Hostname: page-component-76fb5796d-skm99 Total loading time: 0 Render date: 2024-04-26T14:57:39.613Z Has data issue: false hasContentIssue false
  • English
  • Français

Impact of prenatal haemodynamic and functional abnormalities in Ebstein’s anomaly on survival

Published online by Cambridge University Press:  29 December 2014

Grace Freire ,
Thieu Nguyen ,
Priya Sekar ,
Marilyn Wilhm ,
Kathy Arnold ,
Jenny Leshko ,
Ernest K. Amankwah  and
James Huhta
Grace Freire*
Affiliation:
Johns Hopkins All Children’s Heart Institute, All Children’s Hospital, St Petersburg, Florida, United States of America
Thieu Nguyen
Affiliation:
Johns Hopkins All Children’s Heart Institute, All Children’s Hospital, St Petersburg, Florida, United States of America
Priya Sekar
Affiliation:
Department of Cardiology, Johns Hopkins University, Baltimore, Maryland, United States of America
Marilyn Wilhm
Affiliation:
Johns Hopkins All Children’s Heart Institute, All Children’s Hospital, St Petersburg, Florida, United States of America
Kathy Arnold
Affiliation:
Johns Hopkins All Children’s Heart Institute, All Children’s Hospital, St Petersburg, Florida, United States of America
Jenny Leshko
Affiliation:
Clinical and Translational Research Office, Johns Hopkins All Children’s Hospital, St Petersburg, Florida, United States of America
Ernest K. Amankwah
Affiliation:
Clinical and Translational Research Office, Johns Hopkins All Children’s Hospital, St Petersburg, Florida, United States of America
James Huhta
Affiliation:
Department of Perinatal Cardiology, All Children’s Hospital, St Petersburg, Florida, United States of America Department of Pediatrics, University of Florida, Gainesville, Florida, United States of America
*
Correspondence to: Dr G. Freire, MD, Johns Hopkins All Children’s Heart Institute, 2nd Floor Outpatient Care Center # 206, 601 5th Street South, Saint Petersburg, FL 33701, United States of America. Tel: +727 767 6483; Fax: +727 767 8990; E-mail: gfreire1@jhmi.edu
Get access Rights & Permissions [Opens in a new window]

Abstract

Predicting outcomes of foetuses with Ebstein’s anomaly and tricuspid valve dysplasia continues to be challenging. Limited data exist on the prognostic significance of prenatal haemodynamic and functional parameters in this population. Our aim was to investigate the prognostic significance of haemodynamic and ventricular functional parameters in addition to associated morphometric parameters in patients with Ebstein’s anomaly. We reviewed medical records of foetuses with Ebstein’s anomaly and tricuspid valve dysplasia at All Children’s Hospital Johns Hopkins Medicine and Johns Hopkins University between 2005 and 2012. The main outcome was survival past 30 days from birth; participants who died in utero or <30 days after birth were considered non-survivors. There were 13 survivors and seven non-survivors. We found that participants with abnormal right ventricular function predicted by low tricuspid regurgitation velocity (<2.3 m/second) (p=0.012) and low estimated right ventricular pressure (<24 mmHg) (p=0.029), a low (<7) cardiovascular profile score (p=0.029) and high (>0.53) cardiothoracic ratio (p=0.008) at the first foetal echocardiogram were less likely to survive. In addition, participants with a fossa ovalis/atrial septal length ratio <0.36 at the last foetal echocardiogram (p=0.051) were more likely to die, albeit of borderline statistical significance. Low tricuspid regurgitation velocity and low right ventricular estimated pressure, or a low cardiovascular profile score could be potential prognostic factors for Ebstein’s anomaly and tricuspid valve dysplasia. However, future larger prospective studies are needed to confirm these initial findings.

Keywords

Ebstein’s anomalycardiovascular profile scorecardiothoracic ratiofossa ovalis/atrial septal length ratiocerebroplacental ratioright ventricular pressure
Type
Original Article
Information
Cardiology in the Young , Volume 24 , Issue 6: HeartWeek 2014 , December 2014 , pp. 1049 - 1056
Copyright
© Cambridge University Press 2014 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1.Nora, JJ, Nora, AH, Toews, WH. Letter: lithium, Ebsteinʼs anomaly, and other congenital heart defects. Lancet 1974; 2: 594–595.CrossRefGoogle ScholarPubMed
2.Fyler, DC, Buckley, LLP, Hellenbrand, WE, et al. Report of the New England Regional Infant Cardiac Program. Pediatrics 1980; 65: 375–461.Google Scholar
3.Hornberger, LK, Sahn, DJ, Kleinman, CS, Copel, JA, Reed, KL. Tricuspid valve disease with significant tricuspid insufficiency in the fetus: diagnosis and outcome. J Am Coll Cardiol 1991; 17: 167–173.Google Scholar
4.Luis-Miranda, RS, Arias-Monroy, LG, Alcantar-Mendoza, MA, Arroyo, CC, Reyes, EM, Jarer, SC. Fetal diagnosis and prognosis of Ebsteinʼs anomaly. Ginecol Obstet Mex 2013; 81: 221–230.Google Scholar
5.Yu, JJ, Yun, TJ, Won, HS, et al. Outcome of neonates with Ebsteinʼs anomaly in the current era. Pediatr Cardiol 2013; 34: 1590–1596.Google Scholar
6.McElhinney, DB, Salvin, JW, Colan, SD, et al. Improving outcomes in fetuses and neonates with congenital displacement (Ebsteinʼs malformation) or dysplasia of the tricuspid valve. Am J Cardiol 2005; 96: 582–586.Google Scholar
7.Barre, E, Durand, I, Hazelzet, T, David, N. Ebsteinʼs anomaly and tricuspid valve dysplasia: prognosis after diagnosis in utero. Pediatr Cardiol 2012; 33: 1391–1396.CrossRefGoogle ScholarPubMed
8.Lasa, JJ, Tian, ZY, Guo, R, Rychik, J. Perinatal course of Ebsteinʼs anomaly and tricuspid valve dysplasia in the fetus. Prenat Diagn 2012; 32: 245–251.CrossRefGoogle ScholarPubMed
9.Andrews, RE, Tibby, SM, Sharland, GK, Simpson, JM. Prediction of outcome of tricuspid valve malformations diagnosed during fetal life. Am J Cardiol 2008; 101: 1046–1050.CrossRefGoogle ScholarPubMed
10.Pavlova, M, Fouron, JC, Drblik, SP, et al. Factors affecting the prognosis of Ebsteinʼs anomaly during fetal life. Am Heart J 1998; 135 (Pt 1): 1081–1085.Google Scholar
11.Ishii, T, Tworetzky, W, Harrild, DM, Marcus, EN, McElhinney, DB. Left ventricular function and geometry in fetuses with severe tricuspid regurgitation. Ultrasound Obstet Gynecol 2012; 40: 55–61.CrossRefGoogle ScholarPubMed
12.Neves, AL, Mathias, L, Wilhm, M, et al. Evaluation of prenatal risk factors for prediction of outcome in right heart lesions: CVP score in fetal right heart defects. J Matern Fetal Neonatal Med 2014; 27: 1431–1437.Google Scholar
13.Wieczorek, A, Hernandez-Robles, J, Ewing, L, Leshko, J, Luther, S, Huhta, J. Prediction of outcome of fetal congenital heart disease using a cardiovascular profile score. Ultrasound Obstet Gynecol 2008; 31: 284–288.CrossRefGoogle ScholarPubMed
14.Huhta, JC. Right ventricular function in the human fetus. J Perinat Med 2001; 29: 381–389.Google Scholar
15.Inamura, N, Taketazu, M, Smallhorn, JF, Hornberger, LK. Left ventricular myocardial performance in the fetus with severe tricuspid valve disease and tricuspid insufficiency. Am J Perinatol 2005; 22: 91-.CrossRefGoogle ScholarPubMed
16.Chaoui, R, Bollmann, R, Goldner, B, et al. Cardiomegaly: echocardiographic findings and outcome in 19 cases. Fetal Diagn Ther 1994; 9: 92–104.CrossRefGoogle Scholar