Risk factors for mortality and circulatory outcome among neonates prenatally diagnosed with ebstein anomaly or tricuspid valve dysplasia: A multicenter study

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

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish (US)
Article numbere016684
JournalJournal of the American Heart Association
Issue number21
StatePublished - Nov 3 2020
Externally publishedYes


  • Congenital heart disease
  • Ebstein anomaly
  • Mortality
  • Neonate
  • Outcome
  • Palliation
  • Right ventricle
  • Tricuspid regurgitation

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine


Dive into the research topics of 'Risk factors for mortality and circulatory outcome among neonates prenatally diagnosed with ebstein anomaly or tricuspid valve dysplasia: A multicenter study'. Together they form a unique fingerprint.

Cite this