Abstract 18132: Prenatal Evaluation of the Fetal Cardiovascular Profile Score in a Multicenter Study of Ebstein Anomaly/Tricuspid Valve Dysplasia

Abstract

Introduction: The cardiovascular profile (CVP) score is an echo based multivariable approach for assessment of fetal hemodynamics. The CVP score has been shown to be useful in predicting the outcome of fetuses with heart failure, growth restriction, and select congenital heart defects. The score ranges from 0 to 10, with points deducted for abnormal hemodynamics. In a previous pilot study of 21 fetuses with EA/TVD, a CVP score <7 was associated with poor outcomes. The aim of this study was to investigate the prognostic significance of the CVP score in a larger cohort of fetuses with EA/TVD. Methods: This 23-center study was a retrospective review of fetal echo data and outcomes in a cohort of 243 fetuses diagnosed with EA/TVD from 2005-2011. Both the first and last fetal echo in gestation were evaluated. Hydrops, heart size, cardiac function, and umbilical arterial and venous Dopplers were scored. The primary outcome was perinatal death, defined as fetal demise or death prior to neonatal discharge. Categorical variables were compared using Chi-squared tests; p-values <0.05 were considered statistically significant. Results: Complete data on the first and last fetal echocardiograms to calculate a CVP score were available in 144 fetuses at the time of the first fetal echo (median GA 26.07 wks, range 14-40) and 73 fetuses at the time of the last fetal echo (median GA: 25 wks, range 18 -34). The median CVP scores for the first and last fetal echo were 8 (range 4-9) and 7 (range 7-9) among survivors vs. 7 (range 0-10) and 6 (range 1-8) among non-survivors. Fetuses with EA/TVD that died were more likely to have a CVP score <7 compared to those that survived: 33.3% vs. 11.6 %, (p=0.004) on the first fetal echo and 60.0% vs. 28.6 % (p=0.015) on the last fetal echo. However, no statistically significant difference in survival was observed for fetuses whose CVP score decreased from the first to the last echo. (p=0.95). Conclusions: In this study, fetuses with EA/TVD with CVP scores <7 on the first or last fetal echo were less likely to survive. The CVP score may be a valuable tool to identify fetuses with EA/TVD at risk of poor outcome for whom novel therapeutic approaches might be warranted.