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Start free with EleplanPersistent left superior vena cava connecting to the roof of left-sided atrium
ORPHA:99111Morphological anomaly
Also called Persistent left SVC connecting to left-sided atrium · Persistent left SVC connecting to the roof of left-sided atrium · Persistent left superior vena cava connecting to left-sided atrium
What it is
A rare congenital anomaly of superior vena cava characterized by a persistent left superior vena cava that drains into the left atrium through a direct connection to its roof, creating a right-to-left shunt. Patients are at risk of developing chronic hypoxia, decreased exercise tolerance, cyanosis, embolic cerebrovascular events, and heart failure.
Key facts
- Classified as
- Morphological anomaly
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.
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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