Pulmonary atresia with an intact septum (PAIVS) presents a therapeutic challenge and the initial decisions about following a univentricular pathway or creating a biventricular circulation are critical. The data of 1039 neonates reported to the Pediatric Cardiac Care Consortium from 1982 to 2006 were analyzed. As with other reports, the overall mortality has remained high (20% in-hospital deaths). Despite continuing difficulties with these patients, however, the treatment approaches remained variable and have not changed over this period. The variable surgical and interventional approaches presumably reflected the anatomy at the initial evaluation and, as expected, were important in determining whether the patient followed a single or two ventricle repair track. Opening the atrial septum, for example, as an initial procedure resulted in a greater frequency of subsequent Glenn and Fontan procedures than did opening the right ventricular outflow tract. The only procedure change has been the more frequent use of balloon pulmonary valvotomy in the past 15. years to open the pulmonary valve when the anatomy was favorable.
- Pulmonary atresia with an intact septum