A – Dilated cardiomyopathy + ventricular septal defect B – Ebstein’s anomaly + ventricular septal defect C – Tricuspid atresia + ventricular septal defect D – Non-compaction cardiomyopathy
Right answer is B: Ebstein’s anomaly + ventricular septal defect
In the normal heart, the tricuspid valve has 3 leaflets: anterior, posterior, and septal. Ebstein’s anomaly is a malformation of the tricuspid valve and right ventricle characterized by (1) adherence of the septal and posterior leaflets to the underlying myocardium (failure of delamination, namely splitting of the tissue by detachment of the inner layer during embryologic development); (2) downward (apical) displacement of the functional annulus (septal>posterior>anterior); (3) dilation of the “atrialized” portion of the right ventricle, with various degrees of hypertrophy and thinning of the wall; (4) redundancy, fenestrations, and tethering of the anterior leaflet; and (5) dilation of the right atrioventricular junction (true tricuspid annulus).
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