Cause of profound hypoxemia following a bilateral bidirectional Glenn shunt: Clue suggested by agitated saline echo contrast.

Following a bilateral bidirectional Glenn shunt, a child had persistent hypoxemia. Agitated saline contrast injection into the jugular vein during transesophageal echocardiography displayed a rapid appearance of saline particles in the cardiac chambers suggesting the presence of pulmonary arteriovenous malformations. However, the clinical picture was not in agreement and an angiographic contrast injection during an immediate cardiac catheterization revealed the underlying pathology which was immediately corrected surgically.

Identifying Anomalies of Systemic Venous Drainage: Systemic Venous Anomalies; Atrial Morphology.

So as to produce totally anomalous systemic venous connection, all of the systemic venous tributaries, along with the coronary sinus, should be connected with the morphologically left atrium. Previous descriptions of this rare constellation of anomalous connections of the systemic venous tributaries of the heart have been compromised by the inclusion of individuals having isomeric atrial appendages. In these settings, most frequently, the totally, or almost totally, anomalous systemic venous connections are associated with a sinus venosus defect.