Severely obstructed totally anomalous pulmonary venous return with residual connection to the left atrium

Totally anomalous pulmonary venous return may present diagnostic difficulty to clinicians, as it often appears similar to severe neonatal pulmonary disease. We describe a neonate who presented with severely obstructed pulmonary venous return, but with a residual venous connection to the left atrium. The unusual anatomy confounded the diagnosis. To our knowledge, this particular permutation has not previously been described. Our experience indicates that echocardiographic interrogation should be repeated in cases where there is no response to conventional therapy, and all individual pulmonary veins should be observed carefully.