Abnormal Microarray, Clinical Outcomes, and Surgical Risk Scores in Young Children with Cardiac Disease.

Abstract

The clinical implications of abnormal chromosomal microarray (CMA) remain unclear for children less than 1year of age with critical heart disease. Our objective was to determine whether abnormal CMA was related to surgical severity scores or to pre-determined clinical outcomes, including cardiac arrest. Retrospective review of children under 1year of age admitted to a pediatric cardiac intensive care unit from December, 2014 to September, 2017. Associations between CMA result and cardiac arrest, syndromic abnormalities, and extracardiac anomalies were evaluated. A simple and multivariable logistic regression model was used to analyze associations between STAT mortality category and CMA result. The overall prevalence of abnormal microarray was 48/168 (29%), with peak prevalence in AV septal defects and left-sided obstructive lesions. There was no statistical association between surgical severity scores and abnormal CMA (STAT 1/2 vs. 3+, odds ratio 0.56, p = 0.196). Abnormal CMA was associated with a higher prevalence of cardiac arrest (5/48 abnormal CMA vs. 2/120 normal CMA, p = 0.02). Abnormal CMA was associated with a higher frequency of syndromic abnormalities (18/48 abnormal CMA vs. 13/120 normal CMA, p < 0.001). There was a high prevalence of abnormal CMA findings in the pediatric cardiac population less than 1year of age (29%), associated with cardiac arrest, but not associated with surgical risk score. The absence of a standardized protocol for ordering a CMA in the setting of congenital heart disease results in a highly variable prevalence data.