Abstract 17047: Pathophysiologic Progression From the Second to Third Trimester in Fetuses With Ebstein's Anomaly or Tricuspid Valve Dysplasia: Are Early Echocardiographic Features Reliable Indicators of Late Gestation Status?
Background: Ebstein’s anomaly and tricuspid valve dysplasia (EA/TVD) are associated with high perinatal mortality. Poor hemodynamic status in both early and late gestation is associated with worse outcome. However, it is not known whether EA/TVD fetuses with more favorable physiology earlier in gestation progress to more severe disease in 3rd trimester. The goal of this study was to evaluate whether initial echocardiographic indices in fetuses with EA/TVD presenting at gestational age (GA) <24 wks are reliable indicators of physiologic status later in pregnancy.
Methods: This multi-center, retrospective study included fetuses diagnosed with EA/TVD from 2005-11 at 23 centers. Patients with initial diagnosis at <24 wks gestation and with ≥2 fetal echocardiograms ≥4 wks apart were included. A core laboratory analyzed the echocardiograms. Markers of poor outcome were defined as absence of antegrade flow across the pulmonary valve, pulmonary valve regurgitation (PR), cardiothoracic ratio >0.48, left ventricular (LV) dysfunction or TV annulus Z score >5.6.
Results: The study included 51 fetuses. Median GA at diagnosis was 21 wks [18-24], and the median duration from 2nd to 3rd trimester echocardiograms was 12 wks [4-18]. There were 21 fetuses (41%) with >2 markers of poor outcome at <24 wks, which increased to 32 (63%) in later gestation (p=0.001). Nine of 27 fetuses (33%) with antegrade pulmonary blood flow on first echocardiogram developed anatomic or functional pulmonary atresia during follow-up, and 7 of 39 fetuses (18%) without PR initially developed it later. LV dysfunction was present in 2 patients at <24 wks but present in 14 (37%) later (p<0.01). The only variable associated with worsening physiology, from <2 markers of poor outcome in 2nd trimester to ≥2 markers in 3rd trimester, was larger TV annulus Z-score (4.1±1.0 vs. 2.4±1.9, p=0.008).
Conclusion: These results confirm a wide spectrum of disease presentation and progression in fetuses with EA/TVD. Thus, it is imperative to assess these fetuses serially. In this cohort, there were no early markers of progression other than larger TV annulus Z-score. Great care must be taken in counseling prior to 24 wks, as the absence of factors associated with poor outcome early in pregnancy may be falsely reassuring.
Author Disclosures: E. Selamet Tierney: None. D.B. McElhinney: None. L.R. Freud: None. W. Tworetzky: None. B. Cueno: None. M.C. Escobar-Diaz: None. C. Ikemba: None. B.T. Kalish: None. R. Komarlu: None. S. Levasseur: None. M.D. Puchalski: None. G.M. Satou: None. N.H. Silverman: None. A.J. Moon-Grady: None.
- © 2015 by American Heart Association, Inc.