Pulmonary atresia with intact ventricular septum: limitations of catheter-based intervention

Pulmonary atresia with intact ventricular septum (PAIVS) has a wide spectrum of anatomic heterogeneity and invokes a wide variety of treatment strategies. We reviewed the outcome of our patients with PAIVS in order to delineate strategies for the optimal management of PAIVS. In particular, the possibility of avoiding neonatal surgical intervention with catheter-based technology was assessed.

The study cohort was composed of all patients presented with PAIVS from January 1999 through December 2005. Demographic and anatomic variables were analyzed to determine association with in-hospital mortality.

Forty-four infants with PAIVS underwent catheter valvuloplasty (n = 17) and (or) surgical intervention (n = 42). The mean age and weight of the infants was six days and 3.1 kg, and the average follow-up was 40 +/- 29.5 months. Five (11%) had right ventricle dependent coronary circulation (RVDCC) and six (14%) had Ebstein's anomaly. Five (11%) patients died. Of those who underwent catheter valvotomy, three (18%) underwent shunt placement, 12 (71%) underwent right ventricular outflow tract reconstruction with shunt placement, and only two (12%) did not require a further surgical intervention in the newborn period. Multivariable analyses demonstrated RVDCC (odds ratio 21.3, p = 0.025) and Ebstein's anomaly (odds ratio 16.0, p = 0.038) to be risk factors for in-hospital mortality. Of those patients with Ebstein's anomaly, a single ventricle approach had a better outcome.

We demonstrated excellent recent outcomes for patients with PAIVS. Catheter-based interventions rarely avoid surgical repair. The RVDCC and Ebstein's anomaly were associated with high mortality. In patients with Ebstein's anomaly, single ventricular pathway may be the better strategy for this specific patient population.