Code System Concept

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Code System Concept

Code System Concept Code	1197417009
Code System Concept Name	Congenital portosystemic shunt (disorder)
Code System Preferred Concept Name	Congenital portosystemic shunt (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Superseded By Concept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	Inactive
SDO Concept Status Date	09/01/2022
SDO Date Created	02/28/2022
SDO Date Revised	11/30/2022
Description	A rare congenital anomaly of the great veins characterized by an abnormal communication between one or more veins of the portal and the caval systems, resulting in complete or partial diversion of the portal blood away from the liver to the systemic circulation. Clinical manifestations include liver atrophy, hypergalactosemia without uridine diphosphate enzyme deficiency, hyperammonemia, encephalopathy (resulting in learning disabilities, extreme fatigability and seizures), pulmonary hypertension, hypoxemia from hepatopulmonary syndrome and benign or malignant tumors.
Sequence

Extended Properties

No extended properties.

Concept Alternate Designations

SDO Designation ID	Concept Designation Description	Status	Designation Type	PHIN Preferred Term Flag	Code Flag
4696264015	Congenital portosystemic venous fistula	Active	Synonym	false	false
4696265019	Congenital portosystemic shunt	Active	Synonym	false	false

Associated Value Sets

No associated value sets.

Parent Concepts

Parent Concepts

Parent/Child (Relationship Type)

Anomalies of great veins (disorder) {204451006 , SNOMED-CT } Congenital abnormality of vein (disorder) {297222002 , SNOMED-CT } Congenital anomaly of abdomen (disorder) {363024001 , SNOMED-CT } Congenital anomaly of cardiovascular structure of trunk (disorder) {363028003 , SNOMED-CT } Disorder of portal venous system (disorder) {128056006 , 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,