RA, right atrial ARV, atrialized right ventricle FRV, functional right ventricle. There are four types as described below.įigure 1 Carpentier classification of EA. It is particularly useful in the initial surgical planning. While different morphological classification exists, the most commonly described is the Carpentier classification ( 4) ( Figure 1). There is an Ebstein like affection of the left atrioventricular valve in Congenitally corrected transposition of great arteries which is a completely different condition from Classic EA and will not be discussed here. It accounts for 0.5% ASD/PFO (50%), pulmonary valve stenosis/atresia (4%) and VSD (3%) ( 6). Accepted for publication Nov 28, 2019.įirst described by Wilhelm Ebstein in 1866 ( 1), Ebstein’s anomaly (EA) is a rare congenital cardiac anomaly involving the tricuspid valve (TV) and the right ventricle (RV) ( 2). Keywords: Ebstein’s anomaly (EA) Modified Starnes procedure Knott-Craig procedure da Silva Cone repair Heart transplantation should be considered in patients with associated left ventricular dysfunction. If valve replacement is the only option, a bioprosthetic valve should be used as it is less thrombogenic especially with marginal right ventricular function. Adding a Bidirectional Glenn to a valvuloplasty may help salvage marginal risk patients or marginally repairable valves. If the chance of a successful TV valvuloplasty is high, it should be offered early in childhood to prevent further cardiac dilation. The da Silva Cone repair has become the valvuloplasty of choice especially beyond the neonatal period and can be applied to a wide morphological variation of the condition with good long-term durability. Some neonates initially committed to a single ventricle pathway may be converted to a biventricular repair by a delayed TV valvuloplasty. Neonates not eligible or failing medical management should be surgically managed either with a single ventricle palliative approach or a more desirable biventricular repair with a neonatal TV valvuloplasty. The neonatal presentation can be severe and every attempt should be made at medical management ideally into infancy and early childhood. Presentation varies from a severe symptomatic form during the neonatal period to an incidental detection later in life due to the wide morphological variation of the condition. It is a disease at a minimum of the tricuspid valve (TV) and the right ventricular myocardium. Policy of Dealing with Allegations of Research MisconductĪbstract: Ebstein’s anomaly (EA) is a rare congenital cardiac anomaly. Policy of Screening for Plagiarism Process.
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