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Right Superior Vena Cava Drainage Into the Left Atrium in Association With Vein of Galen Malformation
Nao Sasaki1, Clare Flanagan2, Michael H Kwon3
1Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts, USA; Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, USA.
Background:
Anomalous drainage of the right superior vena cava to the left atrium is a rare form of congenital heart disease. This typically results in mild cyanosis when present as an isolated defect.
Case Summary:
An asymptomatic term female demonstrated an SpO2 level of 88% despite supplemental oxygen. Transthoracic echocardiography with agitated saline contrast injection revealed drainage of upper-extremity venous blood to the left atrium. Advanced imaging confirmed the diagnosis and revealed partial anomalous pulmonary venous return. Hemodynamic instability noted following elective repair prompted additional investigation and demonstrated a congenital arteriovenous malformation in the brain (vein of Galen malformation).
Discussion:
This case highlights a rare association of a vein of Galen malformation with abnormal drainage of the right superior vena cava. It underscores the importance of maintaining a high index of suspicion for noncardiac anomalies when taking care of a patient with congenital heart disease.
Take-Home Message:
Multimodality imaging is essential to detecting associated intracardiac and often extracardiac anomalies.
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