Outcomes and Predictors of Perinatal Mortality in Fetuses With Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era

Author:

Freud Lindsay R.1,Escobar-Diaz Maria C.1,Kalish Brian T.1,Komarlu Rukmini1,Puchalski Michael D.1,Jaeggi Edgar T.1,Szwast Anita L.1,Freire Grace1,Levasseur Stéphanie M.1,Kavanaugh-McHugh Ann1,Michelfelder Erik C.1,Moon-Grady Anita J.1,Donofrio Mary T.1,Howley Lisa W.1,Tierney Elif Seda Selamet1,Cuneo Bettina F.1,Morris Shaine A.1,Pruetz Jay D.1,van der Velde Mary E.1,Kovalchin John P.1,Ikemba Catherine M.1,Vernon Margaret M.1,Samai Cyrus1,Satou Gary M.1,Gotteiner Nina L.1,Phoon Colin K.1,Silverman Norman H.1,McElhinney Doff B.1,Tworetzky Wayne1

Affiliation:

1. From Boston Children’s Hospital, Department of Cardiology, Harvard Medical School, Department of Pediatrics, MA (L.R.F., M.C.E.-D., R.K., W.T.); Boston Children’s Hospital, Department of Medicine, Harvard Medical School, Department of Pediatrics, MA (B.T.K.); Primary Children’s Hospital, University of Utah School of Medicine, Department of Pediatrics, Division of Cardiology, Salt Lake City (M.D.P.); Hospital for Sick Children, University of Toronto Faculty of Medicine, Department of Pediatrics,...

Abstract

Background— Ebstein anomaly and tricuspid valve dysplasia are rare congenital tricuspid valve malformations associated with high perinatal mortality. The literature consists of small, single-center case series spanning several decades. We performed a multicenter study to assess the outcomes and factors associated with mortality after fetal diagnosis in the current era. Methods and Results— Fetuses diagnosed with Ebstein anomaly and tricuspid valve dysplasia from 2005 to 2011 were included from 23 centers. The primary outcome was perinatal mortality, defined as fetal demise or death before neonatal discharge. Of 243 fetuses diagnosed at a mean gestational age of 27±6 weeks, there were 11 lost to follow-up (5%), 15 terminations (6%), and 41 demises (17%). In the live-born cohort of 176 live-born patients, 56 (32%) died before discharge, yielding an overall perinatal mortality of 45%. Independent predictors of mortality at the time of diagnosis were gestational age <32 weeks (odds ratio, 8.6; 95% confidence interval, 3.5–21.0; P <0.001), tricuspid valve annulus diameter z-score (odds ratio, 1.3; 95% confidence interval, 1.1–1.5; P <0.001), pulmonary regurgitation (odds ratio, 2.9; 95% confidence interval, 1.4–6.2; P <0.001), and a pericardial effusion (odds ratio, 2.5; 95% confidence interval, 1.1–6.0; P =0.04). Nonsurvivors were more likely to have pulmonary regurgitation at any gestational age (61% versus 34%; P <0.001), and lower gestational age and weight at birth (35 versus 37 weeks; 2.5 versus 3.0 kg; both P <0.001). Conclusion— In this large, contemporary series of fetuses with Ebstein anomaly and tricuspid valve dysplasia, perinatal mortality remained high. Fetuses with pulmonary regurgitation, indicating circular shunt physiology, are a high-risk cohort and may benefit from more innovative therapeutic approaches to improve survival.

Publisher

Ovid Technologies (Wolters Kluwer Health)

Subject

Physiology (medical),Cardiology and Cardiovascular Medicine

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