Right superior vena cava connecting to left-sided atrium

Orpha code: 99110OMIM code:

Definition

A rare, congenital vascular malformation of the major vessels characterized by the right SVC passing medially and dorsally to the aortic root and draining into the left atrium. Patients usually present a right-to-left systemic venous blood shunt which may manifest with arterial hypoxemia, cyanosis, exercise dyspnea, clubbing of the fingers, palpitations, murmurs and/or potentially fatal brain abscess. Association with other cardiac anomalies has been reported.

Disease data
Classification

Morphological anomaly

Synonyms
Right SVC connecting to left-sided atrium
Prawa SVC uchodząca do lewego przedsionka
Prawa żyła główna górna łącząca się z lewostronnym przedsionkiem
Right superior caval vein connecting to left-sided atrium
ORPHA code
99110
OMIM code
-
ICD10 code
Q26.8
ICD11 code
-

No additional description.

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