Abstract
Ebstein’s anomaly is a rare congenital heart malformation of the right ventricle and tricuspid valve. Failure of delamination of the tricuspid valve, especially of the posterior and septal leaflets, from the underlying myocardium, results in a variable degree of tricuspid valve and right ventricular deformity. The natural history is influenced by a high rate of intrauterine and neonatal death, especially when the anomaly is associated with severe deformity of the tricuspid valve and cyanosis. At the other end of the spectrum, patients with mild dysfunction of the tricuspid valve can remain asymptomatic until late in adult life. Management therefore requires a choice between a conservative approach and operative intervention. Currently available surgical techniques have demonstrated survival and functional benefits to patients with a greater severity of Ebstein’s anomaly.
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Cone Reconstruction with Longitudinal Plication of the Atrialized Ventricle in the Newborn
Clip 1: Preoperative findings.
Clip 2: Assessment of anatomy.
Clip 3: Detachment of the leaflets of the tricuspid valve.
Clip 4: “Correction” of failure of delamination.
Clip 5: Creating a cone.
Clip 6: Exclusion of the atrialized right ventricle.
Clip 7: Attachment of the tricuspid valve to the annulus.
Clip 8: Plication of the annulus.
Clip 9: Final reattachment of the base of the cone. In addition, the atrial septal defect is partially closed, leaving a 3-mm fenestration. The redundant right atrium is excised from each side of the atriotomy, and then the atriotomy is closed.
Clip 10: The echocardiogram before discharge.
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Cone Reconstruction without Plication of the Atrialized Ventricle
Clip 1: Assessment of the extra- and intracardiac anatomy.
Clip 2: Detachment of the leaflets of the tricuspid valve.
Clip 3: Detachment of the linear attachments of the posterior leaflet.
Clip 4: Detachment of the septal leaflet.
Clip 5: Creating a cone.
Clip 6: Attachment of the tricuspid valve to the annulus.
Clip 7: Lateral annuloplasty.
Clip 8: Atrial septal defect closure and reduction atriotomy.
Clip 9: The echocardiogram before discharge.
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© 2012 Springer-Verlag Berlin Heidelberg
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Hraška, V., Murín, P. (2012). Ebstein’s Anomaly. In: Surgical Management of Congenital Heart Disease I. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-24169-7_5
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DOI: https://doi.org/10.1007/978-3-642-24169-7_5
Publisher Name: Springer, Berlin, Heidelberg
Print ISBN: 978-3-642-24168-0
Online ISBN: 978-3-642-24169-7
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