Long-Term Outcomes After Intervention for Pulmonary Atresia With Intact Ventricular Septum

This is a cohort study from the Pediatric Cardiac Care Consortium based on data from 616 patients with pulmonary atresia with intact ventricular septum (PA/IVS) treated between 1982 and 2003. Median follow-up was 16.7 years (IQR 12.6-22.7).
Initial interventions included aortopulmonary shunt in 247, right ventricular decompression in 96, and both in 273. Risk factors for death at initial intervention included earlier birth era (1982-1992), chromosomal abnormality, and atresia of one or both coronary ostia. Among 494 survivors of neonatal hospitalization, there were 99 deaths (4 posttransplant) and 10 transplants (median age of death or transplant 0.7 years, IQR, 0.3-1.8). Definite repair or last-stage palliation was achieved in the form of completed 2-ventricle repair (n=201), one-and-a-half ventricle (n=39), or Fontan (n=96). Overall 20-year survival was 66%, but for patients discharged alive after definitive repair, it reached 97.6% for single-ventricle patients, 90.9% for those with one-and-a-half ventricle, and 98.0% for those with complete 2-ventricle repair (log-rank p=0.052).
The authors conclude that transplant-free survival in PA/IVS is poor due to significant infantile and interstage mortality. Survival into early adulthood is excellent for patients reaching completion of their intended treatment path, independent of type of repair.