Modified tricuspid repair in patients with Ebstein’s anomaly

Ebstein’s anomaly is an unusual congenital heart lesion notable for its extremely variable anatomy. One characteristic is the differing degree of dysplasia and displacement of the proximal attachments of the septal and posterior leaflets from the true atrioventricular junction. The anterior leaflet is normally attached to the tricuspid annulus but is usually larger than normal, with a sail-like aspect and various degrees of interchordal space obliteration and adherence to the ventricular wall. In Ebstein’s anomaly, the valve divides the right ventricle into a proximal part between the atrioventricular junction and the displaced valve, called the atrialized portion of the right ventricle, and a distal part, called the functional right ventricle. Between 10% and 15% of these patients have accessory conduction pathways, while Wolff–Parkinson–White syndrome is the most frequent. An atrial septal defect is present in most of the patients and may result in a right-to-left shunt with cyanosis.