Tetralogy of Fallot with absent pulmonary valve and obstructed totally anomalous pulmonary venous connection.

We describe a combination, unique as far as we are aware, of tetralogy of Fallot with so-called "absent" pulmonary valve and supracardiac totally anomalous pulmonary venous connection. The vertical vein was obstructed in a neonate born at term who presented with respiratory compromise.