Right Superior Vena Cava Drainage Into the Left Atrium in Association With Vein of Galen Malformation.

Authors

Nao Sasaki, Clare Flanagan, Michael H Kwon, Alfred Pokmeng See, Victoria Habet, Thomas J Seery

Published in

JACC. Case reports. Pages 109804. Aug 13, 2026. Epub Aug 13, 2026.

Abstract

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.
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).
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.
Multimodality imaging is essential to detecting associated intracardiac and often extracardiac anomalies.

PMID:
42593383
Bibliographic data and abstract were imported from PubMed on 13 Aug 2026.

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