Fontan Operation: Indications, Short and Long Term Outcomes.

Fontan operation, since its original description, has undergone a number of modifications so that it is now a staged, total cavo-pulmonary connection with fenestration. Stage I is palliation, depending upon the pathophysiology of the defect complex in early life, Stage II is bidirectional Glenn at about the age of 6 mo and Stage III is transfer of inferior vena caval blood to the pulmonary circuit along with fenestration between 2 to 4 y. Any patient that has only one functioning ventricle is a candidate for Fontan surgery. The morbidity and mortality have remarkably improved since the institution of staged, total cavo-pulmonary connection with fenestration. Complications during follow up continue to occur, though diminished with the newer modifications, and should be promptly addressed.