Risk Factors for Mortality and Circulatory Outcome Among Neonates Prenatally Diagnosed With Ebstein Anomaly or Tricuspid Valve Dysplasia: A Multicenter Study

• Type of Material: Article
• Authors: Lindsay R. Freud, Columbia University; Doff B. McElhinney, Lucile Packard Children’s Hospital; Brian T. Kalish, Harvard Medical School; Maria C. Escobar-Diaz, University of Barcelona; Rukmini Komarlu, Case Western Reserve University; Michael D. Puchalski, University of Utah; Edgar T. Jaeggi, University of Toronto; Anita L. Szwast, University of Pennsylvania; Grace Freire, Johns Hopkins All Children’s Hospital; Stephanie M. Levasseur, Columbia University; Ann Kavanaugh-McHugh, Vanderbilt University; Erik Michelfelder, Emory University; Anita J. Moon-Grady, University of California, San Francisco; Mary T. Donofrio, George Washington University; Lisa W. Howley, University of Colorado; Elif Seda Selamet Tierney, Lucile Packard Children’s Hospital; Bettina F. Cuneo, University of Colorado; Shaine A. Morris, Texas Childrens Hospital; Jay D. Pruetz, University of Southern California; Mary E. van der Velde, University of Michigan; John P. Kovalchin, Ohio State University; Catherine M. Ikemba, University of Texas Southwestern Medical School; Margaret M. Vernon, University of Washington; Cyrus Samai, Emory University; Gary M. Satou, University of California Los Angeles; Nina L. Gotteiner, Northwestern University; Colin K. Phoon, New York University; Norman H. Silverman, Lucile Packard Children’s Hospital; Wayne Tworetzky, Harvard Medical School
• Language: English
• Date: 2020-11-03
• Publisher: WILEY
• Publication Version: Final Published Version
• Copyright Statement: © 2020 The Authors.
• License: Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International
• Final Published Version (URL): http://dx.doi.org/10.1161/JAHA.120.016684
• Title of Journal or Parent Work: JOURNAL OF THE AMERICAN HEART ASSOCIATION
• Volume: 9
• Issue: 21
• Start Page: e016684
• End Page: e016684
• Keywords: tricuspid regurgitation; Ebstein anomaly; palliation; Science & Technology; outcome; Cardiac & Cardiovascular Systems; mortality; Cardiovascular System & Cardiology; right ventricle; congenital heart disease; REPAIR; Life Sciences & Biomedicine; neonate
• Research Categories: Health Sciences, Medicine and Surgery

Abstract

BACKGROUND: In a recent multicenter study of perinatal outcome in fetuses with Ebstein anomaly or tricuspid valve dysplasia, we found that one third of live-born patients died before hospital discharge. We sought to further describe postnatal management strategies and to define risk factors for neonatal mortality and circulatory outcome at discharge. METHODS AND RESULTS: This 23-center, retrospective study from 2005 to 2011 included 243 fetuses with Ebstein anomaly or tricuspid valve dysplasia. Among live-born patients, clinical and echocardiographic factors were evaluated for association with neonatal mortality and palliated versus biventricular circulation at discharge. Of 176 live-born patients, 7 received comfort care, 11 died <24 hours after birth, and 4 had insufficient data. Among 154 remaining patients, 38 (25%) did not survive to discharge. Nearly half (46%) underwent intervention. Mortality differed by procedure; no deaths occurred in patients who underwent right ventricular exclusion. At discharge, 56% of the cohort had a biventricular circulation (13% following intervention) and 19% were palliated. Lower tricuspid regurgitation jet velocity (odds ratio [OR], 2.3 [1.1–5.0], 95% CI, per m/s; P=0.025) and lack of antegrade flow across the pulmonary valve (OR, 4.5 [1.3–14.2]; P=0.015) were associated with neonatal mortality by multivariable logistic regression. These variables, along with smaller pulmonary valve dimension, were also associated with a palliated outcome. CONCLUSIONS: Among neonates with Ebstein anomaly or tricuspid valve dysplasia diagnosed in utero, a variety of management strategies were used across centers, with poor outcomes overall. High-risk patients with low tricuspid regurgitation jet velocity and no antegrade pulmonary blood flow should be considered for right ventricular exclusion to optimize their chance of survival.