Code System Concept

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Code System Concept

Code System Concept Code	1279844009
Code System Concept Name	Primary triglyceride deposit cardiomyovasculopathy (disorder)
Code System Preferred Concept Name	Primary triglyceride deposit cardiomyovasculopathy (disorder)
Concept Status	Published
Concept Status Date	09/01/2024
Code System Name	SNOMED-CT

Concept Relationships
Concept Details

Concept Details

Code System Code	PH_SNOMED-CT
Code System OID	2.16.840.1.113883.6.96
HL7 Table 0396 Code	SCT
Superseded By Concept
SDO Concept Status	Active
SDO Concept Status Date	03/01/2024
SDO Date Created	04/30/2023
SDO Date Revised	04/30/2023
Description	A rare inborn error of metabolism with characteristics of massive accumulation of triglycerides in the myocardium and coronary arteries while plasma triglyceride levels are normal. Patients present in adulthood with signs and symptoms of coronary artery disease and severe heart failure. Concomitant skeletal myopathy is common. Vacuole formation in polymorphonuclear leukocytes is typically observed.
Sequence

Extended Properties

No extended properties.

Concept Alternate Designations

SDO Designation ID	Concept Designation Description	Status	Designation Type	PHIN Preferred Term Flag	Code Flag
5234090013	Primary triglyceride deposit cardiomyovasculopathy	Active	Synonym	false	false
5234091012	Neutral lipid storage disease with severe cardiovascular involvement	Active	Synonym	false	false

Associated Value Sets

Value Set Name	Version(s)
Clinical Finding (NNDSS)	2 , 1

Parent Concepts

Parent Concepts

Parent/Child (Relationship Type)

Autosomal recessive hereditary disorder (disorder) {85995004 , SNOMED-CT } Cardiovascular system hereditary disorder (disorder) {363005004 , SNOMED-CT } Disorder of coronary artery (disorder) {414024009 , SNOMED-CT } Disorder of lipid metabolism (disorder) {267431006 , SNOMED-CT } Lipid storage disease (disorder) {10741005 , SNOMED-CT } Myocardial disease (disorder) {57809008 , SNOMED-CT } Structural disorder of heart (disorder) {128599005 , SNOMED-CT } Vascular degeneration (disorder) {280871000 , SNOMED-CT }

Other Relationships

No other relationships present.

Child Concepts

No child concepts present.

Page last reviewed:June 20, 2024
Page last updated:June 20, 2024
Content source:
- Centers for Disease Control and Prevention,