Evolution of the management approach for pulmonary atresia with intact ventricular septum

To review the evolution of the management approach for pulmonary atresia with intact ventricular septum (PAIVS) in the past two decades and to assess its impact on patient outcomes.

Retrospective review of the management and outcomes of 94 patients (55 male patients) with PAIVS diagnosed between July 1980 and August 2003.

Tertiary paediatric cardiac centre.

Seven patients died before interventions. Of the remaining 87 patients who underwent intervention at a median age of 9 days (from 1 day to 2 years), 12 had right ventricular outflow tract reconstruction (RVOTR), 42 had closed pulmonary valvotomy (CPV), and 15 had laser assisted valvotomy with balloon valvoplasty. A systemic-pulmonary shunt was inserted in 18 patients, six of whom had subsequent RVOTR (n = 4) or laser assisted valvotomy (n = 2). Since 1990, catheter intervention accounted for 38% (17 of 45) of the right ventricular outflow procedures. The mean (SEM) freedom from reintervention was 93 (7)%, 71 (12)%, and 57 (13)% after RVOTR, 75 (7)%, 40 (8)%, and 14 (6)% after CPV, and 54 (13)%, 24 (12)%, and 16 (10)% after laser assisted valvotomy at one month, six months, and one year, respectively (RVOTR versus CPV, p < 0.001; RVOTR versus laser assisted valvotomy, p = 0.001). Low cardiac output syndrome was significantly less common after catheter intervention than after RVOTR (0% v 44%, p = 0.003) or CPV (0% v 29%, p = 0.01). The overall mean (SEM) survival was 77 (5)% and 70 (5)% at one and five years, respectively, and the overall mortality was 33% (29 of 87). There were no significant differences in survival between the three groups.

Multiple interventions are often required in the treatment algorithm of PAIVS. The shift towards increased use of the transcatheter approach has reduced the occurrence of postprocedural low cardiac output syndrome.