Pulmonary Atresia with Ventricular Septal Defect and Major Aorto-Pulmonary Collateral Arteries: Management Strategy at Our Hospital and the Results

Background and Objectives:Based on our previous studies on pulmonary atresia with ventricular septal defect (VSD) and major aorto-pulmonary collateral arteries (MAPCAs), a management strategy for this disease was for- mulated at our hospital in 1999. To evaluate this strategy, it was applied to the new patients and their outcomes were measured. Subjects and Methods:The subjects were a cohort of newly diagnosed patients who were without any prior interventions and these patients were treated at our hospital from January 2000 to December 2003. The management strategy focused on promoting the growth of the pulmonary arterial confluence (PAC), if present, by performing a right ventricle-pulmonary artery conduit operation (RV-PA conduit). If the PAC was large or absent, then the management plan was discussed among the surgeons after performing an exhaustive work-up. Results:Seventeen patients were enrolled and their age at the time of diagnosis ranged from 8 days to 34 months with a median age of 3 months. The initial surgical method varied from one-stage total correction (4 cases) to a conduit operation with or without additional MAPCAs procedures (13 cases). All but three of the patients survived their initial surgery. There were two late deaths. In addition to the 3 survivors from one-stage total correction, 4 patients underwent a staged total corrective operation. The 5 remaining patients are still a subtotal correction state or a palliated state. Conclusion:When a large PAC is present, one-stage total correction is the best surgical option. However, when the PAC is small, then RV-PA conduit without any vascular procedures may be a good alternative. This staged approach makes a larger PAC, which enables surgeons to create a much easier vascular anastomosis later on. (Korean Circulation J 2007;37:348-352)