Cardiovascular Profile Score and Perinatal Survival Among Fetuses With Ebstein's Anomaly or Tricuspid Valve Dysplasia: A Multi-Center Retrospective Cohort Study.

OBJECTIVE: We sought to perform multi-variable modeling to assess the independent value of the CVPS in fetuses with Ebstein anomaly or tricuspid valve dysplasia (EA/TVD).

METHODS: CVPS was assessed at a core lab using the first and last echocardiograms during gestation. A receiver operating characteristic (ROC) curve analysis was conducted. Changes in the CVPS from the first to the last echo were assessed with Wilcoxon signed-rank tests.

RESULTS: There were 164 fetuses with EA/TVD with complete CVPS at the first echo. Nearly half, 48.8% (n = 80), had intrauterine fetal demise (IUFD) or died neonatally. At the first echo, median gestational age (GA) was 27.6 weeks (IQR: 23.0-31.0) and median CVPS was 7 (IQR: 6-8). The optimal cut-point for classification of perinatal survival was observed at CVPS ≥ 6.5 (Youden index = 0.46). After adjustment, there remained a significant independent association between every 1-point increase in the CVPS at first echo and the odds of perinatal survival (adjusted odds ratio: 2.0, 95% CI: 1.3 to 3.2, and p = 0.003). The CVPS at the last echo decreased by a median of 1 point among both survivors (p < 0.01) and non-survivors (p < 0.001).

CONCLUSION: Among fetuses with EA/TVD, the CVPS may be used as an additional tool to assess perinatal survival throughout gestation.