BACKGROUND: Tetralogy of Fallot (TOF) with pulmonary atresia and major aortopulmonary collateral arteries (MAPCAs) is complex lesion with marked heterogeneity of pulmonary blood supply and arborization anomalies. Patients with TOF with PA and MAPCAs have traditionally required multiple staged unifocalization of pulmonary blood supply before undergoing complete repair. In this report, we describe recent change of strategy and the results in our institution. MATERIAL AND METHOD: We established surgical stratagies: early correction, central mediastinal approach, initial RV-PA conduit interposition, and aggressive intervention. Between July 1998 and August 2004, 23 patients were surgically treated at our institution. We divided them into 3 groups by initial operation method; group I: one stage total correction, group II: RV-PA conduit and unifocalization, group III: RV-PA conduit interposition only. RESULT: Mean ages at initial operation in each group were 13.9+/-16.0 months (group I), 10.4+/-15.6 months (group II), and 7.9+/-7.7 months (group III). True pulmonary arteries were not present in 1 patient and the pulmonary arteries were confluent in 22 patients. The balloon angioplasty was done in average 1.3 times (range: 1~6). There were 4 early deaths relating initial operation, and 1 late death due to incracranial hemorrhage after definitive repair. The operative mortalities of initial procedures in each group were 25.0% (1/4: group I), 20.0% (2/10: group II), and 12.2% (1/9: group III). The causes of operative mortality were hypoxia (2), low cardiac output (1) and sudden cardiac arrest (1). Definitive repair rates in each group were 75% (3/4) in group I, 20% (2/10, fenestration: 2) in group II, and 55.6% (5/9, fenestration: 1) in group III. CONCLUSION: In patients of TOF with PA and MAPCAs, RV-PA connection as a initial procedure could be performed with relatively low risk, and high rate of definitive repair can be obtained in the help of balloon pulmonary angioplasty. One stage RV-PA connection and unifocalization appeared to be successful in selected patients.
Angioplasty ; Angioplasty, Balloon ; Anoxia ; Arteries* ; Cardiac Output, Low ; Death, Sudden, Cardiac ; Hemorrhage ; Humans ; Mortality ; Population Characteristics ; Pulmonary Artery ; Pulmonary Atresia* ; Tetralogy of Fallot*