Objective: To improve functional status following the Fontan procedure, it is important to alleviate cyanosis. Factors contributing to cyanosis following the Fontan procedure are systemic venous-to-pulmonary venous collaterals, pulmonary arteriovenous malformations (PAVMs), or shunting through residual intra-atrial communication. The current study was undertaken to review our institutional experience to alleviate cyanosis after the Fontan procedure.

Patients and Method: Records of 93 patients who survived the Fontan procedure between February 1993 and June 2008 at our institution were reviewed. Our combined approach is to perform contrast echocardiography, pulmonary perfusion scan, multi-detector-row computed tomography, and cardiac catheterization.

Results: We performed coil embolization for systemic venous-to-pulmonary venous collaterals or PAVMs in 19 patients. After coil embolization, arterial oxygen saturation as measured using pulse oximetry(SpO2) was improved from 92.9% to 95.6%. For surgical reinterventions, repair of lateral tunnel baffle leak was performed in two patients. The SpO2 was dramatically improved from 87.0% to 94.0% after leak repair. Redirection of the hepatic venous flow was performed in two polysplenia patients to attain the balanced hepatic flow to both lungs. Immediately after surgery, SpO2 was improved from 81.0% to 93.5%. One of the patients kept high SpO2 (96.0%) and showed regression of the PAVMs 5.7 years after redirection. Recent SpO2 was 94.8 ± 3.7% in 93 patients during the follow-up period (8.5 ± 4.3 years).

Conclusion: Interventions following the Fontan procedure to alleviate cyanosis can be safely performed. We should continue our efforts to lessen cyanosis and improve the quality of life for the patients.