Orphanet Rare Disease Ontology

Last uploaded: July 3, 2024
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Preferred Name

Absence of the pulmonary artery
Synonyms

Aplasia of pulmonary artery

Unilateral Pulmonary Artery Absence

Unilateral pulmonary artery agenesis

UAPA
Definitions

A rare vascular anomaly characterized by congenital absence of the right or left pulmonary artery, usually ending within 2 cm of its expected origin from the pulmonary trunk. Patients often also have other cardiovascular abnormalities and respective symptoms and are then typically diagnosed in infancy or childhood, while isolated cases generally present with a mild clinical course and may go undiagnosed until adulthood. Presenting clinical features in isolated cases include hemoptysis, exertional dyspnea, and recurrent respiratory infections. The condition is typically accompanied by marked changes of lung tissue and may, if unrecognized, result in massive hemoptysis and pulmonary hypertension.
ID

http://www.orpha.net/ORDO/Orphanet_980
alternative_term

Aplasia of pulmonary artery

Unilateral Pulmonary Artery Absence

Unilateral pulmonary artery agenesis

UAPA
definition

A rare vascular anomaly characterized by congenital absence of the right or left pulmonary artery, usually ending within 2 cm of its expected origin from the pulmonary trunk. Patients often also have other cardiovascular abnormalities and respective symptoms and are then typically diagnosed in infancy or childhood, while isolated cases generally present with a mild clinical course and may go undiagnosed until adulthood. Presenting clinical features in isolated cases include hemoptysis, exertional dyspnea, and recurrent respiratory infections. The condition is typically accompanied by marked changes of lung tissue and may, if unrecognized, result in massive hemoptysis and pulmonary hypertension.
definition_citation

Orphanet
expertlink

https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=en&Expert=980
has_age_of_onset

All ages
has_inheritance

Not applicable
hasDbXref

ICD-11:LA8B.1

UMLS:C0265905

ICD-10:Q25.7
label

Absence of the pulmonary artery
notation

ORPHA:980
part_of

http://www.orpha.net/ORDO/Orphanet_98719
prefixIRI

ORDO:Orphanet_980
prefLabel

Absence of the pulmonary artery
present_in

Europe AND has_annual_incidence_average_value : 0.5 AND has_annual_incidence_range : 1-9 / 1 000 000

Worldwide AND has_point_prevalence_range : Unknown
treeView

http://www.orpha.net/ORDO/Orphanet_98719
subClassOf

http://www.orpha.net/ORDO/Orphanet_557493

http://www.orpha.net/ORDO/Orphanet_377791
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