447562003~MAPTARGET~I45.8

6011000124106~MAPRULE~OTHERWISE TRUE

6011000124106~MAPRULE~IFA 719907006 | Timothy syndrome type 2 |

6011000124106~MAPCATEGORYID~447639009

6011000124106~MAPRULE~IFA 422348008 | Andersen Tawil syndrome (disorder) |

6011000124106~MAPADVICE~IF TIMOTHY SYNDROME TYPE 1 CHOOSE Q87.89 | CONSIDER ADDITIONAL CODE TO IDENTIFY SPECIFIC CONDITION OR DISEASE | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT

900000000000490003~VALUEID~900000000000483008

6011000124106~MAPRULE~IFA 699256006 | Timothy syndrome type 1 (disorder) |

6011000124106~MAPPRIORITY~1

900000000000497000~MAPTARGET~XUjIl

6011000124106~CORRELATIONID~447561005

6011000124106~MAPRULE~IFA 699256006 | Timothy syndrome type 1 |

6011000124106~MAPADVICE~IF TIMOTHY SYNDROME TYPE 2 CHOOSE Q89.7 | MAP OF SOURCE CONCEPT IS CONTEXT DEPENDENT

6011000124106~MAPRULE~TRUE

6011000124106~MAPADVICE~ALWAYS I45.81 | DESCENDANTS NOT EXHAUSTIVELY MAPPED

6011000124106~MAPPRIORITY~3

900000000000509007~ACCEPTABILITYID~900000000000549004

6011000124106~MAPPRIORITY~2

6011000124106~MAPTARGET~I45.81

6011000124106~MAPADVICE~ALWAYS I45.81

tui

T047

Type ID

900000000000003001

900000000000013009

subClassOf

http://purl.bioontology.org/ontology/SNOMEDCT/698270004

http://purl.bioontology.org/ontology/SNOMEDCT/363005004

http://purl.bioontology.org/ontology/SNOMEDCT/1899006

http://purl.bioontology.org/ontology/SNOMEDCT/9651007

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