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KEY FACTS
Terminology
Imaging
Pathology
Clinical Issues
TERMINOLOGY
Definitions
- Downward/apical displacement of septal & posterior leaflets of tricuspid valve → tricuspid regurgitation
- Classic radiographic appearance: Massive right-sided cardiomegaly (box-shaped heart)
- Category: Cyanotic, (severe) cardiomegaly, normal or ↓ pulmonary vascularity
- Hemodynamics: Determined by severity of tricuspid valve regurgitation
- Volume overload to right heart
- Right-to-left shunting through patent foramen ovale (PFO) → cyanosis
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Echocardiographic Findings
Angiographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
Selected References
- Qureshi MY et al: Commentary: gold or silver? Value of cardiac magnetic resonance imaging over echocardiography in Ebstein's anomaly. J Thorac Cardiovasc Surg. 161(3):1109-10, 2021
- Rajagopal R et al: Ebstein's anomaly with imperforate tricuspid valve: an extremely rare congenital anomaly. J Cardiovasc Comput Tomogr. 14(6):e95-6, 2020
- Holst KA et al: Ebstein's anomaly. Methodist Debakey Cardiovasc J. 15(2):138-44, 2019
- Mrad Agua K et al: Preoperative predictability of right ventricular failure following surgery for Ebstein's anomaly. Eur J Cardiothorac Surg. 55(6):1187-93, 2019
- Qureshi MY et al: Tricuspid valve imaging and intervention in pediatric and adult patients with congenital heart disease. JACC Cardiovasc Imaging. 12(4):637-51, 2019
- Ciepłucha A et al: Clinical aspects of myocardial fibrosis in adults with Ebstein's anomaly. Heart Vessels. 33(9):1076-85, 2018
- Qureshi MY et al: Cardiac imaging in Ebstein anomaly. Trends Cardiovasc Med. 28(6):403-9, 2018
- Enaba MM et al: Multidetector computed tomography (CT) in evaluation of congenital cyanotic heart diseases. Pol J Radiol. 82:645-59, 2017
- Kumar P et al: Ebstein anomaly with right atrial clot. Cardiol Res. 6(4-5):319-23, 2015
- Kühn A et al: Non-volumetric echocardiographic indices and qualitative assessment of right ventricular systolic function in Ebstein's anomaly: comparison with CMR-derived ejection fraction in 49 patients. Eur Heart J Cardiovasc Imaging. 17(8):930-5, 2016
- Li X et al: More than valve repair: effect of cone reconstruction on right ventricular geometry and function in patients with Ebstein anomaly. Int J Cardiol. 206:131-7, 2016
- Malik SB et al: The right atrium: gateway to the heart--anatomic and pathologic imaging findings. Radiographics. 35(1):14-31, 2015
- Arya P et al: Ebstein anomaly: assessment, management, and timing of intervention. Curr Treat Options Cardiovasc Med. 16(10):338, 2014
- Hösch O et al: The total right/left-volume index: a new and simplified cardiac magnetic resonance measure to evaluate the severity of Ebstein anomaly of the tricuspid valve: a comparison with heart failure markers from various modalities. Circ Cardiovasc Imaging. 7(4):601-9, 2014
- Bonello B et al: Review of the role of cardiovascular magnetic resonance in congenital heart disease, with a focus on right ventricle assessment. Arch Cardiovasc Dis. 105(11):605-13, 2012
- Zikria JF et al: Common CTA features of Ebstein anomaly in a middle-aged woman with a heart murmur and dyspnea on exertion. J Cardiovasc Comput Tomogr. 6(6):431-2, 2012
- Yalonetsky S et al: Cardiac magnetic resonance imaging and the assessment of ebstein anomaly in adults. Am J Cardiol. 107(5):767-73, 2011
- Nakamura I et al: Ebstein anomaly by cardiac magnetic resonance imaging. J Am Coll Cardiol. 53(17):1568, 2009
Related Anatomy
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Pathology
Clinical Issues
TERMINOLOGY
Definitions
- Downward/apical displacement of septal & posterior leaflets of tricuspid valve → tricuspid regurgitation
- Classic radiographic appearance: Massive right-sided cardiomegaly (box-shaped heart)
- Category: Cyanotic, (severe) cardiomegaly, normal or ↓ pulmonary vascularity
- Hemodynamics: Determined by severity of tricuspid valve regurgitation
- Volume overload to right heart
- Right-to-left shunting through patent foramen ovale (PFO) → cyanosis
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Echocardiographic Findings
Angiographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
Selected References
- Qureshi MY et al: Commentary: gold or silver? Value of cardiac magnetic resonance imaging over echocardiography in Ebstein's anomaly. J Thorac Cardiovasc Surg. 161(3):1109-10, 2021
- Rajagopal R et al: Ebstein's anomaly with imperforate tricuspid valve: an extremely rare congenital anomaly. J Cardiovasc Comput Tomogr. 14(6):e95-6, 2020
- Holst KA et al: Ebstein's anomaly. Methodist Debakey Cardiovasc J. 15(2):138-44, 2019
- Mrad Agua K et al: Preoperative predictability of right ventricular failure following surgery for Ebstein's anomaly. Eur J Cardiothorac Surg. 55(6):1187-93, 2019
- Qureshi MY et al: Tricuspid valve imaging and intervention in pediatric and adult patients with congenital heart disease. JACC Cardiovasc Imaging. 12(4):637-51, 2019
- Ciepłucha A et al: Clinical aspects of myocardial fibrosis in adults with Ebstein's anomaly. Heart Vessels. 33(9):1076-85, 2018
- Qureshi MY et al: Cardiac imaging in Ebstein anomaly. Trends Cardiovasc Med. 28(6):403-9, 2018
- Enaba MM et al: Multidetector computed tomography (CT) in evaluation of congenital cyanotic heart diseases. Pol J Radiol. 82:645-59, 2017
- Kumar P et al: Ebstein anomaly with right atrial clot. Cardiol Res. 6(4-5):319-23, 2015
- Kühn A et al: Non-volumetric echocardiographic indices and qualitative assessment of right ventricular systolic function in Ebstein's anomaly: comparison with CMR-derived ejection fraction in 49 patients. Eur Heart J Cardiovasc Imaging. 17(8):930-5, 2016
- Li X et al: More than valve repair: effect of cone reconstruction on right ventricular geometry and function in patients with Ebstein anomaly. Int J Cardiol. 206:131-7, 2016
- Malik SB et al: The right atrium: gateway to the heart--anatomic and pathologic imaging findings. Radiographics. 35(1):14-31, 2015
- Arya P et al: Ebstein anomaly: assessment, management, and timing of intervention. Curr Treat Options Cardiovasc Med. 16(10):338, 2014
- Hösch O et al: The total right/left-volume index: a new and simplified cardiac magnetic resonance measure to evaluate the severity of Ebstein anomaly of the tricuspid valve: a comparison with heart failure markers from various modalities. Circ Cardiovasc Imaging. 7(4):601-9, 2014
- Bonello B et al: Review of the role of cardiovascular magnetic resonance in congenital heart disease, with a focus on right ventricle assessment. Arch Cardiovasc Dis. 105(11):605-13, 2012
- Zikria JF et al: Common CTA features of Ebstein anomaly in a middle-aged woman with a heart murmur and dyspnea on exertion. J Cardiovasc Comput Tomogr. 6(6):431-2, 2012
- Yalonetsky S et al: Cardiac magnetic resonance imaging and the assessment of ebstein anomaly in adults. Am J Cardiol. 107(5):767-73, 2011
- Nakamura I et al: Ebstein anomaly by cardiac magnetic resonance imaging. J Am Coll Cardiol. 53(17):1568, 2009
