Pulmonary Atresia With Intact Ventricular Septum: Initial Management

Background Outcomes for pulmonary atresia with intact ventricular septum are suboptimal, while initial management remains controversial. This study was undertaken to determine the effect of catheter-based therapy on the need for early surgical intervention. Methods A single-institution retrospective chart review was made of all 25 neonates with pulmonary atresia with intact ventricular septum from 1999 to 2005. Results Mean age at first intervention was 3.1 ± 2.2 days, mean weight 3.3 ± 0.5 kg. Right ventricular hypoplasia varied: 20% normal, 16% mild, 28% moderate, 28% moderately severe or severe, 8% not classified. Median tricuspid valve z-score was −2.3 ± 2.6. First intervention was catheter-based therapy in 56% (14 of 25), operative in 36% (9 of 25), and no therapy in 2. Technically adequate valvotomy was achieved in 79% (11 of 14). Serious catheter-related complications occurred in 3 of 14 (21%). Only 5 of 14 (36%) with catheter-based therapy weaned from prostaglandins without surgery. Of these, 2 required surgery for cyanosis at 1 and 3 months. Surgery after catheter-based therapy consisted of right ventricular outflow patch in 36% (4 of 11), systemic to pulmonary shunt in 64% (7 of 11). Median time between catheter-based therapy and surgery was 8.5 days (range, 1 to 89). Only 3 of the 23 treated patients avoided operation during infancy. There was 1 early and 1 late death after operation after initial catheter-based therapy, and 1 late death after primary surgery alone during a mean follow-up of 33 months (range, 1.5 to 79). Conclusions Balloon valvotomy alone for pulmonary atresia with intact ventricular septum rarely obviates the need for an additional source of pulmonary blood flow—either shunt or ductal stenting.