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

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.