Narrative review of Ebstein's anomaly beyond childhood

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 -