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Review
. 2020 Mar;12(3):1161-1173.
doi: 10.21037/jtd.2020.01.18.

Ebstein's anomaly: contemporary management strategies

Sandeep Sainathan  1 , Luciana da Fonseca da Silva  2 , Jose Pedro da Silva  2
Affiliations
Review

Ebstein's anomaly: contemporary management strategies

Sandeep Sainathan et al. J Thorac Dis. 2020 Mar.

Abstract

Ebstein's anomaly (EA) is a rare congenital cardiac anomaly. It is a disease at a minimum of the tricuspid valve (TV) and the right ventricular myocardium. 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. The neonatal presentation can be severe and every attempt should be made at medical management ideally into infancy and early childhood. 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. Some neonates initially committed to a single ventricle pathway may be converted to a biventricular repair by a delayed TV valvuloplasty. 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. If the chance of a successful TV valvuloplasty is high, it should be offered early in childhood to prevent further cardiac dilation. Adding a Bidirectional Glenn to a valvuloplasty may help salvage marginal risk patients or marginally repairable valves. If valve replacement is the only option, a bioprosthetic valve should be used as it is less thrombogenic especially with marginal right ventricular function. Heart transplantation should be considered in patients with associated left ventricular dysfunction.

Keywords: Ebstein’s anomaly (EA); Knott-Craig procedure; Modified Starnes procedure; da Silva Cone repair.

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Conflict of interest statement

Conflicts of Interest: The series “Management of Congenital Heart Disease” was commissioned by the editorial office without any funding or sponsorship. The authors have no other conflicts of interest to declare.

Figures

Figure 1
Figure 1
Carpentier classification of EA. EA, Ebstein’s anomaly. RA, right atrial; ARV, atrialized right ventricle; FRV, functional right ventricle.
Figure 2
Figure 2
Celermajer index. RA, right atrial; aRV, atrialized right ventricle; RV, right ventricle; LA, left atrium; LV, left ventricle.
Figure 3
Figure 3
Management algorithm for neonatal EA. EA, Ebstein’s anomaly; PVR, pulmonary vascular resistance; PBF, pulmonary blood flow.
Figure 4
Figure 4
Surgical pathway in neonatal EA. EA, Ebstein’s anomaly; FRV, functional right ventricle; TV, tricuspid valve; RV, right ventricle; PA, pulmonary artery.
Figure 5
Figure 5
Knott-Craig monocusp technique.
Figure 6
Figure 6
Right ventricular exclusion procedures. (A) 1: modified Starnes technique, 2: Sano technique of the right AV orifice closure; (B) Sano RV exclusion technique. ASD, atrial septal defect; MS, membranous septum; TV, tricuspid valve; RV, right ventricle.
Figure 7
Figure 7
da Silva Cone technique (2.5 times magnified).
See this image and copyright information in PMC

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