TY - JOUR
T1 - Risk Factors for Mortality and Circulatory Outcome Among Neonates Prenatally Diagnosed With Ebstein Anomaly or Tricuspid Valve Dysplasia
T2 - A Multicenter Study
AU - Freud, Lindsay R.
AU - McElhinney, Doff B.
AU - Kalish, Brian T.
AU - Escobar-Diaz, Maria C.
AU - Komarlu, Rukmini
AU - Puchalski, Michael D.
AU - Jaeggi, Edgar T.
AU - Szwast, Anita L.
AU - Freire, Grace
AU - Levasseur, Stéphanie M.
AU - Kavanaugh-McHugh, Ann
AU - Michelfelder, Erik C.
AU - Moon-Grady, Anita J.
AU - Donofrio, Mary T.
AU - Howley, Lisa W.
AU - Selamet Tierney, Elif Seda
AU - Cuneo, Bettina F.
AU - Morris, Shaine A.
AU - Pruetz, Jay D.
AU - van der Velde, Mary E.
AU - Kovalchin, John P.
AU - Ikemba, Catherine M.
AU - Vernon, Margaret M.
AU - Samai, Cyrus
AU - Satou, Gary M.
AU - Gotteiner, Nina L.
AU - Phoon, Colin K.
AU - Silverman, Norman H.
AU - Tworetzky, Wayne
N1 - Copyright:
This record is sourced from MEDLINE/PubMed, a database of the U.S. National Library of Medicine
PY - 2020/11/3
Y1 - 2020/11/3
N2 - 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.
AB - 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.
KW - Ebstein anomaly
KW - congenital heart disease
KW - mortality
KW - neonate
KW - outcome
KW - palliation
KW - right ventricle
KW - tricuspid regurgitation
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U2 - 10.1161/JAHA.120.016684
DO - 10.1161/JAHA.120.016684
M3 - Article
C2 - 33076749
AN - SCOPUS:85095671040
VL - 9
SP - e016684
JO - Journal of the American Heart Association
JF - Journal of the American Heart Association
SN - 2047-9980
IS - 21
ER -