Risk factors for interstage death after stage 1 reconstruction of hypoplastic left heart syndrome and variants

The risk of death during the interstage period remains high after stage 1 reconstruction for single ventricle lesions, despite improved surgical results. The purpose of this study is to identify risk factors for interstage death and to describe the events leading to interstage death.

A nested case-control study was conducted of 368 patients who underwent stage 1 reconstruction at a single center between January 1998 and April 2005.

Among the 313 (85%) hospital survivors, there were 33 (10.5%) interstage deaths. Cases more frequently presented with intact or restrictive atrial septum (9 [27%] vs 4 [4%]; P < .001), were older at the time of surgery (5 [2-40] vs 3 [1-42] days; P = .005), had more postoperative arrhythmias (12 [36%] vs 15 [15%]; P = .01), and a higher incidence of airway or respiratory complications (12 [36%] vs 19 [19%]; P = .04). By multivariate analysis, only intact atrial septum (odds ratio 7.6; 95% confidence intervals 1.9-29.6; P = .003) and age at operation greater than 7 days (odds ratio 3.8; 95% confidence intervals 1.3-11.2; P = .017) were predictors of interstage death.

The presence of intact atrial septum and older age at the time of surgery are associated with a higher risk of interstage death. In addition, postoperative arrhythmia and airway complications are associated with a higher risk of interstage death in univariate analysis. The results of this study provide a focus for interstage monitoring and risk stratification of these high-risk infants, which may improve overall survival.