**Neonatal Ebstein's Anomaly**

**Neonatal Ebstein's Anomaly**

Umar Boston, Ken-Michael Bayle, TK Susheel Kumar and Christopher Knott-Craig TK Susheel Kumar and Christopher Knott-Craig Additional information is available at the end of the chapter

Additional information is available at the end of the chapter

http://dx.doi.org/10.5772/intechopen.72891

Umar Boston, Ken-Michael Bayle,

#### **Abstract**

[84] Souka AP, von Kaisenberg CS, Hyett JA, Sonek JD, Nicolaides KH. Increased nuchal translucency with normal karyotype. American Journal of Obstetrics and Gynecology.

[85] Makrydimas G, Sotiriadis A, Huggon IC, Simpson J, Sharland, Carvalho JS, Daubeney PE, Ioannidis JPA. Nuchal translucency and fetal cardiac defects: A pooled analysis of major fetal echocardiography centers. American Journal of Obstetrics and Gynecology.

[86] Clur SA, Ottenkamp J, Bilardo CM. The nuchal translucency and the fetal heart: A litera-

[87] Souka AP, von Kaisenberg CS, Hyett JA, Sonek JD, Nicolaides KH. Increased nuchal translucency with normal karyotype. American Journal of Obstetrics and Gynecology.

[88] Bilardo CM, Timmerman E, Pajkrt E, van Maarle M. Increased nuchal translucency in euploid fetuses – what should we be telling the parents? Prenatal Diagnosis.

[89] Mavrides E, Cobian-Sanchez F, Tekay A, Moscoso G, Campbell S, Thilaganathan B, Carvalho JS. Limitations of using first-trimester nuchal translucency measurement in routine screening for major congenital heart defects. Ultrasound in Obstetrics & Gynecology.

[90] Simpson LL, Malone FD, Bianchi DW, Ball RH, Nyberg DA, Comstock CH, et al. Nuchal translucency and the risk of congenital heart disease. Obstetrics and Gynecology. 2007

[91] Maiz N, Nicolaides KH. Ductus Venosus in the first trimester: Contribution to screening of chromosomal, cardiac defects and monochorionic twin complications. Fetal Diagnosis

[92] Faiola S, Tsoi S, Huggon IC, Allan LD, Nicolaides KH. Likelihood ratio for trisomy 21 in fetuses with tricuspid regurgitation at the 11 to 13+6-week scan. Ultrasound in Obstetrics

[93] Grande M, Arigita M, Borobio V, Jimenez JM, Fernandez S, Borrell A. First-trimester detection of structural abnormalities and the role of aneuploidy markers. Ultrasound in

ture review. Prenatal Diagnosis. 2009;**29**:739-748

2005:1005-1021

148 Congenital Anomalies - From the Embryo to the Neonate

2005;192:89-95

2005:1005-1021

2010;**30**:93-102

2001;**17**:106-110

Feb;**109**(2 Pt 1):376-383

and Therapy. 2010;**28**:65-71

& Gynecology. 2005;**26**:22-27

Obstetrics & Gynecology. 2012 Feb;**39**(2):157-163

Ebstein's anomaly is a congenital heart disease that results from failure of delamination of the tricuspid valve with resulting apical displacement of the septal and posterior leaflets of the tricuspid valve. Age at presentation can vary greatly but neonatal presentation is associated with extraordinary high mortality rates. Comprehensive multispecialty care is required starting at the time of fetal diagnosis. Fetal echocardiography is vital in monitoring progression of the disease in utero. Fetal echocardiogram can evaluate for complications such as arrhythmias, pericardial effusion, or fetal hydrops. Post-natal evaluation should include evaluation of functional pulmonary atresia or circular shunt. Despite advances in surgical technique for Ebstein's anomaly, mortality for it remains high with early surgical intervention. Aggressive medical management should be used to support patients with Ebstein's anomaly during the neonatal period. Surgical procedures for neonatal Ebstein's vary widely from systemic to pulmonary shunts with or without tricuspid valve closure to tricuspid valve repair.

DOI: 10.5772/intechopen.72891

**Keywords:** neonatal Ebstein's anomaly, Ebstein's anomaly, tricuspid valve dysplasia, Fetal Ebstein
