What is Ebstein's abnormality primarily associated with?

Ebstein's anomaly is a congenital heart defect that primarily affects the tricuspid valve and its associated structures. The condition is characterized by a malformation of the tricuspid valve, leading to the valve being positioned lower than normal in the right ventricle. This malposition results in significant functional dysfunction and can cause the right atrium to enlarge due to increased volume overload.

The abnormal tricuspid valve can lead to a variety of symptoms, but the hallmark feature of Ebstein's anomaly is the enlargement of the right atrium, which is directly linked to the tricuspid valve's abnormalities. This enlargement can contribute to arrhythmias and other complications.

Understanding this connection helps clarify why the right atrial enlargement resulting from the tricuspid abnormality is a central aspect of Ebstein's anomaly, distinguishing it from other congenital heart defects which may be associated with different pathological features.