Repair of Ebstein Anomaly in Children: Avoiding Pitfalls

Ebstein anomaly (EA) is a congenital heart disease, which main feature is the rotational displacement of the tricuspid valve (TV) into the right ventricle (RV). Tricuspid regurgitation and RV dysfunction are common findings. The Cone procedure is a well-recognized technique for anatomical repair of EA. The basic principles of Cone repair are mobilization of all available TV leaflets and construction of a cone-like valve that is reattached to the normal atrioventricular junction. Since creation of the technique in 1993, we have introduced many technical refinements to improve TV performance, prevent cardiac arrhythmias, and improve RV function. Here, we provide some surgical details to avoid atrioventricular block and coronary compromise, facilitate patient recovery, and provide sustainable long-term results. From 2016, we have applied these refinements to 110 consecutive patients at our institution, including those with previous Starnes procedure or valve replacement, resulting in no mortality, very low morbidity, and excellent valve function.