Narrative review of Ebstein's anomaly beyond childhood
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Narrative review of Ebstein's anomaly beyond childhood : Imaging, surgery, and future perspectives. / Neumann, Stephan; Rüffer, André; Sachweh, Jörg; Biermann, Daniel; Herrmann, Jochen; Jerosch-Herold, Michael; Hazekamp, Mark; Sinning, Christoph; Zengin, Elvin; Blankenberg, Stefan; Girdauskas, Evaldas; Reichenspurner, Hermann; Kehl, Torben; Müller, Götz; Kozlik-Feldmann, Rainer; Rickers, Carsten.
In: CARDIOVASC DIAGN THE, Vol. 11, No. 6, 12.2021, p. 1310-1323.Research output: SCORING: Contribution to journal › SCORING: Review article › Research
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TY - JOUR
T1 - Narrative review of Ebstein's anomaly beyond childhood
T2 - Imaging, surgery, and future perspectives
AU - Neumann, Stephan
AU - Rüffer, André
AU - Sachweh, Jörg
AU - Biermann, Daniel
AU - Herrmann, Jochen
AU - Jerosch-Herold, Michael
AU - Hazekamp, Mark
AU - Sinning, Christoph
AU - Zengin, Elvin
AU - Blankenberg, Stefan
AU - Girdauskas, Evaldas
AU - Reichenspurner, Hermann
AU - Kehl, Torben
AU - Müller, Götz
AU - Kozlik-Feldmann, Rainer
AU - Rickers, Carsten
N1 - 2021 Cardiovascular Diagnosis and Therapy. All rights reserved.
PY - 2021/12
Y1 - 2021/12
N2 - Ebstein's anomaly is a rare congenital heart disease with malformation of the tricuspid valve and myopathy of the right ventricle. The septal and inferior leaflets adhere to the endocardium due to failure of delamination. This leads to apical displacement of their hinge points with a shift of the functional tricuspid valve annulus towards the right ventricular outflow tract with a possibly restrictive orifice. Frequently, a coaptation gap yields tricuspid valve regurgitation and over time the "atrialized" portion of the right ventricle may dilate. The highly variable anatomy determines the clinical presentation ranging from asymptomatic to very severe with need for early operation. Echocardiography and magnetic resonance imaging are the most important diagnostic modalities to assess the tricuspid valve as well as ventricular morphology and function. While medical management of asymptomatic patients can be effective for many years, surgical intervention is indicated before development of significant right ventricular dilatation or dysfunction. Onset of symptoms and arrhythmias are further indications for surgery. Modified cone reconstruction of the tricuspid valve is the state-of-the-art approach yielding the best results for most patients. Alternative procedures for select cases include tricuspid valve replacement and bidirectional cavopulmonary shunt depending on patient age and other individual characteristics. Long-term survival after surgery is favorable but rehospitalization and reoperation remain significant issues. Further studies are warranted to identify the optimal surgical strategy and timing before adverse right ventricular remodeling occurs. It is this article's objective to provide a comprehensive review of current literature and an overview on the management of Ebstein's Anomaly. It focuses on imaging, cardiac surgery, and outcome. Additionally, a brief insight into arrhythmias and their management is given. The "future perspectives" summarize open questions and fields of future research.
AB - Ebstein's anomaly is a rare congenital heart disease with malformation of the tricuspid valve and myopathy of the right ventricle. The septal and inferior leaflets adhere to the endocardium due to failure of delamination. This leads to apical displacement of their hinge points with a shift of the functional tricuspid valve annulus towards the right ventricular outflow tract with a possibly restrictive orifice. Frequently, a coaptation gap yields tricuspid valve regurgitation and over time the "atrialized" portion of the right ventricle may dilate. The highly variable anatomy determines the clinical presentation ranging from asymptomatic to very severe with need for early operation. Echocardiography and magnetic resonance imaging are the most important diagnostic modalities to assess the tricuspid valve as well as ventricular morphology and function. While medical management of asymptomatic patients can be effective for many years, surgical intervention is indicated before development of significant right ventricular dilatation or dysfunction. Onset of symptoms and arrhythmias are further indications for surgery. Modified cone reconstruction of the tricuspid valve is the state-of-the-art approach yielding the best results for most patients. Alternative procedures for select cases include tricuspid valve replacement and bidirectional cavopulmonary shunt depending on patient age and other individual characteristics. Long-term survival after surgery is favorable but rehospitalization and reoperation remain significant issues. Further studies are warranted to identify the optimal surgical strategy and timing before adverse right ventricular remodeling occurs. It is this article's objective to provide a comprehensive review of current literature and an overview on the management of Ebstein's Anomaly. It focuses on imaging, cardiac surgery, and outcome. Additionally, a brief insight into arrhythmias and their management is given. The "future perspectives" summarize open questions and fields of future research.
U2 - 10.21037/cdt-20-771
DO - 10.21037/cdt-20-771
M3 - SCORING: Review article
C2 - 35070800
VL - 11
SP - 1310
EP - 1323
JO - CARDIOVASC DIAGN THE
JF - CARDIOVASC DIAGN THE
SN - 2223-3652
IS - 6
ER -