Apical displacement of septal and posterior leaflets of tricuspid valve
Although annulus remains in normal position, proximal portions of leaflets are attached to ventricular walls, effectively displacing leaflet hingepoints toward apex
Classic plain film appearance: Massive right-sided cardiomegaly (box-shaped heart)
Category: Cyanotic, (severe) cardiomegaly, normal or decreased pulmonary vascularity
Hemodynamics: Severe tricuspid valve regurgitation
Volume overload to right heart
Right-to-left shunting through patent foramen ovale (PFO) leads to cyanosis
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Echocardiographic Findings
Angiographic Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
Selected References
Tobler D et al: Right heart characteristics and exercise parameters in adults with Ebstein anomaly: new perspectives from cardiac magnetic resonance imaging studies. Int J Cardiol. 165(1):146-50, 2013
Attenhofer Jost CH et al: Prospective comparison of echocardiography versus cardiac magnetic resonance imaging in patients with Ebstein's anomaly. Int J Cardiovasc Imaging. 28(5):1147-59, 2012
Beerepoot JP et al: Case 71: Ebstein anomaly. Radiology. 231(3):747-51, 2004
Chauvaud S et al: Ebstein's anomaly: repair based on functional analysis. Eur J Cardiothorac Surg. 23(4):525-31, 2003
Dearani JA et al: Ebstein's anomaly of the tricuspid valve. In Mavroudis C et al: Pediatric Cardiac Surgery. 3rd ed. Philadelphia: Mosby. 524-36, 2003
MacLellan-Tobert SG et al: Ebstein anomaly of the tricuspid valve. In Moller JH et al: Pediatric Cardiovascular Medicine. 1st ed. Philadelphia: Churchill Livingstone. 461-8, 2000
Ammash NM et al: Mimics of Ebstein's anomaly. Am Heart J. 134(3):508-13, 1997
Choi YH et al: MR imaging of Ebstein's anomaly of the tricuspid valve. AJR Am J Roentgenol. 163(3):539-43, 1994
Eustace S et al: Ebstein's anomaly presenting in adulthood: the role of cine magnetic resonance imaging in diagnosis. Clin Radiol. 49(10):690-2, 1994
Farb A et al: Anatomy and pathology of the right ventricle (including acquired tricuspid and pulmonic valve disease). Cardiol Clin. 10(1):1-21, 1992
Lau MK et al: Magnetic resonance imaging of Ebstein's anomaly: report of two cases. J Formos Med Assoc. 91(12):1205-8, 1992
Saxena A et al: Late noninvasive evaluation of cardiac performance in mildly symptomatic older patients with Ebstein's anomaly of tricuspid valve: role of radionuclide imaging. J Am Coll Cardiol. 17(1):182-6, 1991
Kastler B et al: Potential role of MR imaging in the diagnostic management of Ebstein anomaly in a newborn. J Comput Assist Tomogr. 14(5):825-7, 1990
Link KM et al: MR imaging of Ebstein anomaly: results in four cases. AJR Am J Roentgenol. 150(2):363-7, 1988
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KEY FACTS
Imaging
Top Differential Diagnoses
Pathology
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TERMINOLOGY
Definitions
Apical displacement of septal and posterior leaflets of tricuspid valve
Although annulus remains in normal position, proximal portions of leaflets are attached to ventricular walls, effectively displacing leaflet hingepoints toward apex
Classic plain film appearance: Massive right-sided cardiomegaly (box-shaped heart)
Category: Cyanotic, (severe) cardiomegaly, normal or decreased pulmonary vascularity
Hemodynamics: Severe tricuspid valve regurgitation
Volume overload to right heart
Right-to-left shunting through patent foramen ovale (PFO) leads to cyanosis
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Echocardiographic Findings
Angiographic Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
Selected References
Tobler D et al: Right heart characteristics and exercise parameters in adults with Ebstein anomaly: new perspectives from cardiac magnetic resonance imaging studies. Int J Cardiol. 165(1):146-50, 2013
Attenhofer Jost CH et al: Prospective comparison of echocardiography versus cardiac magnetic resonance imaging in patients with Ebstein's anomaly. Int J Cardiovasc Imaging. 28(5):1147-59, 2012
Beerepoot JP et al: Case 71: Ebstein anomaly. Radiology. 231(3):747-51, 2004
Chauvaud S et al: Ebstein's anomaly: repair based on functional analysis. Eur J Cardiothorac Surg. 23(4):525-31, 2003
Dearani JA et al: Ebstein's anomaly of the tricuspid valve. In Mavroudis C et al: Pediatric Cardiac Surgery. 3rd ed. Philadelphia: Mosby. 524-36, 2003
MacLellan-Tobert SG et al: Ebstein anomaly of the tricuspid valve. In Moller JH et al: Pediatric Cardiovascular Medicine. 1st ed. Philadelphia: Churchill Livingstone. 461-8, 2000
Ammash NM et al: Mimics of Ebstein's anomaly. Am Heart J. 134(3):508-13, 1997
Choi YH et al: MR imaging of Ebstein's anomaly of the tricuspid valve. AJR Am J Roentgenol. 163(3):539-43, 1994
Eustace S et al: Ebstein's anomaly presenting in adulthood: the role of cine magnetic resonance imaging in diagnosis. Clin Radiol. 49(10):690-2, 1994
Farb A et al: Anatomy and pathology of the right ventricle (including acquired tricuspid and pulmonic valve disease). Cardiol Clin. 10(1):1-21, 1992
Lau MK et al: Magnetic resonance imaging of Ebstein's anomaly: report of two cases. J Formos Med Assoc. 91(12):1205-8, 1992
Saxena A et al: Late noninvasive evaluation of cardiac performance in mildly symptomatic older patients with Ebstein's anomaly of tricuspid valve: role of radionuclide imaging. J Am Coll Cardiol. 17(1):182-6, 1991
Kastler B et al: Potential role of MR imaging in the diagnostic management of Ebstein anomaly in a newborn. J Comput Assist Tomogr. 14(5):825-7, 1990
Link KM et al: MR imaging of Ebstein anomaly: results in four cases. AJR Am J Roentgenol. 150(2):363-7, 1988
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