Lindsay R Freud1, Maria C Escobar-Diaz1, Brian T Kalish1, Rukmini Komarlu1, Michael D Puchalski1, Edgar T Jaeggi1, Anita L Szwast1, Grace Freire1, Stéphanie M Levasseur1, Ann Kavanaugh-McHugh1, Erik C Michelfelder1, Anita J Moon-Grady1, Mary T Donofrio1, Lisa W Howley1, Elif Seda Selamet Tierney1, Bettina F Cuneo1, Shaine A Morris1, Jay D Pruetz1, Mary E van der Velde1, John P Kovalchin1, Catherine M Ikemba1, Margaret M Vernon1, Cyrus Samai1, Gary M Satou1, Nina L Gotteiner1, Colin K Phoon1, Norman H Silverman1, Doff B McElhinney1, Wayne Tworetzky1. 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, Division of Cardiology, ON, Canada (E.T.J.); Children's Hospital of Philadelphia, Perelman School of Medicine at the University of Pennsylvania, Department of Pediatrics, Division of Cardiology, PA (A.L.S.); All Children's Hospital, Johns Hopkins Medicine, Department of Pediatrics, Division of Cardiology, St. Petersburg, FL (G.F.); Morgan Stanley Children's Hospital of New York-Presbyterian, Columbia University Medical Center, Department of Pediatrics, Division of Cardiology, New York (S.M.L.); Monroe Carell Jr. Children's Hospital, Vanderbilt University School of Medicine, Department of Pediatrics, Division of Cardiology, Nashville, TN (A.K.-M.); Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, Department of Pediatrics, The Heart Institute, OH (E.C.M.); UCSF Benioff Children's Hospital, University of California-San Francisco School of Medicine, Department of Pediatrics, Division of Cardiology (A.J.M.-G., N.H.S.); Children's National Medical Center, Division of Cardiology, George Washington University School of Medicine and Health Sciences, Department of Pediatrics, Washington, DC (M.T.D.); Heart Institute Children's Hospital Colorado, University of Colorado School of Medicine, Department of Pediatrics, Division of Cardiology, Aurora (L.W.H.); Lucile Packard Children's Hospital, Stanford School of Medicine, Department of Pediatrics, Division of Cardiology, Palo, Alto, CA (E.S.S.T., N.H.S., D.B.M.); Advocate Children's Hospital, Oak
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.
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.
Authors: Tristan K W Ramcharan; Donna A Goff; Christopher E Greenleaf; Suhair O Shebani; Jorge D Salazar; Antonio F Corno Journal: Pediatr Cardiol Date: 2022-04-23 Impact factor: 1.838
Authors: Laura I Parikh; Katherine L Grantz; Sara N Iqbal; Chun-Chih Huang; Helain J Landy; Melissa H Fries; Uma M Reddy Journal: Am J Obstet Gynecol Date: 2017-05-31 Impact factor: 8.661