Childhood systemic-pulmonary shunts: subsequent suitability for Fontan operation

We investigated whether or not a systemic arteriopulmonary shunt (SAPS) after 1 year of age affects a child's chances for a successful Fontan procedure. From 1967 to 1986, 50 patients more than 1 year old with tricuspid atresia or other complex lesions amenable to a Fontan operation received a SAPS. Follow-up was incomplete in six patients. Two patients died within 1 week of surgery, and two died within 9 months for a total mortality of 8%. Each of the 40 remaining patients was catheterized 6 months to 10.8 years (mean 4.9 years) after placement of a SAPS. By the criteria of Choussat and Fontan, there were one or more adverse SAPS-induced changes in nine patients (23%). Three developed pulmonary hypertension, one with associated elevation of pulmonary arteriolar resistance and pulmonary artery distortion, and one in conjunction with severe congestive heart failure. Six additional patients developed isolated distortion of the ipsilateral pulmonary artery. Of these nine patients, three had successful Fontan operations (33%), two died after the Fontan operation, and four are no longer candidates. In contrast, of 31 patients without documented SAPS-induced complications, 27 have undergone Fontan operations, 23 successfully (74%). Palliation with a SAPS after 1 year of age can place children at increased risk for a subsequent Fontan operation.