How Does Ebstein ’ s Anomaly Present?

Discussion Ebstein’s anomaly (EA) accounts for about 0.3-0.5% of all congenital heart disease and about 40% of all tricuspid valve (TV) pathologies. It was first described by Dr. William Ebstein in 1866. In EA’s simplest form, the TV is displaced inferiorly from the normal hinge points into the right ventricle (RV). This is caused by delamination of both the septal and mural leaflets and they usually form a combined leaflet which can be more adherent to the RV. These anatomical changes causes poor movement of the leaflet(s) and tricuspid regurgitation. Displacement inferiorly can cause decreased RV capacity and RV outflow obstruction. Displacement also causes part of the RV to be incorporated into the right atrium (RA) which itself can cause functional RV hypoplasia. It’s not surprising that numerous other problems result because of these anatomical changes. These include atrial septal defects (with right-to-left shunting and cyanosis), RV fibrosis, pulmonary atresia or stenosis, Tetralogy of Fallot and transposition of the great vessels. The heart’s left side is also affected including left ventricle (LV) and mitral valve (MV) problems. Arrhythmias are not uncommon with both atrial, ventricular and accessory pathway tachycardias. Atrial tachycardias are quite common in unoperated older patients. Tachycardias themselves exacerbate the problem. For example, supraventricular tachycardia decreases the RV filling time, decreases RV outflow, and increases R...
Source: PediatricEducation.org - Category: Pediatrics Authors: Tags: Uncategorized Source Type: news