MANAGEMENT EVOLUTION OF PULMONARY ATRESIA AND INTACT VENTRICULAR SEPTUM

To examine the impact on survival and clinical course of incorporating the morphologic classification of the right ventricle into the evolvi ng management strategy for babies with pulmonary atresia and intact ve ntricular septum, the surgical results and follow-up status of the fir st 62 consecutive patients managed in this hospital between 1979 and 1 990 were reviewed. Before 1984, all 23 babies from group I underwent p rimary right ventricular outflow reconstruction irrespective of right ventricular morphology and size. Since 1984, depending on the morpholo gy and size of the right ventricle, 39 babies from group II had either closed transventricular pulmonary valvotomy (n = 31) or a shunt opera tion (n = 8). There were 10 hospital (43%) and 2 late deaths (total mo rtality 52%) in our group I patients. Three of the 11 long-term surviv ors had cyanosis at rest but none had any residual pressure gradient a cross the pulmonary outflow. Group II had 6 hospital (15%) and 4 late deaths (total mortality = 26%). Of the 29 long-term survivors, 9 had a second-stage right ventricular outflow reconstruction, 8 had balloon valvuloplasty and 2 had successful Fontan operation. At the latest fol low-up, 5 children from this group have cyanosis at rest, 1 has a resi dual gradient (55 mm Hg) across the infundibulum, and 3 have right ven tricular dysfunction. The hospital and total mortality for babies in g roup II was significantly lower than that in group I (p < 0.01). These data suggest that tailoring the treatment to the right ventricular an atomy results in a lower overall mortality although long term postoper ative hemodynamic abnormalities are observed in both groups.