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.
2020 Journal of Thoracic Disease. All rights reserved.