Fontan operation for patients with severe distal pulmonary artery stenosis, atresia, or a single lung

In the absence of a ventricular pump, the status of the pulmonary circulation is crucial to the success of the Fontan operation. In an updated version (1999) of the optimal criteria for the Fontan operation, several of these criteria address the pulmonary circulation: pulmonary artery pressure, pulmonary vascular resistance, pulmonary artery size, and absence of significant pulmonary artery branch stenosis. This chapter reviews the role of the pulmonary circulation in a successful Fontan operation, with a particular emphasis on surgical techniques to repair severe distal or hilar pulmonary artery stenosis or atresia. The special situation of the patient with a single pulmonary artery is also addressed. Severe hilar pulmonary artery stenosis or atresia can be repaired by the technique of intrapulmonary pulmonary artery reconstruction with pericardial patch or tube and allow the successful completion of the Fontan operation. In the selected patient with a single pulmonary artery and optimal hemodynamics, the Fontan operation is possible with good outcome. However, further experience is needed in a larger number of patients to assess the long-term outcome of these treatment strategies.