Timing, frequency, and results of catheter intervention following recruitment of major aortopulmonary collaterals in patients with pulmonary atresia and ventricular septal defect.

The condition of pulmonary atresia (PA), ventricular septal defect (VSD), and major aortopulmonary collaterals (MAPCAs) can have an extremely variable pulmonary blood supply and there are essentially three main anatomical subtypes, one with confluent native pulmonary arteries, one with intrapulmonary but nonconfluent pulmonary arteries, and one with MAPCAs and no native pulmonary arteries (1) Surgical repair can be very challenging and the results are further complicated by patchy vascular distribution to the lung parenchyma, multiple stenoses, progressive narrowing of the MAPCAs, and high pulmonary vascular resistance; despite these hurdles, surgical repair is being carried out with increasing frequency and success, but this is also creating a new population of patients who require a specific interventional strategy. These problems, some of which are not easily addressed surgically, can be helped by catheter intervention methods and the two approaches are complementary. Between 1989 and 2002, 164 patients had surgical recruitment of MAPCAs at Birmingham Children's Hospital with repair in 70%. On average, these patients require 2.3 catheter procedures up to the point of repair. The catheter interventions are technically demanding, time consuming, must be repeated, and require specific techniques due to the pathology of the vessels. The management of PA/VSD/MAPCAs involves a multidisciplinary team approach often requiring repeated procedures, especially for those at the worst end of the spectrum. A proactive approach is essential due to known disease progression. Considerable improvement in patients' quality of life and, hopefully, life expectancy makes this approach worthwhile.