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Understanding Ebstein's Anomaly

As a Foundation working for children whose lives are on the lines due to Congenital Heart Defects (CHD) it is important for us to understand the various types of CHD that can and have affected the little ones under our care. Regardless of how complicated the technicalities may be, it is important to especially understand the complex types such as Ebstein’s Anomaly.

The first case of Ebstein’s Anomaly was reported in the year of 1866, when a 19-year old labourer died of cyanotic heart disease. By the year 1950, there were only three cases of this anomaly that had been reported.

This particular type of heart ailment now accounts for almost 1% of all Congenital Heart Defects.

Normally the Tricuspid Valve (TV) has three leaflets: anterior, inferior (posterior) and septal. The leaflets equally from the endocardial cushion tissues and the myocardium. The leaflets and the tensile apparatus of the AV valves are formed by a process of delamination of the inner layers of the inlet zone of the ventricles. In Ebstein’s delamination of the TV leaflets fail to occur – but the mechanism is not understood.

Presentation

The presentation varies widely, and it can range from the severely symptomatic new-born to an incidental finding in a grown-up. In general, symptoms are related to the anatomic severity.

Management

Neonatal Ebstein’s Anomaly carries a poor prognosis, with a reported survival of only 68% in one series. Surgery is required in the presence of heart failure of profound cyanosis and there are different options for the surgical repair. Such as: Biventricular repair, single ventricle pathways with right ventricular exclusion, cardiac transplantation (rare). A Brazilian surgeon Dr Da Silva had developed the Cone Repair technique which has ever since been followed by many hospitals.

As a Foundation working for children, from the six cases of Ebstein’s that we have taken, young John underwent the Cone Repair technique which we financially supported. Below is a diagram that shows…

A. The abnormally displaced valve and dilated right atrial chamber

B. The abnormal valve which is detached from its position

C. Entire apparatus which is rotated clockwise so that it forms a cone (note the multiple sutures taken to join the cut edges)

D. The valve in its new shape which has been reattached. Also, at the 3 o’clock position you can see how the redundant chamber wall has been bunched up to make the chamber small and more functional