EP09.91: Stratification of fetal therapy indication for the fetus with severe tricuspid valve dysplasia

Tricuspid valve dysplasia (TVD), exemplified by Ebstein anomaly varies in severity, especially with severe cases of circular shunt (CS) during fetal development potentially leading to intrauterine fetal death (IUFD). Recently, administering NSAIDs to pregnant women to constrict the fetal ductus arteriosus and improve fetal circulation has been reported, but its indications are controversy. The aims are to elucidate TVD severity and clinical course and explore suitable cases for fetal treatment. We present SAS score, TRIPP score and clinical results for 20 TVD cases from 2013 to 2023. SAS scores range from 0-2 for 5 parameters (total 10 points), and TRIPP scores range from 0-2 for 4 parameters (total 8 points). Mean maternal age was 31.9 years, gestational age at first fetal ultrasound examination was 31.3 weeks, and delivery occurred at 36.3 weeks. Fetal ultrasound findings presented SAS score averaged 4.7 ± 3.1 points, and TRIPP score averaged 3.4 ± 2.7 points. Survival cases scored SAS 3.7 ± 2.8 points/TRIPP 2.8 ± 2.5 points, while IUFD cases scored 7.3 ± 2.8 points/5.5 ± 2.4 points. Using a cutoff of 6 points for SAS score/TRIPP score, mortality prediction sensitivity was 75%, specificity 69%/sensitivity 75%, specificity 81%. One surviving case with a high score had pulmonary atresia, ineligible for fetal treatment. One neonatal death case with a low score had a severe congenital heart disease and maternal adaptation (Miller syndrome), inducing labour at 29 weeks. Excluding these cases, SAS score/TRIPP score cutoff of 6 points had 100% sensitivity, 69% specificity/100% sensitivity, 81% specificity. Stratifying severity via SAS and TRIPP scores is valuable, suggesting scores of 6 or higher are suitable for fetal treatment considering IUFD risk.