Which heart valve is involved in Ebstein's anomaly?

From the Guidelines

Ebstein's anomaly primarily involves the tricuspid valve, which is a malformation of the valve and the right ventricle, as stated in the 2019 AHA/ACC guideline for the management of adults with congenital heart disease 1. The tricuspid valve is located between the right atrium and right ventricle of the heart, and in Ebstein's anomaly, it is abnormally formed and positioned lower than normal in the right ventricle. The valve leaflets are malformed, with the septal and posterior leaflets being displaced downward and attached to the ventricular wall rather than at the normal valve annulus, leading to tricuspid regurgitation and potential right-sided heart enlargement, arrhythmias, and heart failure 1. Key characteristics of Ebstein's anomaly include:

• Malformation of the tricuspid valve and right ventricle
• Displacement of the septal and posterior leaflets downward
• Atrialization of part of the right ventricle
• Potential for tricuspid regurgitation, right-sided heart enlargement, arrhythmias, and heart failure The severity of Ebstein's anomaly varies widely, with some patients having minimal symptoms while others require surgical intervention early in life to repair or replace the tricuspid valve and restore proper heart function, as recommended in the 2019 guideline 1.

From the Research

Heart Valve Involved in Ebstein's Anomaly

• The heart valve involved in Ebstein's anomaly is the tricuspid valve 2, 3, 4, 5, 6.

Characteristics of the Tricuspid Valve in Ebstein's Anomaly

• The tricuspid valve is characterized by its displacement and malformed leaflets 2.
• The septal and inferior leaflets adhere to the endocardium due to failure of delamination, leading to apical displacement of their hinge points 4.
• The tricuspid valve is usually regurgitant, but may rarely be stenotic 6.

Clinical Implications of Tricuspid Valve Involvement

• The involvement of the tricuspid valve in Ebstein's anomaly leads to a reverse flow of blood into the right-sided atrium, which may cause cardiac hypertrophy and edema of the lower extremities 2.
• The tricuspid valve regurgitation and reduced right ventricular contractility result in a decreased flow of blood out of the right heart 2.