Perinatal course of Ebstein's anomaly and tricuspid valve dysplasia in the fetus

Objective: We sought to better define echocardiographic predictors of perinatal mortality in fetuses with Ebstein's anomaly (EA) or tricuspid valve dysplasia (TVD). Method: Parameters included measured chamber size, the presence of hydrops, and Doppler recordings of the left ventricular (LV) myocardial performance index (MPI). Results: Between 1 January 2000 and 31 December 2008, 21 fetuses were diagnosed with either EA (17) or TVD (4). Five fetuses were lost to follow-up, and 12 of 16 fetuses were born live (75%). Survivors were found to have smaller right atrial area index scores when compared with non-survivors (1.025±0312 vs 1.502±0.105, respectively, p=0.013) and were less likely to present with hydrops (0% survivors vs 75% of non-survivors, p<0.01). LV MPI sub-analysis revealed a shorter combined isovolemic contraction and relaxation time for non-survivors compared wth survivors (46.5±8.2ms vs 82.3±21.2ms, respectively, p=0.004) although no difference was observed for LV ejection times or overall LV MPI between survivors and non-survivors. Conclusion: Physiologic analysis of left ventricular function via the LV Tei index and its component measurements demonstrates potentially novel insights into hemodynamic derangements and their association with outcomes in patients with EA/TVD.