Abstract
Ebstein’s anomaly is a rare congenital heart disease that affects approximately 1 in 200,000 live births, and <1 % of all congenital heart diseases [1]. The anomaly was originally described in 1866 by Dr. Wilhelm Ebstein in a patient with progressive tricuspid insufficiency due to a congenital malformation [2]. Until 1950s, the diagnosis of Ebstein’s anomaly was mainly based on autopsy findings. It was not until 1951 when Soloff et al. first described a method of diagnosing Ebstein’s anomaly while the patient is still alive by invasive angiogram [3]. In the 1970s, with the development of M-mode, two-dimensional, and Doppler echocardiography, echocardiography became the primary modality in diagnosing Ebstein’s anomaly [4]. However, echocardiography is highly dependent on operator experience, the availability of good acoustic windows, and spatial resolution distal to the probe. Due to the position of the right ventricle being directly behind the sternum and its complex geometry, imaging the right ventricle with echocardiogram is often difficult (Movie 8.1).
This is a preview of subscription content, log in via an institution.
Buying options
Tax calculation will be finalised at checkout
Purchases are for personal use only
Learn about institutional subscriptionsReferences
Attenhofer Jost CH, et al. Ebstein’s anomaly. Circulation. 2007;115(2):277–85.
Ebstein W. Ueber einen sehr seltenen Fall von insufficienz der valvula tricuspidatis, bedingt durch eine angeborene hochgradige Missbildung derselben. Arch Anat Physiol. 1866;33:238–54.
Soloff LA, Stauffer HM, Zatuchni J. Ebstein’s disease: report of the first case diagnosed during life. Am J Med Sci. 1951;222(5):554–61.
Shiina A, et al. Two-dimensional echocardiographic spectrum of Ebstein’s anomaly: detailed anatomic assessment. J Am Coll Cardiol. 1984;3(2 Pt 1):356–70.
Edwards WD. Embryology and pathologic features of Ebstein’s anomaly. Prog Pediatr Cardiol. 1993;2(1):5–15.
Carpentier A, et al. A new reconstructive operation for Ebstein’s anomaly of the tricuspid valve. J Thorac Cardiovasc Surg. 1988;96(1):92–101.
Celermajer DS, et al. Ebstein’s anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol. 1994;23(1):170–6.
Grothues F, et al. Interstudy reproducibility of right ventricular volumes, function, and mass with cardiovascular magnetic resonance. Am Heart J. 2004;147(2):218–23.
Alfakih K, et al. Comparison of right ventricular volume measurements between axial and short axis orientation using steady-state free precession magnetic resonance imaging. J Magn Reson Imaging. 2003;18(1):25–32.
Holland BJ, Printz BF, Lai WW. Baseline correction of phase-contrast images in congenital cardiovascular magnetic resonance. J Cardiovasc Magn Reson. 2010;12:11.
Nakamura I, et al. Ebstein anomaly by cardiac magnetic resonance imaging. J Am Coll Cardiol. 2009;53(17):1568.
Dearani JA, Danielson GK. Congenital heart surgery nomenclature and database project: Ebstein’s anomaly and tricuspid valve disease. Ann Thorac Surg. 2000;69(4 Suppl):S106–17.
Chuah SY, Hughes-Nurse J, Rowlands DB. A successful pregnancy in a patient with congenital tricuspid stenosis and a patent oval foramen. Int J Cardiol. 1992;34(1):112–4.
Khan AN, Boatman J, Anderson AS. Management of new-onset congestive heart failure in a patient with complex congenital heart disease. Congest Heart Fail. 2002;8(1):54–6.
Krishnamoorthy KM. Balloon dilatation of isolated congenital tricuspid stenosis. Int J Cardiol. 2003;89(1):119–21.
Kobza R, et al. Aberrant tendinous chords with tethering of the tricuspid leaflets: a congenital anomaly causing severe tricuspid regurgitation. Heart. 2004;90(3):319–23.
Motoyoshi N, et al. Cleft on tricuspid anterior leaflet. Ann Thorac Surg. 2001;71(4):1350–1.
Katogi T, et al. Surgical management of isolated congenital tricuspid regurgitation. Ann Thorac Surg. 1998;66(5):1571–4.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
8.1 Electronic Supplementary Material
Below is the link to the electronic supplementary material.
Echocardiogram in apical 4 chamber orientation. Abnormal and apically displaced tricuspid valve is shown. Right ventricle is severely dilated (AVI 2080 KB)
218193_1_En_8_MOESM2_ESM.avi
4-Chamber SSFP cine demonstrating the apical displacement of the tricuspid valve, along with turbulent jet of tricuspid regurgitation (AVI 404 KB)
In-plane phase contrast in 4-Chamber view demonstrating tricuspid regurgitation (AVI 55592 KB)
Right ventricular outflow tract SSFP cine (AVI 69489 KB)
218193_1_En_8_MOESM5_ESM.avi
4-Chamber SSFP cine demonstrating hypoplastic tricuspid annulus. There is prominent intrapericardial fat in the atrioventricular groove (arrows) and small pericardial effusion (asterisk) (AVI 97974 KB)
Movie 8.2
4-Chamber SSFP cine demonstrating the apical displacement of the tricuspid valve, along with turbulent jet of tricuspid regurgitation (AVI 404 KB)
Movie 8.5
4-Chamber SSFP cine demonstrating hypoplastic tricuspid annulus. There is prominent intrapericardial fat in the atrioventricular groove (arrows) and small pericardial effusion (asterisk) (AVI 97974 KB)
Rights and permissions
Copyright information
© 2012 Springer-Verlag London
About this chapter
Cite this chapter
Leung, S.W., Syed, M.A. (2012). Ebstein’s Anomaly and Other Tricuspid Valve Anomalies. In: Syed, M., Mohiaddin, R. (eds) Magnetic Resonance Imaging of Congenital Heart Disease. Springer, London. https://doi.org/10.1007/978-1-4471-4267-6_8
Download citation
DOI: https://doi.org/10.1007/978-1-4471-4267-6_8
Published:
Publisher Name: Springer, London
Print ISBN: 978-1-4471-4266-9
Online ISBN: 978-1-4471-4267-6
eBook Packages: MedicineMedicine (R0)