Objective: Introduction of the bidirectional Glenn procedure (BDG) in low-risk Fontan candidates would improve clinical outcomes. Over the last decade, not only high-risk Fontan candidates, but all candidates underwent BDG and staged Fontan operation (TCPC) in our hospital. Methods: Three hundred and thirty-three consecutive patients (age range, 42 days to 16 years old) underwent BDG at Fukuoka Children's Hospital Medical Center from 1992 to 2004. Diagnoses included hypoplastic left heart syndrome in 47, pulmonary atresia with intact ventricular septum in 32, tricuspid valve atresia in 35, and other complex univentricular heart defects in 219 patients (right dominant in 166, left dominant in 53). Results: There were three hospital deaths and 27 late deaths (five after TCPC). Six patients underwent takedown operation. Two hundred and thirty patients underwent TCPC, while 66 patients were waiting for TCPC. In five patients, completion of TCPC was contraindicated. A univariate analysis revealed that for patients less than six months old, diagnoses besides tricuspid atresia, right ventricular morphology, mean pulmonary arterial pressure, pulmonary vascular resistance, ventricular end-diastolic pressure, atrioventricular valve regurgitation greater than moderate, atrioventricular valvuloplastyyvalve replacement in concomitant procedure, and total anomalous pulmonary venous connection repair in concomitant procedure were significant predictors of death, takedown, or out of indication for completion of TCPC. A stepwise logistic regression analysis showed that mean pulmonary arterial pressure and heterotaxy were independent predictors. Conclusions: The staged strategy used for all Fontan candidates provides excellent clinical results. The main risk factor for death, takedown, or out of indication for completion of TCPC was elevated pulmonary arterial pressure. Appropriate surgical interventions such as atrioventricular valvuloplasty and total anomalous pulmonary venous connection repair, before andyor on BDG for the control of pulmonary circulation are of great importance to prevent elevation of pulmonary arterial pressure.
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