Surgical results for pulmonary atresia with intact ventricular septum: a single-centre 15-year experience and medium-term follow-up

Abstract

Objectives: The optimal surgical strategies for pulmonary atresia with intact ventricular septum (PAIVS) are still not well established. This study reviewed our 15-year experience in the management of PAIVS.

Methods: Between July 1999 and June 2014, 170 patients were treated for PAIVS in our heart centre. Based on the morphology of the right ventricle (RV), age and surgical approaches, the patients were divided into two groups: the one-stage surgery group (n = 33) and the staged surgery group (n = 137), in which patients received definitive repair, including biventricular repair, 1.5 ventricular repair and univentricular palliation without or with initial intervention. The median follow-up time was 6.6 years (range: 1-15 years); survival rates, risk factors for death and clinical status after operation were assessed.

Results: In the one-stage surgery group, there were three deaths post operation; the estimated 1-, 5- and 15-year survival rates were 97.0, 93.7 and 88.5%, respectively. In the staged surgery group, 23 patients died, including 15 in the waiting period after initial intervention. The estimated 1-, 5- and 15-year survival rates of the staged group were 89.8, 88.2 and 69.1%, without significant difference when compared with the one-stage surgery group (P > 0.05). Independent predictors of mortality were severe RV hypoplasia (P < 0.05) and lower tricuspid valve Z-scores (P < 0.01). At the latest follow-up, most of the patients in both groups had a good clinical status after definitive repair. The re-operation rate was 16.0% (4/25) in the one-stage surgery group compared with 15.4% (6/39) in the staged surgery group.

Conclusions: Both one-stage repair and the staged surgical procedure had acceptable surgical outcomes in this retrospective study. Initial intervention is suitable for neonates or younger patients to promote the growth of the RV, and one-stage definitive repair is a beneficial choice for older patients with PAIVS, in whom the growth potential of the RV is limited.

Keywords: Congenital heart disease; Follow-up; Pulmonary atresia with intact ventricular septum; Surgical treatment; Transcatheter management.