ONE-STAGE MIDLINE UNIFOCALIZATION AND COMPLETE REPAIR IN INFANCY VERSUS MULTIPLE-STAGE UNIFOCALIZATION FOLLOWED BY REPAIR FOR COMPLEX HEART-DISEASE WITH MAJOR AORTOPULMONARY COLLATERALS

Background: Patients with pulmonary atresia, ventricular septal defect , and major aortopulmonary collateral arteries have traditionally requ ired multiple unifocalization staging operations before undergoing com plete repair, Recently, the feasibility of a single-stage unifocalizat ion and repair was demonstrated by Hanley. In this report, we describe our experience with each approach, Methods and Results: Since 1989, 1 1 of 12 patients with pulmonary atresia, ventricular septal defect, an d major aortopulmonary collateral arteries have undergone complete sur gical correction, The first seven patients were subjected to staged bi lateral unifocalizations, with repair being achieved in six (group I), The last five patients have undergone a single-stage midline unifocal ization and repair via a sternotomy (group II), Four of these were inf ants (2 weeks to 9 months) and one was 13 years old, All patients in g roup I had tetralogy of Fallot, whereas in group II three patients had tetralogy of Fallot, one patient had double-outlet right ventricle, a nd one patient had complete atrioventricular canal and transposition, In group I, the median age at the first operation was 43 weeks, Comple te repair was performed at a median age of 3.5 years, with a mean numb er of 3.3 operations required, In group II, only one operation was req uired to achieve complete repair at a median age of 28 weeks, The post operative right ventricular/left ventricular pressure ratio was 0.49 i n group I and 0.45 in group II, One intraoperative death and one late death occurred in group I and no early or late deaths in group II. Cur rently, four patients in group I and all five patients in group II are alive and well, Conclusions: Early intervention with both surgical ap proaches can lead to complete biventricular repair in most patients, B ecause the single-stage midline unifocalization and repair can achieve a completely repaired heart in infancy with one operation, it is curr ently our approach of choice.