Tetralogy of Fallot with pulmonary atresia - Ontology Report

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Term:	Tetralogy of Fallot with pulmonary atresia	go back to main search page
Accession:	HP:0012516	browse the term
Definition:	An extreme form of tetralogy of Fallot characterized by absence of flow from the right ventricle to the pulmonary arteries.
Comment:	Tetralogy of Fallot with Pulmonary Atresia (TOF/PA) is a complex lesion with many different anatomic variants. The primary source of variability in this lesion is in the anatomy of the pulmonary arteries, with the spectrum ranging from well-formed, confluent pulmonary artery branches to completely absent native pulmonary arteries and major aorto-pulmonary collaterals (MAPCA's) providing all the pulmonary blood flow. The majority of cases fall somewhere in between these two ends of the spectrum, with the total pulmonary blood flow provided by a combination of diminutive native pulmonary arteries and multiple MAPCA.
Synonyms:	xref:	SNOMEDCT_US:253513005; UMLS:C0344882

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Term paths to the root

Path 1
Term	Annotations
Human phenotype	0
Phenotypic abnormality	0
Abnormality of the cardiovascular system	0
Abnormal cardiovascular system morphology	0
Abnormal heart morphology	0
Abnormal connection of the cardiac segments	0
Abnormal ventriculoarterial connection	0
Conotruncal defect	0
Tetralogy of Fallot	0
Tetralogy of Fallot with pulmonary atresia	0
Tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries	0
Path 2
Term	Annotations
Human phenotype	0
Phenotypic abnormality	0
Abnormality of the cardiovascular system	0
Abnormality of the vasculature	0
Abnormal vascular morphology	0
Abnormal blood vessel morphology	0
Abnormal morphology of the great vessels	0
Congenital malformation of the great arteries	0
Conotruncal defect	0
Tetralogy of Fallot	0
Tetralogy of Fallot with pulmonary atresia	0
Tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries	0

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