A Fontan operation was planned for a 5-year-old girl with complex cyanotic heart disease and bilateral superior venae cavae. The unexpected finding of total anomalous pulmonary venous connection to the left superior vena cava led to a change of strategy. During a bilateral bidirectional Glenn procedure, the pulmonary confluence had to be disconnected to avoid pulmonary venous obstruction. This resulted in bilateral unidirectional cavopulmonary flow.
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