NCT01250327.

Pulmonary artery rehabilitation allows complete repair in the majority of patients with pulmonary atresia, ventricular septal defect, hypoplastic pulmonary arteries, and major aortopulmonary collaterals. However, long-term management often requires pursuit of the rehabilitation process.

Pulmonary atresia with ventricular septal defect (VSD), hypoplastic native pulmonary arteries (PAs) and major aorto-pulmonary collateral arteries (MAPCAs) is a rare and complex congenital cardiac disease. In broad outline, two surgical approaches are available for patients with this condition. The first is characterized by one or several stages of complete unifocalization of the supplying MAPCAs, with or without incorporation of the native pulmonary arteries (PAs), connection of the right ventricle to the 'neo-Pas' and, if possible, concomitant or delayed closure of the VSD. The second strategy is based on rehabilitation of the native pulmonary arteries. The first step is a direct right ventricle to native PA connection, to promote the growth of native PAs. The establishment of antegrade flow also allows an easier approach for interventional catheterization, enabling dilatation or stenting of the stenosis and then closure of the communicant collaterals. When the development of the native PAs is satisfactory, the complete repair is performed. If it is necessary to suture a MAPCA to the PA ('unifocalization'), this is accomplished by connecting the collateral artery to an already developed native branch. Our team developed this multidisciplinary strategy with good results. Based on this experience as well as on the published literature, we describe this strategy of management of patients with pulmonary atresia, VSD, hypoplastic pulmonary arteries and major aorto-pulmonary collaterals (MAPCAs).

Transcatheter technique for muscular ventricular septal defect closure remains challenging, particularly in small patients. We report the successful use of the low profile Amplatzer Duct Occluder II for multiple ventricular septal defect closure in an infant with D-transposition of the great arteries, status postpulmonary artery banding. The multiple apical ventricular septal defects were successfully closed with two Amplatzer Duct Occluder II and one Amplatzer Muscular Ventricular Septal Defect Occluder during two interventional catheterizations. The patient subsequently underwent successful surgical repair. V C 2011 Wiley-Liss, Inc.

The majority of secundum atrial septal defect is amenable to transcatheter closure, using a modified implantation technique in 16% of cases.