부산대학교 도서관

Introduction: Ebstein anomaly and tricuspid valve dysplasia (EA/TVD) are congenital malformations associated with high perinatal mortality. In a recent multi-center study of fetuses with EA/TVD, we found that early gestational age (GA) at diagnosis, large tricuspid valve (TV) annulus Z-score, presence of pulmonary regurgitation (PR), and pericardial effusion predicted mortality. We sought to describe the extracardiac Doppler indices in this cohort and to assess their additive value in predicting mortality. Methods: Fetuses with EA/TVD from 23 centers between 2005 and 2011 were included for this retrospective study. Extracardiac Doppler indices from the last available fetal echo were collected, including pattern and velocity of the umbilical artery (UA), umbilical vein (UV), ductus venosus (DV), and middle cerebral artery (MCA). Pulsatility and resistive indices (PI and RI) of the UA and MCA as well as cerebroplacental ratio (CPR) were calculated. The primary outcome was perinatal mortality, defined as fetal demise or neonatal death prior to discharge. Univariable and multivariable analyses were performed with the aforementioned predictors of mortality. Results: Of 243 cases, 213 had umbilical Doppler data and 96 had all extracardiac Doppler indices for analysis. At a mean GA of 30.2 ± 6 weeks, 12.2% had abnormal UA, 14.8% abnormal UV, 18.8% abnormal DV, and 7.4% abnormal MCA patterns. The presence of PR was highly associated with increased UA PI (P <0.001). Table 1 summarizes the associations of the indices with mortality. On multivariable analysis, abnormally elevated UA PI and UA PI Z-score as well as lower UV mean velocity and UV mean velocity Z-score were significant independent predictors of mortality while abnormal DV, MCA and CPR indices were not. Conclusions: Elevated UA PI and decreased UV velocity are independent predictors of mortality in fetuses with EA/TVD and should be routinely evaluated. Elevated UA PI may be due to altered cardiac hemodynamics, as evidenced by the high association with PR, while decreased UV velocity may be due to abnormal central venous pressure. Our findings also suggest that the fetal-placental relationship plays a crucial role in the health of these fetuses, which warrants further investigation. [ABSTRACT FROM AUTHOR]