pulmonary atresia with ventricular septal defect

Go to external page http://purl.obolibrary.org/obo/MONDO_0008343


Pulmonary atresia with ventricular septal defect (PA-VSD) is a rare cyanotic congenital heart malformation characterized by underdevelopment of the right ventricular outflow tract and atresia of the pulmonary valve, ventricular septal defect (VSD) and pulmonary collateral vessels. Clinical features depend on the anatomic variability of the lesion and patients may be minimally symptomatic, severely cyanotic or may develop congestive heart failure. PA-VSD may represent a severe form of Tetralogy of Fallot. [ Orphanet:1207 ]

This is just here as a test because I lose it

Term information

database cross reference
  • NCIT:C99033 (MONDO:equivalentTo)
  • MESH:C562833 (MONDO:equivalentTo)
  • UMLS:C0344976 (Orphanet:1207)
  • GARD:0004588 (MONDO:equivalentTo)
  • Orphanet:1207 (OMIM:178370)
  • SCTID:253591008 (MONDO:equivalentTo)
  • OMIM:178370 (Orphanet:1207)
  • ICD10:Q25.5 (Orphanet:1207)
Subsets

gard_rare, ordo_morphological_anomaly

definition

Pulmonary atresia with ventricular septal defect (PA-VSD) is a rare cyanotic congenital heart malformation characterized by underdevelopment of the right ventricular outflow tract and atresia of the pulmonary valve, ventricular septal defect (VSD) and pulmonary collateral vessels. Clinical features depend on the anatomic variability of the lesion and patients may be minimally symptomatic, severely cyanotic or may develop congestive heart failure. PA-VSD may represent a severe form of Tetralogy of Fallot.

exactMatch

http://purl.obolibrary.org/obo/NCIT_C99033

http://www.orpha.net/ORDO/Orphanet_1207

http://identifiers.org/mesh/C562833

http://identifiers.org/omim/178370

http://identifiers.org/snomedct/253591008

http://linkedlifedata.com/resource/umls/id/C0344976

has exact synonym

pulmonary valve atresia with ventricular septal defect

pulmonary atresia with ventricular septal defect

id

MONDO:0008343

seeAlso

https://rarediseases.info.nih.gov/diseases/4588/pulmonary-atresia-with-ventricular-septal-defect