Abstract 17203: Postnatal Management of Fetuses With Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era: A Multi-center Study
Background: A recent multi-center study of perinatal outcome in fetuses with Ebstein anomaly or tricuspid valve dysplasia (EA/TVD) found that 1/3rd of live-born patients (pts) died prior to hospital discharge. The purpose of this study was to explore differences in postnatal management and the relationship to outcome.
Methods: This 23-center, retrospective study included 243 fetuses with EA/TVD from 2005 to 2011. Neonatal procedure (NP) was defined as surgery or interventional catheterization (cath) prior to discharge. Associations between postnatal management and outcome at discharge were explored.
Results: Of 176 live-born pts, 7 received comfort care only, 11 died <24 hrs of life, and 4 had insufficient data. Among 154 remaining pts, 38 (25%) did not survive to discharge. Pts who required ECMO at any point (n=18) had 83% mortality. More than half of pts (54%) did not have an NP, 34% had surgery, 8% had interventional cath, and 4% had both. The median age at 1st NP was 6 days (quartiles: 1-11). Survival did not differ between pts who had an NP and those who did not (70% vs. 80%; p=0.19) or between pts who had surgery and those who did not (68% vs. 80%; p=0.09). However, mortality differed by NP performed and whether pulmonary regurgitation, an indicator of high risk, was present prenatally (Figure). No pts with a right ventricular exclusion (RVE) died. Of 49 surviving neonates with ≥1 procedure, 28 (57%) were palliated with a shunt or RVE and 21 (43%) had a biventricular circulation. Thus, in total, 86 of 154 live-born pts (56%) survived with a biventricular circulation: 65 with medical management only and 21 with ≥1 NP.
Conclusion: Among live-born pts diagnosed with EA/TVD in utero, a variety of postnatal management strategies were employed with overall poor outcomes. If surgery beyond PDA ligation is necessary, then RVE or other palliative procedure may need to be considered. A prospective, multi-center study utilizing a management algorithm would help elucidate the optimal strategy.
Author Disclosures: L.R. Freud: None. B.T. Kalish: None. M.C. Escobar-Diaz: None. R. Komarlu: None. M.D. Puchalski: None. E.T. Jaeggi: None. A.L. Szwast: None. G. Freire: None. S.M. Levasseur: None. A. Kavanaugh-McHugh: None. E.C. Michelfelder: None. A.J. Moon-Grady: None. M.T. Donofrio: None. L.W. Howley: None. E. Selamet Tierney: None. B.F. Cuneo: None. S.A. Morris: None. J.D. Pruetz: None. M.E. van der Velde: None. J.P. Kovalchin: None. C.M. Ikemba: None. M.M. Vernon: None. C. Samai: None. G.M. Satou: None. N.L. Gotteiner: None. C.K. Phoon: None. N.H. Silverman: None. D.B. McElhinney: None. W. Tworetzky: None.
- © 2015 by American Heart Association, Inc.