Development of a validated risk score for interstage death or transplant after stage I palliation for single-ventricle heart disease.

Abstract

OBJECTIVE\nTo develop a risk score to predict mortality or transplant in the interstage period.\n\n\nBACKGROUND\nThe interstage period between the stage 1 and stage 2 palliation is a time of high morbidity and mortality for infants with single-ventricle congenital heart disease.\n\n\nMETHODS\nThis was an analysis of patients with single-ventricle congenital heart disease requiring arch reconstruction who were enrolled in the National Pediatric Cardiology Quality Improvement Collaborative registry from 2008 to 2015. The primary composite endpoint was interstage mortality or transplant. Multivariable logistic regression and classification and regression tree analysis were performed on two-thirds of the patients ( learning cohort ) to build a risk score for the composite endpoint, that was validated in the remaining patients ( validation cohort ).\n\n\nRESULTS\nIn the 2128 patients analyzed in the registry, the overall event rate was 9% (153 [7%] deaths, 42 [2%] transplants). In the learning cohort, factors independently associated with the composite endpoint were (1) type of Norwood; (2) postoperative ECMO; (3) discharge with Opiates; (4) No Digoxin at discharge; (5) postoperative Arch obstruction, (6) moderate-to-severe Tricuspid regurgitation without an oxygen requirement, and (7) Extra Oxygen required at discharge in patients with moderate-to-severe tricuspid regurgitation. This model was used to create a weighted risk score ( NEONATE score; 0-76 points), with >75% accuracy in the learning and validation cohorts. In the validation cohort, the event rate in patients with a score >17 was nearly three times those with a score ≤17.\n\n\nCONCLUSIONS\nWe introduce a risk score that can be used post-stage 1 palliation to predict freedom from interstage mortality or transplant.