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.