In 1971, Fontan and Baudet first introduced a new procedure for the repair of tricuspid atresia (1). Subsequently, the procedure became widely accepted as the operation of choice for univentricular heart physiology. However, several subsequent reports have demonstrated progressive deterioration of the original Fontan circulation on long-term follow up (2-4). Conversion of the Fontan pathway to either lateral tunnel or extracardiac conduit with additional right atrial maze procedure is considered to be the treatment of choice in the failed previous atrio-pulmonary connection. Progressive decline in systemic ventricular function and PAVM can be managed by heart and heart/lung transplantation, respectively. However, antiarrhythmic management, medically or interventionally, can be applied in selected patients. Failure of the Fontan's pathway is rather complicated and can be difficult to manage. Comprehensive evaluation and accurate diagnosis is crucial for the selection of an appropriate treatment modality.
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