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Start free with EleplanAbnormal origin of right or left pulmonary artery from the aorta
ORPHA:99050Morphological anomaly
Also called Hemitruncus arteriosus · Pulmonary artery coming from the aorta
What it is
A rare, congenital, heart malformation characterized by anomalous origin of one branch of the pulmonary arteries directly from the aorta and a normal origin of the other pulmonary artery from the main pulmonary artery coming from the right ventricular outflow tract. Patients present respiratory distress, congestive heart failure and failure to thrive within the first days/months of life.
Key facts
- Age of onset
- Infancy, Neonatal
- Classified as
- Morphological anomaly
Signs and symptoms
Very common80–99%
2Common30–79%
9Sometimes5–29%
9- Abnormal aortic arch morphology
- Anomalous origin of left pulmonary artery from ascending aorta
- Aortopulmonary window
- Arteria lusoria
- Heart murmur
- Pulmonary arterial hypertension
- Tetralogy of Fallot
- Transposition of the great arteries
and 1 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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