Pulmonary artery hypoplasia

Orpha code: 99083OMIM code:

Definition

A rare, congenital anomaly of the great arteries characterized by various clinical signs and symptoms, shortness of breath, including recurrent lower respiratory tract infections, lung hypoplasia, pulmonary hypertension, and haemoptysis. The anomaly can be isolated or associated with congenital heart disease, such as tetralogy of Fallot, atrial septal defect, coarctation of the aorta, right aortic arch, truncus arteriosus, patent ductus arteriosus and pulmonary atresia.

Disease data
Classification

Morphological anomaly

Synonyms
PAH
Unilateral Pulmonary Artery Hypoplasia
PAH
Unilateral Pulmonary Artery Hypoplasia
ORPHA code
99083
OMIM code
-
ICD10 code
Q25.7
ICD11 code
LA8B.1

No additional description.

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