We herein report a case of a hypoplastic left heart syndrome variant complicated with partial anomalous pulmonary venous connection to the left innominate vein. We left the vertical vein at the time of the bidirectional Glenn procedure, and ligated the vertical vein at the time of the total cavopulmonary connection procedure, without reconnecting the vertical vein to the left atrium. Because of the development of an interlobar vein draining from the left upper lung into the lower lung after the bidirectional Glenn procedure, the circulation of the left upper lung was preserved after the total cavopulmonary connection procedure.
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